Recent Advances in Pediatrics. Special Volume 19, Developmental

Recent Advances in Pediatrics Special Volume 19 Developmental and Behavioral Pediatrics • Recent Advances in Pediatrics (RAP): Hot Topics in General/ Miscellaneous Pediatrics Vol 17 (2007), Vol 16 (2006), Vol 15 (2005), Vol 14 (2004), Vol 13 (2003), Vol 12 (2002), Vol 11 (2001), Vol 10 (2000), Vol 9 (1999), Vol 8 (1998), Vol 7 (1997), Vol 6 (1996), Vol 5 (1995), Vol 4 (1994), Vol 3 (1993), Vol 2 (1992), Vol 1 (1991)

• Recent Advances in Pediatrics (RAP): Special Volumes RAP Special Vol 18 (2007): Pediatric Neurology RAP Special Vol 17 (2006): Adolescence RAP Special Vol 16 (2006): Pediatric Cardiology RAP Special Vol 15 (2006): Nephrology RAP Special Vol 14 (2005): Critical Care Pediatrics RAP Special Vol 13 (2004): Pediatric Endocrinology RAP Special Vol 12 (2003): Neonatal Emergencies RAP Special Vol 11 (2002): Community Pediatrics RAP Special Vol 10 (2002): Pulmonology RAP Special Vol 9 (2001): Neurology RAP Special Vol 8 (2001): Emergency Pediatrics RAP Special Vol 7 (2000): Hematology RAP Special Vol 6 (2000): Gastroenterology, Hepatology and Nutrition RAP Special Vol 5 (2000): Neonatology-2 RAP Special Vol 4 (1999): Neonatology RAP Special Vol 3 (1999): Tropical Pediatrics-2 RAP Special Vol 2 (1998): Tropical Pediatrics RAP Special Vol 1 (1997): Nutrition, Growth and Development

• RAP Volumes in Pipeline for 2007 RAP 18: Hot Topics in General/Miscellaneous Pediatrics RAP Special Vol 20: Pediatric Hematology RAP Special Vol 21: Nutrition, Growth and Development

• Other Books by Prof (Dr) Suraj Gupte The Short Textbook of Pediatrics, 11th edition, 2007 Differential Diagnosis in Pediatrics, 5th edition, 2007 Instructive Case Studies in Pediatrics, 5th edition, 2007 Pediatric Drug Directory, 9th edition, 2007 Recent Advances in Pediatrics Special Volume 19 Developmental and Behavioral Pediatrics Editor Suraj Gupte MD, FIAP Professor and Head Department of Pediatrics Maharaja Agrasen Medical College Agroha, Hisar 125047 Haryana, India E-mail: [email protected] [email protected] Honorary Director: Pediatric Education Network, Children’ Health Center Editor: The Short Textbook of Pediatrics, Textbooks of Pediatric Emergencies, Neonatal Emergencies and Pediatric Nutrition, Pediatric Yearbook Series, Pediatric Today Monograph Series, Newer Horizons in Tropical Pediatrics, Towards MRCPCH Part II (Theory) Examination Author: Differential Diagnosis in Pediatrics, Instructive Case Studies in Pediatrics, Pediatric Drug Directory, Infant Feeding, Speaking of Child Care: Everything You Wanted to Know, The Baby Book: The Parents’ Guide from Birth to Infancy Co-editor: Asian Journal of Maternity and Child Health (Manila, Philippines) Section Editor: Pediatrics Today (New Delhi, India) Editorial Adviser: Asian Journal of Pediatric Practice (New Delhi, India) Editorial Advisory Board Member/Reviewer: Indian Journal of Pediatrics (New Delhi, India), Synopsis: A Current Survey of World Literature in Pediatrics (Detroit, USA), Indian Journal of Pediatric Gastroenterology, Hepatology and Nutrition (Jaipur, India), Maternal and Child Nutrition (Preston, UK) Examiner: National Board of Examinations (NBE) for DNB, New Delhi, All India Institute of Medical Sciences (AIIMS), New Delhi, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, Sher-i-Kashmir Institute of Medical Sciences (SKIMS) and several universities Pediatric Faculty Selection Expert: All India Institute of Medical Sciences, New Delhi, Punjab Public Service Commission, Jammu and Kashmir Public Service Commission, Union Public Service Commission

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Typeset at JPBMP typesetting unit Printed at Ajanta Offset To The fond memory of our late parents whose inspiration, blessings and moral support continue to contribute a great deal to our academic endeavors and everybody contributing to child health and welfare for a better future

Contributors

AE Abraham MS Bhatia Senior Consultant Professor and Head Department of Child Development Department of Psychiatry Child Health Center University College of Medical Sciences Edinburgh, United Kingdom and GTB Hospital Ch 1: Developmental Assessment and the Delhi, India Pediatrician Ch 20: Tic Disorders Kiran Aggarwal PK Bhatnagar Sr. Specialist (Pediatrics) Associate Professor Municipal Corporation of Delhi, Child and Adolescent Development Delhi, India Institute of Child and Adolescent Ch 27: Child Abuse and Neglect Health, London, United Kingdom FA Ahmed Ch 28: Childhood Injury Control Consultant Department of Child Development Parthasarathy Biswas Child Health Center Senior Research Officer Edinburgh, United Kingdom Department of Psychiatry Ch 1: Developmental Assessment and the National Drug Deaddiction and Pediatrician Treatment Center) All India Institute of Medical Sciences Shabina Ahmed New Delhi, India Director, Assam Autism Foundation, Ch 9: Childhood Disintegrative Disorder Guwahati, India and Rett’s Syndrome Ch 10: Autism and Related Disorders: Regression of Milestones Shabari Dutta Akash Bang Consultant Psychiatrist Lecturer Nur Manzil Psychiatric Center Department of Pediatrics Lucknow, UP, India Medical College, Amravati, India Ch 5: Eating Disorders Ch 23: Anger, Hostility and Aggression Syndrome David H Van Dyke Professor of Neurology and Pediatrics B Vishnu Bhat Michigan State University College of Professor and Head Human Medicine Department of Pediatrics A217 Clinical Center Jawaharlal Postgraduate Institute of East Lansing Medical Education and Research Michigan , USA Puducherry, India Ch 13: Epilepsy in the Adolescent Ch 25: Play Therapy Ch 14: Headaches in the Adolescent viii RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Arthur Feinberg Ravi Gupta Department of Pediatrics Senior Resident Michigan University College of Department of Psychiatry Human Medicine University College of Medical Sciences Kalamazoo Center for Medical Studies and GTB Hospital, Delhi, India Kalamazoo, Michigan, USA Ch 20: Tic Disorders Ch 12: Lead Toxicity and Neurobehavior Suraj Gupte Ajay Gaur Professor and Head Associate Professor Department of Pediatrics Department of Pediatrics Maharaja Agrasen Medical College GR Medical College Agroha, Hisar, India Gwalior, India Ch 1: Developmental Assessment and the Ch 24: Behavior Analysis in Children Pediatrician Ch 2: Normal Development Sapna Gill Ch 17: Dyslexia Clinical Psychologist Ch 18: Mathematical Disabilities: 2710, Sorority Lane Developmental Aspects Holt, Michigan Ch 19: Behavioral Problems: Overview USA Ch 26: Developmental Aspects of Self- Ch 22: Disruptive Behavior Disorders esteem Ch 28: Childhood Injury Control Donald E Greydanus Ch 29: Adoption Professor Pediatrics and Human Development Tina Head Michigan State University College of Doctoral Scholar Human Medicine Department of Psychology Pediatrics Program Director Western Michigan University Michigan State University/ Kalamazoo, Michigan , USA Kalamazoo Center for Medical Studies Ch 15: The Adolescent with Spina Bifida: Kalamazoo, Michigan, USA Ch 8: Attention-Deficit Hyperactivity Dis- Psychological Aspects of Development order in Adolescent: Psychopharmacology AE Laurence Ch 11: Obessive-Compulsive Disorder Associate Professor Ch 13: Epilepsy in the Adolescent Division of Child Development Ch 14: Headaches in the Adolescent Center for Medical Sciences Studies Hong Kong Nitin Gupta Ch 19: Behavioral Problems: Overview Consultant Psychologist South South Staffordshire Healthcare E Levine NHS Trust Honorary Professor Burton Upon Trent Child and Adolescent Development United Kingdom Institute of Child and Adolescent Ch 9: Childhood Disintegrative Disorder Health, London, United Kingdom and Rett’s Syndrome Ch 18: Mathematical Disabilities: Ch 22: Disruptive Behavior Disorders Developmental Aspects Contributors ix

Khyati Mehtalia Helen D Pratt Consultant Psychiatrist Professor, Hospital for Mental Health Pediatrics and Human Development BJ Medical College and Michigan State University College of Civil Hospital, Ahmedabad, India Human Medicine Ch 6: Adolescent Depression Director, Behavioral/Developmental Ch 16. Post-traumatic Stress Disorder Pediatrics, Pediatrics Program Michigan State University/ Devendra Mishra Kalamazoo Center for Medical Studies Assistant Professor (Pediatrics) Kalamazoo, Michigan, USA Chacha Nehru Bal Chikitsalaya, Ch 7: Attention-deficit Hyperactivity (Maulana Azad Medical College) Disorder: Evaluation and Psychological Geeta Colony, Delhi, India Management Ch 27: Child Abuse and Neglect Ch 11: Obsessive-Compulsive Disorder Tina Nichols Department of Pediatrics Rathi Sharmila R Michigan University College of Senior Resident Human Medicine Department of Pediatrics Kalamazoo Center for Medical Studies Jawaharlal Postgraduate Institute of Kalamazoo MI, USA Medical Education and Research Ch 12: Lead Toxicity and Neurobehavior Puducherry India Daniel Olson Ch 25: Play Therapy Michigan State University College of Human Medicine JR Ram East Lansing and Kalamazoo, Consultant Psychiatrist Michigan, USA Apollo Gleneagles Hospitals Ch 8: Attention-Deficit Hyperactivity Dis- Kolkata order in Adolescent: Psychopharmacology India Ch 14: Headaches in the Adolescent Ch 3: Pediatric Psychopharmacology: Samir Parekh Recent Controversies Consultant Psychiatrist Ch 4: Somatization Disorders Max Hospitals, New Delhi, India Ch 6: Adolescent Depression Ansuya A Reddy Consultant Dilip R Patel Developmental Pediatrics Professor Prince of Wales Hospital Pediatrics and Human Development Hong Kong Michigan State University College of Ch 29: Adoption Human Medicine Adolescent and Sports Medicine PSN Reddy Director Associate Professor Pediatrics Program Human Developmental and Social Michigan State University/ Sciences Kalamazoo Center for Medical Studies The Chinese University of Hong Kong Kalamazoo, Michigan, USA Hong Kong Ch 11: Obsessive-compulsive Disorder Ch 29: Adoption x RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Utpal Kant Singh GK Vankar Professor and Head Professor and Head Department of Pediatrics BJ Medical College and Nalanda Medical College Civil Hospital Patna, India Ahmedabad Ch 17: Dyslexia India Ch 18: Mathematical Disabilities: Ch 16: Post-traumatic Stress Disorder Developmental Aspects Ch 19: Behavioral Problems: Overview Lilly Wilson Junior Clinical Psychologist Satish K Tiwari Center for Psychology and Mental Associate Professor Health Department of Pediatrics Washington DC Medical College USA Amravati, India Ch 26: Developmental Aspects of Self- Ch 21: Risk-Taking Behavior esteem Ch 23: Anger, Hostility and Aggression Syndrome WE Wilson Clinical Psychologist Artemis K Tsitsika Center for Psychology and Mental Scientific Supervisor Health Pediatrics-Adolescent Medicine Washington DC Adolescent Health Unit USA 2nd Department of Pediatrics Ch 17: Dyslexia University of Athens, Greece Ch 26: Developmental Aspects of Ch 14: Headaches in Adolescent Self-esteem Preface

“Developmental and behavioral health of the child is important,” says British Medical Journal in a recent issue. So is the prevention and early detection and management of persistent developmental and behavioral disorders! These disorders are usually multifactorial in origin, their clinical expression depending on such variables as temperament, developmental level, stress level and coping and adoptive ability of the family. Parents are frequently concerned about the so-called “odd behavior” of the child. Is it normal? Will the child grow out of it ? Does it need intervention? Though developmental and behavioral pediatrics remained by and large neglected in the past, over the years spectacular advances have occurred in this field. Mercifully the process is going on and on. According to the World Health Organization, all these issues plus much more needs to be addressed and documented for interaction, research, innovation and real action for the eventual improvement in child and adolescent health and care. This, the 19th Special Volume of the noted international series, Recent Advances in Pediatrics, first launched in 1991 and now concurrently in the 17th Annual (RAP 17: Hot Topics) and 18th special volume (RAP Special Vol 18: Pediatric Neurology), aims at covering key topical issues concerning developmental and behavioral problems in children and adolescents. The 29 state-of-the-art chapters covered by as many as 38 experts drawn from India and abroad attempt to review the depth and breadth of knowledge in the field of developmental and behavioral pediatrics with special reference to its applicability in the Indian subcontinent. Each and every chapter is remarkable by its excellence, providing a stimulating and, not infrequently, even provocative, matured and informed update. Understandably, such an approach is befitting when the goal is not only to inform but also to motivate discussion, interaction, research and innovation. xii RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In keeping with the professed policy of the series, multidisciplinary approach with an eye on future continues to occupy pride of place in this volume too. We wish to record our indebtedness to our distinguished contributors for providing excellent state-of-the-art chapters in various aspects developmental and behavioral. They were also gracious enough to repose confidence in our editorship. Without their understanding and cooperation, this volume wouldn’t have seen the light of the day. Thanks are also due to the Advisory Editorial Board for providing inputs and advice in various editorial matters and peer reviewers for not only providing us the benefit of their critical expertise but also for the promptness in responding to our requests for speedy action. Our daughter, Dr. Novy, and son, Er. Manu, voluntarily provided all sorts of assistance in taking the project to its logical conclusion. Jaypee Brothers Medical Publishers (P) Ltd and their dedicated staff deserve our special thanks for speedy production and skillful production qualities of the book.

Suraj Gupte Academic Editor

Shamma-Bakshi Gupte Executive Editor Contents

1. Developmental Assessment and the Pediatrician ...... 1 AE Abraham, FA Ahmed, Suraj Gupte 2. Normal Development ...... 10 Suraj Gupte 3. Pediatric Psychopharmacology: Recent Controversies...... 23 JR Ram 4. Somatization Disorders...... 30 JR Ram 5. Eating Disorders ...... 43 Shabari Dutta 6. Adolescent Depression ...... 52 Samir Parekh, Khyati Mehtalia 7. Attention-Deficit Hyperactivity Disorder in Adolescents: Evaluation and Psychological Management ...... 60 Helen D Pratt 8. Attention-Deficit Hyperactivity Disorder in Adolescent: Psychopharmacology ...... 79 Donald E Greydanus, Daniel Olson 9. Childhood Disintegrative Disorder and Rett’s Syndrome ...... 99 Parthasarathy Biswas, Nitin Gupta 10. Autism and Related Disorders: Regression of Milestones ..... 115 Shabina Ahmed 11. Obsessive-Compulsive Disorder ...... 131 Helen D Pratt, Dilip R Patel, Donald E Greydanus 12. Lead Toxicity and Neurobehavior...... 137 Tina Nichols, Arthur Feinberg 13. Epilepsy in the Adolescent ...... 149 Donald E Greydanus, David H Van Dyke 14. Headaches in the Adolescent ...... 158 Donald E Greydanus, Artemis K Tsitsika, David H Van Dyke, Daniel Olson xiv RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

15. The Adolescent with Spina Bifida: Psychological Aspects of Development ...... 170 Tina Head 16. Post-traumatic Stress Disorder ...... 196 GK Vankar, Khyati Mehtalia 17. Dyslexia ...... 210 Suraj Gupte, Utpal Kant Singh, WE Wilson 18. Mathematical Disabilities: Developmental Aspects ...... 215 E Levine, Suraj Gupte, Utpal Kant Singh 19. Behavioral Problems: Overview...... 222 Utpal Kant Singh, AE Laurence, Suraj Gupte 20. Tic Disorders...... 244 MS Bhatia, Ravi Gupta 21. Risk-Taking Behavior ...... 259 Satish K Tiwari 22. Disruptive Behavior Disorders...... 271 Sapna Gill, Nitin Gupta 23. Anger, Hostility and Aggression Syndrome ...... 300 Akash Bang, Satish K Tiwari 24. Behavior Analysis in Children...... 309 Ajay Gaur 25. Play Therapy...... 341 B Vishnu Bhat, Rathi Sharmila R 26. Developmental Aspects of Self-esteem ...... 348 Suraj Gupte, WE Wilson, Lilly Wilson 27. Child Abuse and Neglect ...... 357 Devendra Mishra, Kiran Aggarwal 28. Childhood Injury Control ...... 388 PK Bhatnagar, Suraj Gupte 29. Adoption ...... 394 Suraj Gupte, Ansuya A Reddy, PSN Reddy

Index ...... 401 RAP Special Volume 19: Developmental and Behavioral Pediatrics

Developmental Assessment and 1 the Pediatrician

AE Abraham, FA Ahmed, Suraj Gupte

INTRODUCTION The pediatrician must play a pivotal role in identifying developmental disability since he is the one who gets uniquely involved in the care of the infant.1-5 Unfortunately, in actual practice, it does not often happen.6-8 The major reason appears to be much-too-much load on the pediatrician in busy settings. And, perhaps, a notion on the part of a substantial proportion of pediatricians that developmental screening is not only time-consuming but also the domain of neurologist/psychiatrist.9-13 How untrue ! In a recent internet study, conducted under the aegis of Pediatric Education and Communication Network (PECN), 72% European, 70% American, and only 20% Indian pediatricians said they were routinely carrying out some form of developmental screening in their general practice. According to Needlman,14 in one large multicity study, physicians identified only 23% of emotional problems and 19% of hyperactivity before other professionals did. Though Down Syndrome and cerebral palsy were detected at 0.6 months and 10.3 months, respectively, mean ages at which mental retardation, speech defects and hearing problems were detected happened to be 34 months, 38 months and 39 months, respectively. This chapter intends to provide a short-yet-comprehensive account of developmental screening and assessment. Needless to say, in doing so, liberal use has been made of the commendable works of many authorities, including Professor Arnold Gesell (“Developmental Diagnosis”, “Biographies of Child Development”, Bayley (“Manual Growth During the First Three Years” ), Knobloch, Pasamanick (“Gessel and Amatruda’s Developmental Diagnosis”), Griffiths (“The Abilities of Babies”), Denver, 2 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Illingworth (“The Development of the Infant and Young Child”, “Your Child’s Development in the First Five Years”, “Basic Developmental Screening”, “Babies and Young Children”, “Some Problems of the Early Years and their Treatment”), intensely involved in the field. Undoubtedly, also has been made the full use of authors’ over 3 decades of experience in dealing with thousands of neonates, infants and children as also close observations on our own children (and nephews and nieces) in the form of notes that we had started making right since their births, especially commencing with the first noteworthy experiences that they exhibited. Before dwelling on the details, let us recall what developmental assessment denotes. Developmental assessment should be considered a 2-step process comprising screening and diagnosis. The first step, screening, should be reserved for picking up children in need of detailed assessment. The second step, diagnosis, should aim at precisely defining the developmental problems as also their significance from the angle of child’s biologic, psychologic and social strengths and weaknesses. Of late, developmental screening is giving way to developmental surveillance. The latter should be considered an ongoing process based on inputs from parents and other multiple sources and detailed longitudinal observations with or without the assistance of screening tests such as DDST. In fact, assessment takes off with the history per se, open-ended provocative questions for ascertaining the temperament and personality of the child and the close family members as also for identifying concerns of parents. The role of previsit questionnaires in this behalf is remarkable. These are very helpful in soliciting concerns and focusing the agenda of the visit, conveying interest of the pediatricians in developmental issues and encouraging parents to express concerns that may have otherwise escaped attention. A direct examination, including office testing (say DDST) is important for documenting abilities.

HISTORICAL ASPECTS Charles Darwin was the first to publish a detailed record of child development, including observations on crying, sobbing, laughter and other emotions in “The Expression of the Emotions in Man and Animals” (1872) and “A Biographical Sketch of an Infant” (1877) followed by Shin in 1893 and Shirley in 1931. Developmental Assessment and the Pediatrician 3

Stern and Kuhlman in 1912 gave the concept of intelligence quotient (IQ), the ratio between assessed mental age and actual chronological age. In early and mid-1990s, Arnold Gessel followed by Knobloch, Pasamanick, Ruth Griffiths and others published norms of development on a large number of children. In 1967, the famous Denver Developmental Screening test (DDST) was documented. It was based on a sample of 1000 selected children. It assessed development of infants and children (usually up to 3 years) in 4 vital areas, namely gross motor, fine motor adaptive, language and personal social behavior. There were 105 items, some indeed difficult to administer. Moreover, it was not quite appropriate for children with mothers who were not having enough education. In addition, it had less items related to language. As a shortcut, a “short” DDST became available but it had got to be followed up by the full DDST subsequently for dependable results. In 1981, a major revision, modification and standardization of the original DDST occurred in the form of Denver II which has 125 items instead of 105 and yet takes only 5-7 minutes. Otherplus points of Denver II over DDST include availability of Denver II Screening Manual, availability of Denver II Technical Manual and availability of a video instructional program and proficiency test. Make no mistake that it is only a screening test for identifying children who are not performing in keeping with their ages, irrespective of the reason(s). It undoubtedly does not measure intelligence or developmental quotient. Attempts are on way to produce a short (abbreviated) Denver II needing just 5 minutes for assessment. Details are provided in Chapter XX. At this stage, remember, the major limitation of DDST lies in its wrong use as an IQ test which it indeed is not. About the same time, Boel tests for visual, auditory and tactile sense emerged on the scene for use in infants aged 7-9 months. In these tests, a red object is employed to attract visual attention, four bells, attached to the tester’s fingers, attract auditory attention. The other development screening tests that appeared on the scene included Brazelton and Dubowitz tests, Gessel DST, Bayley DST, Woodside DST, developmental profile (DP-II), cognitive adaptive test/ clinical linguistic auditory milestone scale (CAT/CALMS), early language milestone scale (ELM) and Vineland social maturity scale. 4 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In India, Phatak from Baroda (now rechristened Varoda) adopted the so-called Baroda screening tests from Bayley developmental scale to suit the Indian infants and children, aged 0-36 months. It has 25 test items listed according to child’s age. It is primarily meant for use by the child psychologists rather than pediatricians. Domains evaluated are gross motor, fine motor, and cognitive. Administration time is 10 minutes. Sensitivity is 0.66-0.93 while specificity is 0.77-0.94. Yet another test developed in India (Trivandrum DST) is based on Baroda norms. It has 17 test items and is relevant for age 0-2 years. Domains evaluated are gross motor, fine motor and cognitive. Administrative time is 5 minutes. Validity and specificity are 0.67 and 0.79 respectively.

WHY DEVELOPMENTAL SCREENING?11-15 Developmental screening is important to parents, to obstetricians, to neonatologists and to pediatricians. Let’s see how in the following paragraphs. First thing first! Every parent is eager to know if the child is developing normally, especially if there is history of a miscarriage or stillbirth, mental retardation, physical disability, maternal infection, disease or drug abuse. Developmental screening, is, therefore, an essential prerequisite for developmental assessment and, subsequently, for any corrective intervention.

WHO IS THE BEST FOR DEVELOPMENTAL SCREENING: PEDIATRICIAN OR PSYCHOLOGIST? As an accepted convention, a pediatrician is supposed to base his developmental screening on detailed history and physical examination with special reference to developmental examination, some investigations and the overall interpretation of the whole spectrum. This is absolutely logical because a large number of factors (prenatal, natal and postnatal) have a significant bearing on child’s development. The pediatrician must obtain relevant information in relation to these factors if he is to reach the right conclusion about the developmental quotient (DQ). The pediatrician should be reassuring but only up to a point. In no case should he dismiss parental developmental concerns prematurely in his over-enthusiasm to provide support and advocacy to parents. The probability of premature reassurance becomes most likely when the child has normal motor ability Developmental Assessment and the Pediatrician 5 or when he is cute, sweet, alert or sociable. The pediatrician’s role should, therefore, be considered “central” in early and fair identification of developmental defects. Once developmental delays are identified, he is also expected to have the full evaluation and provide support to the child and the family to maximize child’s potential abilities. In contradistinction, the psychologist is not much bothered about the history and physical examination and depends, by and large, on the purely objective tests based on scoreable items of behavior. His major goal is a unitary figure or score for assessment. This approach is likely to lead to fallacies.

DEVELOPMENTAL SCREENING AND DEVELOPMENTAL PREDICTION Despite doubts raised by certain workers about the value of developmental screening, as per the conviction of Professor Illingworth and many other experts, developmental testing in infancy does have a definite predictive value. Yet, let me put it straight that developmental prediction through screening is not without limitations. Illingworth has eminently listed these as follows: • We cannot draw a clear-cut dividing line between normal and abnormal. • We cannot make accurate predictions of child’s future intelligence and achievements because these will be profoundly affected by environmental and other factors. • We cannot eliminate the possibility that he will undergo mental deterioration in future months or years. • We cannot assess at one solitary examination the extent of the damage that he has suffered or its reversibility if the child has already suffered severe emotional deprivation. • We cannot be sure that he is slow starter/maturer if he is retarded and has no microcephaly. • We cannot tell after the neonatal period if we should allow for prematurity or not if he was a low birthweight baby and we do not know the duration of gestation. • We cannot make a sensible prediction for a fullterm baby at birth or in the first 4 weeks unless there are grossly abnormal signs. 6 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• We cannot rely on diagnosing mild cerebral palsy (CP) or mild mental subnormality in the early weeks of life. • We cannot really be too sure about the dependability of the abnormal neurologic signs detected in the first few weeks. • We cannot eliminate infancy the possibility that he will subsequently display specific learning disorders or difficulties of spatial appreciation. • We cannot translate into figures Gessell’s insurance factors, namely the baby’s alertness, interest in surroundings, social responsiveness, determination and power of concentration, neither can we score the quality of his vocalization without special equipment. • We cannot say what he will do with his talents or developmental potential. • We cannot prove, in any but exceptional cases, that a child’s mental or neurologic deficits are due to birth rather than to prenatal causes. • We cannot normally predict mental superiority.

HARMS AND LIMITATIONS OF THE DEVELOPMENTAL SCREENING According to one of us (SG), “I had never realized that there indeed was some danger inherent in developmental screening until I came across an absolutely normal child whom, as per the parents, I had designated as abnormal (Cerebral palsy) when he was 5 months of age. Undoubtedly, my wrong diagnosis had caused much anxiety to the parents. That was in the beginning of my career as a junior pediatrician at Snowdon Hospital attached to the HP Medical College (now renamed Indira Gandhi Medical College), Shimla, India, in early 1970s.” Subsequently, the author has encountered many children diagnosed as “spastic”, “mentally retarded”, “hydrocephalic” though, in fact, there is nothing wrong with them. It is not so easy to visualize how much anxiety can such a mistake cause to vulnerable parents. Likewise, a missed diagnosis too is undesirable. It becomes a tragedy for the parents when a child labelled as “normal” subsequently turns out to be abnormal. Table 1.1 lists the abuses to which developmental screening tests may be subjected. Developmental Assessment and the Pediatrician 7

Table 1.1: Abuses to which screening tests are subject

• Failure to follow the instructions for administration • Failure to follow the instructions for scoring • Overinterpretation of screening results • Focusing on screening tests to the exclusion of other sources of information • Screening too infrequently • Employing tests that are culturally biased • Failure to follow up with further assessment and therapeutic intervention as and when indicated.

It needs to be emphasized—yes, even at the expense of repetition— that, when in doubt about the real status of the child, it is always wise to re-examine him and, if found necessary, to advise the parents to come for follow-up. But, remember, this must be done without causing worry to parents.

PARENTS’ OPINION AS A PRESCREENING DEVELOPMENTAL TEST Eliciting parents’ concerns about child’s developmental status is on the threshold of emerging as an important prescreening procedure for detecting developmental delay.13-15 Studies have shown that parents who express concerns about speech, language, fine motor or cognitive skills have children with an 80% chance of failing standardized developmental screening. In a recently-concluded study from Chandigarh, India, it has been demonstrated that parents of delayed children very often do not raise global/cognitive concerns and are more likely to raise social, gross motor, behavior, expressive language and medical concerns (e.g. not growing well, remains sick, not eating). We do agree to the suggestion that “ pediatricians should routinely and carefully elicit parents’ opinions and concerns which need to be viewed as helpful adjuncts to routine assessment and should be used to make appropriate referrals” ( Malhi and Singhi, CHD).

KEY LEARNING POINTS • Pediatrician must play a pivotal role in identifying developmental disability since he is the one who gets uniquely involved in the care of the infant • Pediatrician is supposed to base his developmental screening on detailed history and physical examination with special reference to 8 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

developmental examination, some investigations and the overall interpretation of the whole spectrum. • Psychologist depends, by and large, on the purely objective tests based on scoreable items of behavior. His major goal is a unitary figure or score for assessment. This approach is likely to lead to fallacies. • Notwithstanding limitations, developmental screening has a definite value. • Erratically administered screening tests may prove counterproductive and harmful. • When in doubt about the real status of the child, it is always wise to re-examine him and, if found necessary, to advise the parents to come for follow-up. This must be done without causing worry to parents • Pediatricians should routinely and carefully elicit parents’ opinions and concerns which need to be viewed as helpful adjuncts to routine assessment and should be used to make appropriate referrals.

REFERENCES 1. Algranati P. Effect of developmental status on the approach to physical examination. Pediatr Clin North Am 1998;45:1-23. 2. Dworkin P. British and American recommendations for developmental monitoring: The role of surveillance. Pediatrics 1989;84:1000-1010. 3. Frank WE. Perspectives in Child Development, 2nd edn. London: Smith and Smith, 2000 4. Frankenburg WK, Dodds J, Archer P, et al. Rhe Denver II: A major revision and restandardization of the Denver developmental screening test. Pediatrics 1992;89: 91-97. 5. Glascoe FP, Dworkin PH. The role of parents in the detection of developmental and behavioral problems. Pediatrics 1995;95:829-836. 6. Glacoe FP. Altmeier WA, McClean WE. The importance of parents concerns about their child’s development. Am J Dis Child 1989’143:955-958. 7. Glascoe FP. Parents’ concerns about children’s development: Prescreening technique or screening test. Pediatrics 1997;99:522-528. 8. Green M. Bright Futures: Guidelines for Health Supervision of Infants, Children and Adolescents. Arlington: National Center for Education in Maternal and Child Health 1994. 9. Gupte S. Growth and development, In: Gupte S (ed) The Short Textbook of Pediatrics, 10th edn. New Delhi : Jaypee Brothers 2004: 10. Malhi P, Singhi P. Role of parents’ evaluation of developmental status in detecting developmental delay in young children. Indian J Pediatr 2002;39:2002. 11. Illingworth RS. The Development of the Infant and Young Child, 9th edn. Edinburgh: Churchill Livingstone, 1987. 12. Illingworth RS. Basic Developmental Screening, 3rd edn. Oxford: Blackwell, 1982. Developmental Assessment and the Pediatrician 9

13. Illingworth RS. Your Child’s Development in the First Five Years. London: Churchill Livingstone, 1981. 14. Needlman RD. Developmental assessment. In: Behrman RE, Kliegnan RM, Jenson HB (eds) Nelson Textbook of Pediatrics, 16th edn. Philadelphia: WB Saunders 2000:61-65. 15. Squires J, Nickel R, Eisert D. Early detection of developmental problems: Strategies for monitoring young children in practice settings. J Dev Behav Pediatr 1996;17:420-427. RAP Special Volume 19: Developmental and Behavioral Pediatrics

2 Normal Development

Suraj Gupte

GOVERNING PRINCIPLES Here’s a story from the beginning of my own career as a pediatrician. During my short stint at the Hospital for Sick Children, Great Ormond Street, London, I had argued against the diagnosis of cerebral palsy in an infant before a senior consultant in a way that brought me some embarrassment. To recapitulate the whole incidence, we had an 11-month-old, with history of perinatal insult, who had significantly delayed motor milestones. At the time of examination, he could only hold his head, sit and crawl. There was clear-cut spasticity, more marked in the legs. What had really baffled me was the observation of nearly normal development of manipulation. The Consultant took pains to drive home to me an important point: if IQ is normal, development of manipulation is likely to be all right. And, yet, it may well be cerebral palsy (CP) provided that other evidence in favor of CP exists as was the state of affairs in the case under review. I have never, never forgotten this anecdote.

Lesson: There may well be lack of parallelism (dissociation) between different fields of development. A child may be slow in developing “grasp” and, yet, rather fast in learning to stand or walk. This is an important principle of development. There are quite a few more. Let’s note down the various principles of development.1-3 • Development begins right from conception, occurs in utero and continues after birth. • Development proceeds in a definite sequence in all children though its rate may vary from child to child. Normal Development 11

• Development in one field need not necessarily run parallel with another field. • Development by and large depends on the maturation of nervous system. Of course, non-availability of opportunity to carry out practice may prove a retarding factor. • There is a tendency for the generalized mass activity in the infant to give way to specific individualized responses as he grows older. • Development is in cephalocaudal direction. • The primitive reflexes have got to disappear before corresponding voluntary movement is acquired.

DEVELOPMENT OF LOCOMOTION, MANIPULATION, ETC.3-5 Locomotion The development of locomotion is most importantly observed in the following positions: • When the infant is held in ventral suspension • When he is placed in prone position • When he is pulled to sitting position • When he is pulled to upright position (only in older infant).

Ventral Suspension Holding the baby off the bed in the prone position with the palm of examiner’s hand under his chest or abdomen shows nearly complete lack of head control, the head immediately flopping down, in the neonate. • By 4 -6 weeks, he tries to hold the head in the body’s plane (horizontal plane) but only for a moment. • By 8 weeks, he can maintain holding the head in the body’s plane for several seconds. • By 12 weeks, he can maintain head well beyond the body’s plane for several seconds.

Supine Position Placing the neonate in supine position and then gently pulling him up by the arms to a sitting position, cases complete head lag and rounding of the back. 12 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Between 12 to 20 weeks, he slowly gains control of the head with the curvature of the spine becoming less prominent.

Prone Position The normal posture of the neonate, particularly in first 2 weeks, is lying with his head turned to one side, the pelvis high and the knees drawn up under the abdomen. There is, however, a progressive tendency for the pelvis to become lower and hip and knees to extend in the subsequent weeks. • About 4-6 weeks, pelvis is still high and there is intermittent extension of hips. • About 6-8 weeks, pelvis becomes flat and hips well extended. • By 4 weeks, he may momentarily lift the chin off the bed. • By 12 weeks, he holds the chin and shoulders off the bed with legs fully extended. This is shortly followed by lifting the front of the chest off the bed. At this stage plane of the face is 90o to it and the weight is borne on the hands. • By 24 weeks, he tends to keep chest and upper part of abdomen off the bed while maintaining weight on the hands with extended elbows. Moreover, he can roll from prone to supine position. • By 28 weeks, he rolls from supine to prone position and shows the frog position with legs abducted and the soles of the feet coming together. At this stage, he can lift his head and greater part of the chest while bearing weight on one or both extended hands. • By 36 weeks, he can crawl. • By 40 weeks, he can creep on hands and knees while the abdomen is off the bed. • By 48-52 weeks, he may creep like a bear.

Sitting Position As soon as the neonate is pulled up to the sitting position, he demonstrates complete head lag. • When he is half pulled up to sitting position, he tries to raise his head. • By 24 weeks, he can sit propped up in his pram with trunk erect. • By 12 weeks, there is only slight head lag. • By 20 weeks, there is no head lag at all. Normal Development 13

• By 24 weeks, he lifts the head off the bed when he is about to be pulled up. • By 26 weeks, he sits on the floor with arms forward for support. • By 28 weeks, he can sit without support for a few seconds. • By 40-44 weeks, he sits steadily and can perform different movements. • By 60 weeks, he can comfortably seat himself in a safe and protected chair.

Standing and Walking Position • By 6-8 weeks, walking reflex should disappear, except when neck is extended. • At 8 weeks, the infant holds his head up momentarily when held in standing position. • By 24 weeks, he no longer sags at the hip and knees and, instead, can bear nearly all weight provided a chance is given. • At 36 weeks, he stands holding on to a support and can pull himself up to the standing position. He, however, cannot let himself down. • At 44 weeks, he can lift one foot off the floor. • At 48 weeks, he walks holding on to the furniture. • At 13 months, he walks without help but with a broad base and steps of unequal direction and length. Usually with the shoulder abducted and elbows flexed. • At 15 months, he creeps upstairs and can get into the standing position without help. • At 18 months, he can get up and down stairs without help and pull a doll or wheeled toy along the floor. • At 2 years, he can pick an object up without falling, can run and walk backward, and can go up and downstairs with two feet per step. • At 3 years, he can stand for a few seconds on one leg, can climb stairs one foot per step, and climb downstairs two feet per step, and ride (with pedaling) a tricycle. • At 4 years, he climbs downstairs one foot per step and skips on one foot. • At 6 years, he can skip on both feet.

Additional Forms of Locomotion In addition to different forms of locomotion, the infant may become quite an expert at getting about in the neighborhood much before becoming proficient in walking: 14 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• By rolling • By, while in supine position, elevating the buttocks and lower part of the body from the floor and progressing by a series of bumps at buttocks • By getting about on one hand and one buttock or both hands and both buttocks • By crawling backwards.

MANIPULATION Hand and Finger Skills Remember, primitive grasp reflex of first 8-12 weeks has got to disappear by the time voluntary grasp starts off. At 4 weeks, the hands are still by and large closed. Then, gradually, hands show a progressive tendency to open. • By 12 weeks, hands are almost open. If a bright object is brought in front of his eyes, he watches it with interest but doesn’t actually reach out to grasp it. If the object is placed in his hand, he would, of course hold it. • At 16 weeks, he attempts to reach out for the object but is ataxic and overshoots the mark. He pulls his dress as his hands come together while he is playing. • At 20 weeks, he actually succeeds in grasping the object voluntarily. In addition he plays with his toes. Until this time, his grasp is ulnar. Now it begins to go through several stages: ulnar-radial-finger-thumb grasp. In short, cube is grasped in the palm on the ulnar side in first 6 months. From 6 to 8 months, it has held against the thenar eminence at the base of the thumb. At 8-10 months, index finger with the assistance of ring finger and little finger presses the cube against the lower part of the thumb. At 10-13 months, the cube is grasped between the volar pads of the finger tip and the distal volar pad of the thumb. If the child repeatedly drops the cube in a matter of seconds, the grasp should be considered immature. • At 6 months, he can chew and feed himself with a biscuit, plays with his toes in supine position, enjoys playing with paper and there is a strong tendency for everything going to the mouth. • At 10 months, he can pick up small objects employing finger and thumb together. He goes for objects with index finger and can deliberately let go of the objects. Normal Development 15

• At 11 months, he spends long time putting objects in and out of the basket. By 1 year, he stops taking things to the mouth. • By 13 months, he can build a tower of 2 one-inch cubes. At 18 months, he turns 2 or 3 pages of a book at a time. The height of throwing objects on to the floor one after the other (termed “casting”) comes at this age. • By 2 years, he can put on his socks and turns over 2 or 3 pages of a book singly. • By 2 .5 years, he can thread beads. • By 3 years, he can fasten buttons, dress and undress himself, draw and paint and build a tower of 10 cubes.

Use of Eyes The neonate blinks at birth in response to sudden sound, movement or touching the cornea. He, as a rule, does not blink on approach of an object right at birth but does so subsequently. This is a protective response. Pupillary response begins in utero per se. At or after 28th week of gestation, he reacts to light. The preterm baby starts turning head to diffuse light at about 32nd or 36th week. A preterm baby of 26-30 weeks avoids bright light. Right at birth, the neonate exhibits visual perception, following a dangling ring held about 25 cm in front, within a range of 45 degree. This range increases to 90 degree by 4 weeks and to 180 degree by 3 months. Fixation at near objects is attained by 3 months. A 3-4 week baby watches his mother intently as she speaks to him, fixating at her face. By 4-6 weeks, he starts smiling at her as she speaks to him. In other words, he begins to “socialize”. He smiles at a face-sized card with 2 dots or, still more, with six dots. By 3 months, he fixes his eyes on mother’s breast. By 4 months, he fixes his eyes on a small brick of 1 cm or more size. This is termed “grasping with the eyes”. It is noteworthy that the neonate is incapable of integrating head and eye movements well. If his head is passively rotated to a side, his eyes lag behind. The so-called “doll’s eye reflex” disappears by 2-3 months. The neonate’s binocular vision appears at 6 weeks, fairly establishing itself by 4 months. Until 6 weeks, his eyes move independently. Hand regard, meaning that he watches his hand with interest while lying supine, appears at 12-20 weeks. The “hand regard” should be regarded as a sheer developmental pattern not requiring visual 16 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics stimulation. In support of this contention is the observation that it is encountered even in blind children. At 5 months, he shows excitement at the site of the “feed”. By 6 months, he bends back, adjusting his position, to watch what is of interest to him. It is not before 1 year of age that he can follow rapidly the moving objects.

Use of Ears Fetus is able to hear in utero. The fullterm neonate can certainly hear. He may respond to sound by a startle reflex, by crying, by quieting, by blinking or by a momentary catch in his respiration. • At 3-4 months, he starts to turn his head more obviously towards the source of sound. • By 6 months, he imitates sounds. • By 7 months, he responds to his name. • By 9-12 months, he knows the meaning of several words and names of the family members. Moreover, he learns to control and adjust his responses to sounds. • By the first year, he is able to localize a sound source as well as an adult.

GENERAL UNDERSTANDING (INTERPERSONAL AND SOCIAL SKILLS) In the very first few days, the neonate starts watching mother when she speaks to him, he becomes quiet, opens and closes his mouth and bobs his head up and down. • By 4-6 weeks, he starts smiling. • By 5-7 weeks, he starts vocalizing. • By 4-8 weeks, he imitates the movements of the mouth and tongue protrusion by the mother. • By 12 weeks, he evinces remarkable interest in the surroundings. • By 12-16 weeks, he anticipates that feed is being offered to him. • By 20 weeks, he smiles at his mirror image. • At 24 weeks, he stretches arms around when he sees his mother about to lift him. He smiles and vocalizes and enjoys watching his own image Normal Development 17

in the mirror. At about 6 months, he also imitates acts such as tongue protrusion or a cough and enjoys “peep-a-boo” game. • At 32 weeks, he reacts to the cotton wool swab by grasping his mother’s hand and pushing it away, responds to “No” and attempts to reach objects far too away from him. • At 40 weeks, he imitates “pat-a-cake” and “bye-bye”, repeats a performance laughed at and pulls mother’s clothes to attract her attention. • At 44 weeks, he cooperates with the mother by holding his arm for a coat and his foot for a shoe. • At 48 weeks, he starts anticipating movements in nursery rhymes and shows interest in books and understanding of words. • At 11-12 months, he laughs at an unusual act by the mother and understands a phrase such as “Where is your shoe?” • Once he enters the second year (after the first birthday), there is a further enhancement in his understanding • Which can be tested by pencil and paper, picture book cutout figures and picture cards with pictures of common objects.

Displeasure and Pleasure It is curious that all babies learn to say “no” before “yes”. Likewise, all babies learn to first express displeasure and later pleasure. The earliest sign indicating “pleasure” is that the crying neonate becomes quiet when picked up. When given feed, he exhibits his “pleasure” by splaying of toes and by their alternate flexion and extension. • At 6 weeks, the baby gives smile when he is spoken to. A week or two later, he begins to vocalize. • About 12 weeks, he sequels with delight and demonstrates his pleasure by movements of trunk, arms and legs and excitement. • At 16 weeks, he laughs loudly, plays with the rattle placed in his hand and smiles when pulled up to the smiling position. • By 4 months, he is ticklish and tends to laugh at the very sight of the approaching finger. • Around 6 months, he enjoys sitting, nursery rhymes and little games. 18 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

FEEDING/DRESSING As the neonate matures, his lips are able to make a tighter grip over the nipple, thereby reducing the swallowing of air (“wind”). • At 6 months, he can chew a biscuit. • After 6 months, he begins holding a spoon. On an average, at 15 months, he can feed himself with spoon though he is likely to poke his fingers into the feed, splitting much of it. • Around 15 months, he is able to manage a cup fully. • Managing a knife and fork comes by 2.5-3 years. • By 3 years, he can dress himself fully under supervision and needing help with difficult buttons. • By 4-5 years, he can tie his shoe laces.

Opening of Mouth Until the age of 4-5 months, the baby does not open his mouth even though nose is blocked.

Speech • At 6-7 weeks, he can vocalize, producing sounds like ah, uh, ch. Addition of consonants like m, p, b (when expressing displeasure) and j and k (when expressing pleasure) comes at 10-11 weeks. • By 12-16 weeks, he starts uttering gaga, ng, ah goo. • At 16 weeks, squeals with delight, laughs loudly and enjoys local play. • Between 3-6 months, he adds syllables ma, da, ka, der, erheh. • At 7 months, he combines consonants to say mumum and dadada and vocalizes with many high and low pitched tones. A nasal tone may become obvious. A tongue-lip activity too develops. • At 7 or 8 months, he vocalizes or coughs to attract attention. • At 8 months, he adds d, t and w. • At 9 or 10 months, he starts imitating sounds. • At 10 months, he says a single word with meaning, obeys orders, responds to nursery rhymes, plays pat-a-cake, communicates by waving bye-bye. • By 12 months, he imitates dogs, cows and clocks and may say 2-3 words with meaning. • At 15-18 months, he jargons, speaking in a language of his own. Normal Development 19

• By 21-24 months, he begins to join words spontaneously. • By 3 years, he is talking incessantly.

Bladder Control During neonatal period, micturition is purely a reflex act, occurring after the feed. Of course, the reflex can be conditioned at one month or so to enable him empty bladder when placed on the pot. • Between 15-18 months, he begins developing voluntary control. The infant first signals the mother when he has already wet the pants, then just about the time he is doing it, and finally when he is about to do so. • By 16-18 months, he can voluntarily pass urine, provided that he is offered the pot immediately. • By 18 months, he is reasonably dry by day. • By 2-2.5 years, he can pull his pants down and sit on the seat without help to micturate. • By 5 years, 90% babies are dry.

Bowel Control It is usual for the bowel control to develop before the bladder control. With the gastrocolic reflex being active, the neonate and the infant in the first 2 to 3 months tends to defecate after each feed. Though the reflex weakens by 4 months, it is only by 7 months that the bowel movements become irregular. Unlike the earlier months, now there is no relationship with the feed. Toilet training can be initiated at 10 months when the infant is in a position to sit comfortably with good control on the toilet seat. By 15-18 months, he can walk to the toilet seat. By 36 months, he can withhold and delay his bowel movement. While imparting toilet training, attitude of the caregivers should be relaxed.

Handedness Whether a baby is going to be left or right handed gets established by 2 years; infrequently it may linger on to 3-4 years. Handedness should be considered partly genetic and partly environmental. All negative traits associated with left-handedness are seemingly myths. A multitude of renowned personalities are on record as being left-handed. They include, to quote Illingworth, such celebrities as 20 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Holbein, Durer, Landseer, Ravel, Johann Sebastian Bach, Lewis Caroll and Thomas Carlyle. With the exception of Jimmy Carter, all Presidents of the United States of America over the past quarter of a century have been left-handed. Of late, the chain stands broken by George Bush who is right- handed. There is evidence that left-handedness often runs in families. In the British royal family, for instance, the Queen Mother, Queen Elizabeth, Prince Charles and Prince William are all left-handed. Table 2.1 gives key development milestone. Table 2.1: Key developmental milestones

Age Milestone

Gross Motor 3 months Head holding 5 months Sitting with support 8 months Sitting without support 9 months Standing with support 10 months Walking with support 11 months Crawling (creeping) 12 months Standing without support 13 months Walking with support 18 months Running 24 months Climbing stairs 36 months Riding tricycle Fine Motor 4 months Grasps a rattle or ring when placed in hand 3-5 months Hand regard hand 5 months Reaches out to an object and holds it with both hands (intentional reaching with bidextrous grasp) 7 months Holds objects with crude grasp from palm (palmar grasp) 9 months Holding small objects, like a pellet, between index finger and thumb (pincer grasp) 15 months Feeds self fully (if given a chance) Language 1 month turns head to sound 3 months cooing 6 months monosyllables (ma, ba) 9 months bisyllables (mama, baba) 12 months two words with meaning 18 months ten words with meaning 24 months simple sentence 36 months telling a story Contd... Normal Development 21

Contd... Age Milestone

Personal and Social 2 months Social smile 3 months Recognizing mother 6 months Smiles at mirror image 9 months Waves bye-bye 12 months Plays a simple ball game 36 months Knows gender

KEY LEARNING POINTS 1. There may well be lack of parallelism (dissociation) between different fields of development. A child may be slow in developing “grasp” and , yet, rather fast in learning to stand or walk. This is an important principle of development. During neonatal period, micturition is purely a reflex act, occurring after the feed. Of course, the reflex can be conditioned at one month or so to enable him empty bladder when placed on the pot. 2. Development begins right from conception, occurs in utero and continues after birth. 3. Development proceeds in a definite sequence in all children though its rate may vary from child to child. 4. Development in one field need not necessarily run parallel with another field. 5. Development by and large depends on the maturation of nervous system. Of course, non-availability of opportunity to carry out practice may prove a retarding factor. 6. There is a tendency for the generalized mass activity in the infant to give way to specific individualized responses as he grows older. 7. Development is in cephalocaudal direction. 8. The primitive reflexes have got to disappear before corresponding voluntary movement is acquired. • Toilet training can be initiated at 10 months when the infant is in a position to sit comfortably with good control on the toilet seat. By 15-18 months, he can walk to the toilet seat. By 36 months, he can withhold and delay his bowel movement. • Handedness gets established by 2 years though infrequently, it may linger on to 3-4 years. All negative traits ascribed to left- handedness are by and large myths. 22 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

REFERENCES 1. Coloson E, Dworkin P. Toddler development. Pediatr Rev 1997;18:224-242. 2. Illingworth RS. The Development of the Infant and Young Child, 9th edn. London: Churchill Livingstone, 1987. 3. Johnson C, Blasco P. Infant growth and development. Pediatr Rev 1997; 18: 224- 242. 4. Sturner R, Howard B. Preschool development . Part I: Communicative and motor aspects. Pediatr Rev 1997;18: 291-310 5. Sturner R, Howard B. Preschool development. Part II. Psychosocial behavioral development. Pediatr Rev 1997;18:327-336 RAP Special Volume 19: Developmental and Behavioral Pediatrics

Pediatric Psychopharmacology: 3 Recent Controversies

JR Ram

INTRODUCTION One of the exciting aspects in child and adolescent psychopharmacology is that over a period of a decade, accepted practices have changed quickly and drastically. These changes did not take place in a scientific vacuum, resulting simply from ‘‘new’’ and ‘‘better’’ evidence. In some cases, the evidence had been there and was effectively suppressed. In other instances, conventional practices were re-evaluated to establish more stringent safety norms. However, these re-evaluations regarding safety and efficacy of drugs are not limited to child psychiatry. Recently, we have seen rancorous re-evaluations of accepted wisdom on estrogens after menopause, cyclo- oxygenase-2 inhibitors and, of course, use of antidepressant medication in children and in adults.

BACKGROUND The treatment modalities in child psychiatric disorders have rapidly evolved over the past 15 years. The therapeutic option in child psychiatry was largely restricted to talking therapies after second world war but, from early 1990s, there has been a significant increase in the use of psychotropic drugs in children. This change has not been without its critics, both in scientific and lay press. Social scientists, psychiatrists and pediatricians have consistently raised concerns about ‘medicalizing’ childhood mental health problems. The concern against using drugs in children stem from the following facts: • A massive increase in the number of children being prescribed psychotropic drugs, especially in the USA 24 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• The majority of psychotropic drugs are not licensed for use in children • Indiscriminate use of psychotropic drugs in children can mask or ignore social origins childhood mental illnesses • The effect of labeling on a child. The following statistics highlight the increasing use of methylphenidate and the growth in the diagnosis of ADHD.1 One marker of the growth of this diagnosis and treatment are annual rates of stimulant production as monitored by the Drug Enforcement Administration (DEA) in USA. Methylphenidate production in USA remained relatively stable throughout the 1980s. But, between 1991 and 2000, the production of methylphenidate rose by 740%. This massive increase in drugs used for ADHD has justifiably raised concerns whether the increase in diagnosis and use of drug reflects a genuine clinical need or whether there is unnecessary medicalization and/or pathologizing of childhood behavior problems. The other group of drug which has been under intense scrutiny recently is specific serotonin reuptake inhibitors SSRIs. SSRIs are antidepressants and include drugs such as fluoxetine, paroxteine, fluvoxamine, citalopram and escitalopram. Questions regarding efficacy and safety of SSRI in children have been raised recently amongst intense media scrutiny in the Western world.2 In the following sections in this paper, the implications of recent research findings of these two groups of drugs, i.e. stimulants and antidepressants, in pediatric population will be examined in greater detail.

THE GROWTH IN PRESCRIPTION OF STIMULANT MEDICATION FOR ADHD—THE US SCENARIO In late February 2000, the Journal of the American Medical Association (JAMA) published an article on the use of Ritalin (methylphenidate) in toddlers3 along with a critical editorial, questioning the trend. Suddenly, in USA, Ritalin was on every evening and morning talk show, the subject of political cartoons showing mothers’ holding nursery bottles and Ritalin pills for their infants.4 Hillary Clinton raised her own voice of concern about using these medications in such young children without clear evidence of the benefits or safety. The response from the general public ranged from questions to condemnation of the use of these medications in children so young.5 Public outcry led to two separate government Pediatric Psychopharmacology: Recent Controversies 25 conferences in the ensuing 6 months. The toddlers-on-Ritalin controversy was a wake-up call. Overall, statistics suggest a trend of ADHD concentrated in the white middle- and upper middle-class suburbs.6 This breadth of community-based epidemiological data strongly implicates important ‘‘extraneurological’’ factors, primarily economic, involved in the diagnosis and treatment of ADHD. These factors have influenced decision-making regarding use of stimulant medication. Demands on children have increased. Children as young as 3 years of age are now expected to know their letters and numbers. Increased educational pressures begin in kinder-garten and continue through in school. Meanwhile, parents are less available to their children because economic pressures have more mothers working and both parents working longer hours. Economics also plays a role in health care in a variety of ways that increase the number of children on medication. The cost and unavailability of effective non-medication treatments, such as behavioral management training, also contribute to a medication-only approach. Finally, the pharmaceutical industry’s influence has been profound, both in determining the kinds of ADHD studies funded and published and in their drug promotions, advertising first to doctors and most recently, in USA, directly to consumers on television. Although ostensibly promoting awareness of ADHD as a public health benefit, a drug company’s advertisement in a woman’s magazine in USA, displays a beaming child holding a pencil surrounded by his happy family. This image directs parents to think of their child’s homework problems only as ADHD—a neurobiological disorder—as opposed to a more complex developmental/social undertaking.7, 8

LESSONS FOR INDIA It is unlikely that the growth in prescription for methylphenidate in India will replicate western statistics. The key reason being the lack of free availability of methylphenidate in our country. However, we cannot afford to be complacent because a new group of drug for treatment of ADHD, which is not a schedule X drug, is now available in India. The factors which have lead to a growth in prescription of methylphenidate in USA are equally applicable in an Indian setting, at least in the upper socioeconomic strata of the society. 26 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In the first half of 2006, stimulant medication again shot into the public limelight. The controversy began after extensive coverage by print and broadcast media of meetings of the Food and Drug Administration (FDA) Drug Safety and Risk Management Advisory Committee, USA. On February 9, 2006, the Advisory Committee voted 8 to 7 (with 1 abstention) supporting the use of a new “black box” warning in the labeling of pharmacotherapies for ADHD that would list sudden death and cardiovascular events as possible sequelae from their use.9 After these meetings, the New England Journal of Medicine released an electronic version on March 20, 2006, of an opinion piece by a consultant to that committee, Dr. SE Nissen, supporting the recommendation. This published version was followed by a print version in their April 6th issue.10 Dr. Nissen is the current president of the American College of Cardiology. For all currently marketed drugs for ADHD, Dr. Nissen urged ‘‘more selective and restricted use, while increasing awareness of potential hazards… .the FDA should act soon, and decisively.’’ As a respected cardiologist, his published views obviously had impact. However, the Food and Drug Administration disregarded the recommendation of the advisory committee and decided not to put a “black box” warning on Ritalin and other ADHD medications, opting instead to use stronger and, clearer language on the packaging so that parents can better understand the risks of using the drugs. Dr. Nissen was critical of the FDA decision and, in reaction to the FDA decision, said “It’s difficult to find physicians wiling to stand up to industry.” In conclusion, what this controversy means for all pediatricians is that a more careful evaluation and follow-up is needed before and after starting a child on methylphenidate. A therapeutic trial with methylphenidate can only be justified after a careful consideration of psychosocial factors and a comprehensive assessment. After the drug is started, increased awareness about the cardiovascular side effects and other rare side effects such as hallucinations and psychosis has to be borne in mind.

ANTIDEPRESSANTS IN CHILDREN: RECENT CONCERNS Depression has peak onset in adolescents and young adulthood. Sur- prisingly, very limited data was available for both psychopharmacology and psychotherapy specific to children and adolescents until very recently. Specifically with antidepressants, prior to 1995, data was only available Pediatric Psychopharmacology: Recent Controversies 27 for about 250 children and adolescents who had been in small double- blind randomized controlled trials (RCTs).11 Prior to 1997, there had been no published reports of any antidepressant being better than placebo for treatment of depression in childhood. However, only in the past 5–10 years has substantial data been collected and now the number of children on whom data is available is over 4400. The increase in numbers of children on whom psychopharmacological data is now available is the direct result of Food and Drug Administration (FDA) Modernization Act. The recent controversies about use of antidepressants hinges primarily around issues of efficacy and safety.12

INCREASE IN USE OF SSRI IN UK AND USA The use of selective serotonin reuptake inhibitors (SSRIs) in children under 18 years old increased tenfold (from 0.5 to 4.6 per 1000) in the UK from 1992 to 2001. Usage rates are even higher in the USA, at 16.6 per 10002.

TIMELINE OF THE GENESIS OF SSRI DEBATE The controversy about the safety and efficacy of antidepressants, particularly selective serotonin reuptake inhibitors (SSRIs) and newer antidepressants (selective norepinephrine reuptake inhibitors—SNRIs) began in June 2003, when Great Britain’s Medicines and Health Care Products Regulatory Agency (MHRA) received all the pediatric data (both depression and anxiety trials) for paroxteine submitted by GlaxoSmithKline (GSK).13 Great Britain’s Department of Health then issued a statement that Paroxteine should not be used to treat depressed youth under 18. The statement reported that paroxteine was not efficacious in treating depressed adolescents, and that it may cause increased suicidal behavior. Shortly thereafter, the US FDA issued a similar warning, but stated that they had not yet completed a full evaluation. Then, in August 2003, Wyeth Pharmaceuticals, maker of venlafaxine, distributed letters to physicians stating that venlafaxine was not recommended for treating depressed youth due to lack of efficacy and potential increased hostility and suicidal behavior. Great Britain’s Department of Health followed suit, stating that Venlafaxine should not be used in the pediatric population. In October 2003, the FDA issued a ‘talk paper’ stating that it was conducting investigations of antidepressants, including citalopram, 28 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics fluoxetine, fluvoxamine, mirtazapine, nefazodone, sertraline, and venlafaxine, but that at present, the data were insufficient to suggest that these medications caused increased suicidal or aggressive behavior in youth. The statement clearly advised physicians to monitor subjects for side effects, particularly increased suicidal risk, but did not unequivocally state there were data to support this concern. In December 2004, the MHRA stated: ‘The majority of SSRIs are not suitable to be used by under 18s year olds...[and that] risks outweigh benefits...” Only fluoxetine was considered to have an acceptable risk– benefit ratio.14 In March 2004, the FDA issued a statement requesting a warning label that recommended close observation of adult and pediatric patients treated with antidepressants for worsening of depression or the emergence of suicidality (fluoxetine, sertraline, paroxteine, fluvoxamine, citalopram, escitalopram, bupropion, venlafaxine, nefazodone, and mirtazapine) in all patients (children, adolescents, and adults). Finally, after a lengthy reanalyses of all suicide-related data from 26 RCTs (all disorders), in October of 2004, the FDA issued a “black box” warning describing an increased risk of worsening of depression and suicidality for all current antidepressants used in those under the age of 18.15 Of note, not all of the data from the 26 trials is available to the public, and no completed suicides have been reported in any of the RCTs of children and adolescents with these medications. Therefore, there is a subtle difference between UK (MHRA) and US (FDA) guideline.

CONCLUSION Over the past 3 years, serious concerns have been raised about the benefit- to-harm ratio of all antidepressants for children, leading to limits on the use of some drugs and warning statements on drug labelling. Whether this truly limits the use of antidepressants in under 18s in Indian setting remains to be seen. Factors which might compromise judicious use of antidepressants in children are heavy promotion of medication, distortion of the published data related to safety and efficacy, and underestimation by clinicians of the importance of the placebo response. Pediatric Psychopharmacology: Recent Controversies 29

KEY LEARNING POINTS • Pediatricians need to be aware of the recent concerns regarding adverse cardiac side effects associated with use of medicines used to treat ADHD. • The opinion, based on scientific data, regarding the safety and efficacy of SSRI in children and adolescents has changed drastically over the last 3 years. • Careful monitoring of patients under 18 who have been started on any SSRI is mandatory, especially with respect to emergence of suicidal ideations and worsening of depressive symptoms.

REFERENCES 1. Olfson M, Marcus SC, Weisman M, Jensen PS. National trends in the use of psychotropic medications by children. J Am Acad Child Adolesc Psychiatry 2002; 41: 514-521. 2. Tonkin A, Jureidini J. Wishful thinking: Antidepressant drugs in childhood. Br J Psychiatry 2005; 187: 304-305. 3. Coyle JT. Psychotropic drug use in very young children. JAMA 2000; 283: 1059– 1060. 4. Huget J. Scandal ! They haven’t tested Ritalin on the children it’s prescribed for! Washington Post 2001; January 2, PT06. 5. Goode E. Sharp rise found in psychiatric drugs for the very young. New York Times 2000; February 23: A1. 6. Zito JM, Safer DJ, dosreis S, et al. Racial disparity in psychotropic medications prescribed for youths with Medicaid insurance in Maryland. J Am Acad Child Adolesc Psychiatry 1998; 37:179-184. 7. Angell M. Is academic medicine for sale? N Engl J Med 2000; 342:1516–1518. 8. Zernike K, Petersen M. Schools’ backing of behavior drugs comes under fire. New York Times; 2001; August 19, p 1. 9. Food and Drug Administration. Drug Safety and Advisory Committee Meeting. February 9 and 10, 2006: Available at: http://www.fda.gov/ohrms/dockets/ac/ 06/briefing/2006-4202B1_05_FDA-Tab05.pdf. Last accessed on 30 July 2006. 10. Nissen SE. ADHD drugs and cardiovascular risk. N Engl J Med 2006; 354:1445– 1447. 11. Cheung AH, Emslie GJ, Mayes TL. Review of the efficacy and safety of antidepressants in youth depression. J Child Psychol Psychiatry 2005; 46: 735-754. 12. Healy D. Let Them Eat Prozac: The unhealthy relationship between the pharmaceutical industry and depression. New York: New York University Press 2004. 13. Hammad TA, Laughren T, Racoosin J. Suicidality in pediatric patients treated with antidepressant drugs. Arch Gen Psychiatry 2006; 63:332-339. 14. UK Medicine and Healthcare Products Regulatory Agency (MHRA). Report of the CSM Expert Committee Working Group on Safety of Selective Serotonin Reuptake Inhibitor Antidepressants 2004. Available at:http://www. mhra. gov. uk/home. Last accessed on July 30 2006. 15. US Food and Drug Administration. Public Health Advisory Suicidality in Children and Adolescents Being Treated with Antidepressant Medications October 15, 2004 Available at: http://www.fda.gov/cder/drug/antidepressants/SSRIPHA 200410.htm. Last accessed on 30 July 2006. RAP Special Volume 19: Developmental and Behavioral Pediatrics 4 Somatization Disorders

JR Ram

INTRODUCTION Practically everyday, the pediatricians see children with somatization, i.e. medically unexplained symptoms like headache, stomachache, and palpitations. Mostly, these symptoms are transient. However, in a significant proportion of children, these become entrenched and/or recurrent. In its narrowest sense, somatization is a term used to denote psychological difficulty or distress that is manifested through physical (somatic) symptoms.1 The child presents with a physical symptom but no organic condition is found which can explain the symptom or, even if a physical condition is present, it does not fully account for the severity and functional impairment.2 In a wider context, somatization is not only a disease entity but a process whose result is the illness experience of medically unexplained symptoms. Several terms have been used to describe this clinical problem— somatization, somatoform disorders, abnormal illness behavior, medically unexplained symptoms, and functional symptoms. In this paper, the terms “medically unexplained symptoms” and “somatization” are used interchangeably.3 One key feature of this group of disorders is that the child and the family are unable to establish a connection between physical symptoms with psychological distress, e.g. they do not see the connection between recurrent abdominal pain where no organic etiology is found and the stress of examinations. This leads to pursuit of medical treatment (consultation behavior) to seek an explanation and treatment of the distressing physical symptom. For the child, there is limitation of normal activities with adoption of sick role (illness behavior).1 Somatization Disorders 31

In somatization, production of these symptoms is not under conscious control or intentional. If the symptoms are thought to be produced deliberately, then it is known as “factitious disorder” or “malingering”. Factitious disorder is a separate disorder and is not under the purview of this review.

CLASSIFICATION The classification of this group of disorders is poorly organized and conceptualized. Wessely et al3 went as far as stating that the classification was a “mess”. The diagnostic categories which are described in DSMIV and ICD10 were formulated for an adult population.4 Therefore, using those in pediatric population is cumbersome. The key problems in the classification of medically unexplained symptoms are as follows.5 • Diagnoses are not based on positive criteria but on the exclusion of organic disease. • They are not supported by substantial empirical evidence. • They have been developed in highly selected adult patient populations. • They only include illness of at least 6 months duration, whereas in children most of these symptoms are short-lasting or transient. Adding to the confusion is the fact that different medical specialities have developed their own syndrome labels to classify patients presenting with medically unexplained symptoms for which no satisfactory physical cause is found, e.g. , chronic fatigue syndrome (CFS), recurrent abdominal pain (RAP), fibromyalgia, irritable bowel syndrome (IBS) and chronic benign pain syndrome, tension headache, non epileptic attack disorder, etc.

DIAGNOSTIC CATEGORIES AND CLINICAL CHARACTERISTICS In ICD-10, 6 there are two broad categories which describe medically unexplained symptoms. They are: 1. Dissociative disorders: They are also known as conversion disorder, previously known as “hysteria”. There are 10 subcategories in this group of this disorder. The common theme in these disorders is that there is a complete or partial loss of memory or control over bodily movements or loss of sensation. The symptoms presentation is usually associated closely in time with traumatic or upsetting events or 32 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

disturbed relationships. The onset and termination of dissociative disorders are mostly sudden. In pediatric clinical practice dissociative convulsions (pseudoseizures) and dissociative motor disorders (paralysis) are the commoner presentations. 2. Somatoform disorders: There are 7 subcategories respectively in this group of disorder. The key feature of this group of disorder is presentation with physical complaints. The physical complaint can involve any system of the body but common presentations include headache, abdominal pain, joint pain or low energy or a combination of the above. Application of specific diagnosis of somatoform disorder as described in ICD 10 is only occasionally possible in pediatric practice. ICD 10 mandates that the unexplained medical symptom/s be present for 6 months to 2 years. The majority of the presentations in common pediatric practice is usually of shorter duration.5

PREVALENCE Studies to elicit rates of medically unexplained symptoms in children and adolescents have been done both in community samples and in those attending pediatric primary care clinics. The general population rates are about one in ten. Children-complain of recurrent physical symptoms, the majority medically unexplained. This is a phenomenon seen throughout childhood, though certain symptoms may be more prevalent at different developmental stages, e.g. abdominal pains in preschoolers, headaches becoming more noticeable in older children.7,8 These general population rates are mirrored in pediatric primary care consultations. Campo et al.9 enquired about unexplained aches and pains amongst children attending pediatric primary care clinics: 13% were identified by pediatricians, 2% as ‘frequent’ somatizers (i.e. complaining ‘often’) and 11% as complaining ‘sometimes’.9

SPECIFIC SYNDROMES Pseudoseizures 2, 10 The most commonly reported conversion symptom in the recent child and adolescent psychiatric literature is pseudoseizure, Pseudoseizure is Somatization Disorders 33 an event that resembles a sudden convulsive event but exhibits no EEG evidence of a seizure and does not follow the typical pattern of a known seizure disorder. Although the convulsions of epilepsy may be difficult to distinguish from pseudoseizures, clinical distinctions have been proposed. More technologically sophisticated methods to distinguish seizures from pseudoseizures include video-EEG and measuring “postictal” elevations in serum prolactin levels. However, because the EEG presentation of epilepsy is variable, obtaining a “normal” EEG recording does not exclude the presence of a seizure disorder. Similarly, the presence of a documented seizure disorder does not exclude the possibility of a pseudoseizure.

Pain Syndromes The diagnosis of pain disorder requires positive evidence of the role of psychological factors, not merely the physician’s inability to explain a child’s pain on a purely organic basis.11 Positive evidence of the importance of psychological factors may include: 1. Onset of pain after specific adverse life event or in response to chronic stresses. 2. Disability or handicap out of proportion to reported pain. 3. Clear secondary gain from the pain. The notion that “real” pain can be differentiated from “psychogenic” pain by the placebo response is unhelpful, since all types of pain are often responsive and placebo has been shown to actually increase the levels of endogenous opioids in the circulation. A related clinical point of importance is that a child with organically caused pain but psychologically determined handicap may also need treatment.

Recurrent Abdominal Pain Recurrent abdominal pain (RAP) is a common and potentially disabling pediatric problem. RAP is commonly defined as three or more episodes severe enough to affect the child’s activities, occurring over a period longer than 3 months.12 Clinicians and researchers acknowledge that the most common etiology of RAP is unknown and likely to be functional in origin. It occurs in approximately 10% of children13,14 and accounts for a significant 34 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics proportion of primary and pediatric health care consultations and time missed from school.9 Decrease in daily function due to RAP may be significant; findings of increased school absenteeism have been reported as high as one day in 10 for patients with RAP.15 RAP is well studied from a psychological standpoint. Studies have reported increased symptoms of anxiety and depression in both the child with RAP and more interestingly in the mother, compared with healthy controls.16 Crowcroft et al 17 have reported an increase in somatization symptoms in parents of patients with RAP, while others have noted increased pain syndromes in family members of children with RAP.18,19 The frequency of reporting physical symptom increases across childhood into adolescence and female symptom reporting predominates. Most studies find no significant gender differences in physical symptom reporting prior to adolescence but an excess of somatic symptom reporting by females during adolescence.20,21

Prevalence of Psychiatric Illness in Children with Somatization There are high levels of mental health problems amongst children with unexplained medical symptoms. 1 in 2 children with high somatic scores have psychiatric disorders whereas the rate of psychiatric disorders in physically healthy children is 1 in 10.21 The psychiatric diagnosis, which is most commonly applicable to children and adolescents who present with medically unexplained symptoms, is “adjustment disorder”. Common presentations are usually mixtures of physical and psychological symptoms (anxiety, temper- tantrums and irritability) arising in response to adverse life events or stresses. The physical symptoms are typically headaches, stomach and joint pains, but may be in any system and multiple.22

IMPORTANCE OF RECOGNIZING SOMATIZATION DISORDERS Physical symptoms for which no satisfactory medical explanation is found are anxiety provoking for parents. This very frequently leads to doctor shopping and unnecessary medical investigations, hospitalization and school absence can ensue. Adoption of sick role in childhood can lead not only to skewed psychological development but may leave scars which impair functioning in adult life. Somatization Disorders 35

ETIOLOGY Psychosocial factors contribute to the development of somatization disorders in the child. A combination of interacting factors in the child and his/her family and environment give rise to the symptoms. It is important to note, however, that none of these factors are, on their own, either necessary or sufficient for such a presentation.21 The mediating influences of parents are also very important. Whether the child’s unexplained physical symptom is ignored or propels the parents to seek medical advice or allows the child to adopt a ‘sick role’ determines the expression of the disorder.24 There could be a wide range of precipitating and maintaining factors, in both child and family that may result in a clinically significant presentation. For a comprehensive understanding of the matrix in which the child’s symptoms have emerged, it is helpful to formulate the child’s presentation in terms of predisposing factors, precipitating factors and maintaining factors in the child, the family and the environment.21

Predisposing Factors Family • Limited in verbal communication about emotional issues, including conflict • ‘Conditional caretaking’, i.e. the culture in the family to look after the child only when she/he is sick • Parental history of somatoform illness, anxiety or depression • Problems with boundary setting for children • Suspicious attitude to medical expertise.

Child • Temperamental factors, including conscientiousness, emotional lability, vulnerability and worthlessness • Earlier emotional abuse • Low IQ • Social-relating difficulties.

Precipitating Factors Child • Anxiety, depression • Life stresses of all types – overt and covert 36 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Physical illness • Peer group problems • Academic problems and cognitive limitations • Low self-esteem.

Family • Life events/crises.

Maintaining Factors in Child, Parents and Professionals • Current family relationship difficulties and the predicament is resolved by symptoms • Family model of serious illness • Current parental mental illness particularly anxiety and somatization • School problems • Professional behavior that reinforces anxieties and sick role.

MANAGEMENT The first task for the clinician is to unravel why the child is presenting with symptoms suggestive of somatization now. Possible organic etiology needs to be excluded first, before entertaining this specific diagnosis. After the clinician is fairly confident that the child does not have organic ailments that account for his/her symptoms, openly communicating and reassuring the parents is crucial.25 The parents need to be told that the child does not have any life- threatening ailments but he does have problems in the emotional sphere, which is giving rise to the symptoms. It is important that the clinician does not convey this message in a pejorative or accusing manner, stating nothing is wrong with the child and she/he is “just acting”. Children with somatization experience genuine psychological distress, and undermining the child’s experience of anxiety is counterproductive.

Evidence Base of Management There is an embarrassing lack of data regarding what works in the management of somatization in Children. The presentations and context in which the disorder has developed are myriad in nature and ‘one size fits all’ kind of approach do not work. Somatization Disorders 37

In the following paragraphs, an attempt is made to highlight the evidence base of treatments of different kind of presentations and later a general approach to management of somatization is discussed.

Recurrent Abdominal Pain Despite the impact of pain syndromes in the child’s life, there is a paucity of evidence to guide clinical practice in this complex area. Pharmacological treatments are used, based usually on data extrapolated from adults. The evidence for effectiveness is limited. Helping the parents to see the link between psychological and physical pain is appreciated by them and is felt to be influential in making them manage the pain better.20,26 There is evidence (Level B) that cognitive-behavioral therapy may be useful in improving pain and disability outcome in the short-term.20

Headache and Migraine Tension headaches can be significantly reduced by relaxation training. In some, but not in all, relaxation training has been found to be superior to placebo in adolescents. The most encouraging aspect is that it is shown to be effective as home based self administered treatment strategy.20 A recent Cochrane systematic review identified 18 randomized controlled trials, of which 13 (12 trials of headache and one on the management of abdominal pain in children) provided data suitable for meta-analysis. The main finding of this review was that the number of patients needing to be treated to show benefit for psychological therapies producing more than 50% pain relief compared with control treatments was 2.32. This compares favorably with numbers needed to treat for other published treatments in chronic pain.27

Dissociative Disorders (Pseudoseizure, Motor Paralysis, etc.) In Indian setting, this is a very common presentation in children and adolescents. Evidence-based treatment strategies are sadly lacking and data is limited to case reports only.28 Available literature and clinical reports suggests that the possibility of sexual abuse should be borne in mind as abuse or sexual stresses are consistently reported in a minority of affected children. Most children improve dramatically in a short span of time. But, ingredients that are effective in achieving remission are not known. Close liaison between 38 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics pediatrician and psychiatrist is needed29 and physiotherapy, family work and above all an empathic approach to understand the child’s predicament facilitates recovery. Clinicians are needed to act as the child’s advocate and attempts to “shame” the child to give up his/her symptoms can be counterproductive and damaging.

Principles of Treatment This has several strands. The intensity and duration of intervention can vary from one off session to a short inpatient admission. Contact with school is very helpful.

Engagement Process and Arriving at a Clinical Formulation The child and the family expect a tangible physical cause to be found for the symptoms when they come to the pediatrician. To help the family from moving on from their search of an organic cause to something more intangible and in the psychological domain, needs patience and skill in gaining the confidence of the family/parents. The first task is, therefore, gaining the family’s confidence. Listening carefully to the history of the illness, its impact and management that has taken place so far, helps in understanding family illness beliefs and the level of conviction that the illness has a physical cause. Gauging the level of satisfaction with physical investigations and exploring whether the parents are happy with the explanations that have been offered to them so far are the next steps. The initial interview and the manner in which psychological issues are explored are keys to a successful forging of therapeutic relationship (Fig. 4.1). Some families remain anxious that undisclosed underlying physical disorder is as yet uncovered. Conveying the idea that lack of physical ailments does not mean lack of underlying physiological mechanisms for child’s symptoms is needed. Aim is to highlight the mind- body link and analogies are useful. Giving examples of tension headache, physical discomfort affected by psychosocial factors, e.g. asthma worsened by emotional distress or panic attack precipitated by fear helps the parents to understand the mind-body link. The clinician’s aim during this interview is to establish a formulation of the illness that incorporates preexisting vulnerabilities, precipitating (‘triggering’) events or illness and maintaining (‘secondary gain’) factors. Somatization Disorders 39

FIGURE 4.1: Patient-physician interaction in somatization23

Sharing this ‘predisposing, precipitating, maintaining’ approach to analysis with the family should follow once the assessment is completed. Parents usually can begin to think of triggers themselves once they gain an understanding of what could have led on to the symptom presentation. At this stage, stressing that recovery through rehabilitation can be achieved is crucial. Resume gradually, rather than avoid, activities normal for the developmental stage. This is crucially important. So, if the child has stopped going to school, plan and execute a school reintegration program. Encourage parents/care-taker to facilitate age appropriate activities which may have been stopped due to the child’s presenting symptoms. For the child, encouraging him/her to express emotional distress, underlying worries or fears through direct verbal means rather than through physical complaints needs to happen. Finally, address the triggering factor. This can range from bullying in school, parental over-expectation, one of chastisement of the child for bad behavior to marital conflict in parents or sexual abuse. Whatever is the trigger, it has to be addressed with the child and the family and often involving the school and key members of the wider community may also become essential.30 40 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

WHAT HAPPENS TO THESE CHILDREN WHEN THEY GROW UP? Eminson24 has summarized the available outcome data. Outcomes may be considered in relation to the future risk of: (a) psychiatric disorders or symptoms, (b) continuing unexplained physical symptoms It has become increasingly clear that the experience of excess or chronic physical symptoms in childhood and adolescence is associated with psychiatric disorders especially depression and anxiety) and in adulthood with excess unexplained hospitalizations.31,32 This is the case even when psychiatric symptoms were not apparently present in childhood. The results of different studies and also outcomes in boys and girls are inconsistent, reinforcing the need for more research and greater specificity in the comparisons made.33 Some data are available on the longer-term course of RAP. Children who complained of stomachaches at age 4 were three times as likely to have similar complaints on follow-up at age 10 than were non-complaining peers.34 Another study which followed up 132 children with recurrent abdominal pain for 5 years showed that approximately 1 in 7 children have poor outcome with respect to symptoms and functional impairments.35

KEY LEARNING POINTS • Medically unexplained symptoms in children and adolescents are common presentations in Pediatric practice. • However, the syndrome remains poorly researched and guidelines for management are not easily available. • A significant proportion of children exhibit marked functional impairment, psychiatric pathology and many have parents with anxiety, depression and somatization disorders. • A through psychosocial assessment of all children presenting with somatization is warranted and therapeutically addressing the factors contributing to the genesis of symptoms does lead to symptom resolution.

REFERENCES 1. Lipowski ZJ. Somatization: The concept and its clinical application. Am J Psychiatry 1988; 145:1358-1368. Somatization Disorders 41

2. Fritz GK, Fritsch S, Hagino O. Somatoform disorders in children and adolescents: A review of the past 10 years [Special article]. J Am Acad Child Adolesc Psychiatry 1997; 36 1329-1338. 3. Wessely S, Nimnuan C, Sharpe M. Functional somatic syndromes: One or many? Lancet 1999; 11; 354:936-939. 4. Peveler R, Kilkenny L, Kinmonth AL. Medically unexplained physical symptoms in primary care: A comparison of self-report screening questionnaires and clinical opinion. J Psychosom Res 1997; 42:245-252. 5. Fink P, Rosendal M, Olesen F. Classification of somatization and functional somatic symptoms in primary care. Aust N Z J Psychiatry 2005; 39:772-781. 6. World Health Organization Tenth Revision of the International Classification of Diseases and Related Health Problems (ICD–10). Geneva: WHO 1992. 7. Garralda, ME. Practitioner Review. Assessment and management of somatisation in childhood and adolescence: A practical perspective. J Child Psychol Psychiatry 1999; 40: 1159-1167. 8. Domenech-Llaberia MD, Jane C, Canals J, Ballespi S, EsparoG, Garralda ME. Somatic symptoms in nursery children: Prevalence and associations in a Spanish sample. J Am Acad Child Adolesc Psychiatry 2004;43: 598-604. 9. Campo JV, Bridge J, Ehmann M. Recurrent abdominal pain, anxiety, and depression in primary care. Pediatrics 2004; 113:817-824. 10. Bowman ES, Markand ON. Psychodynamics and psychiatric diagnoses of pseudoseizure subjects. Am J Psychiatry 1996; 153:57-63. 11. Dunn-Geier BJ, McGrath PJ, Rourke BP, Latter J, D’Astous J. Adolescent chronic pain: The ability to cope. Pain1986; 26:23-32. 12. Subcommittee on Chronic Abdominal Pain. Chronic Abdominal Pain. Pediatrics 2005; 115:370-381. 13. Apley J, Naish N. Recurrent abdominal pains: A field survey of 1,000 school children. Arch Dis Child 1958; 33:165-170. 14. Ramchandani, PG, Stein A, Hotopf M, Wiles NJ and the ALSPAC Study Team. Early parental and child predictors of recurrent abdominal pain at school age: results of a large population-based study J Am Acad Child Adolesc Psychiatry 2006 45; 6:729-736. 15. Campo JV, Di Lorenzo C, Chiappetta L, et al. Adult outcomes of pediatric recurrent abdominal pain: Do they just grow out of it? Pediatrics 2001; 108:E1. 16. Crane C, Martin M. Illness-related parenting in mothers with functional gastrointestinal symptoms. Am J Gastroenterol 2004; 99: 694-702. 17. Crowcroft NS, Strachan DP. The social origins of infantile colic: Questionnaire study covering 76,747 infants. BMJ 1997; 314:1325-1328. 18. Garber J, Zeman J, Walker LS. Recurrent abdominal pain in children: Psychiatric diagnoses and parental psychopathology. J Am Acad Child Adolesc Psychiatry 1990; 29:648-656. 19. Walker LS, Garber J, Greene JW. Somatic complaints in pediatric patients: A prospective study of the role of negative life events, child social and academic competence and parental somatic symptoms. J Consult Clin Psychol 1994; 62:1213- 1221. 20. Garralda E. The interface between physical and mental health problems and medical help seeking in children and adolescents: A research perspective. Child and Adolesc Mental Health 2004; 9:146-155. 21. Eminson DM. Somatising in children and adolescents 1. Clinical presentations and aetiological factors. Adv Psychiat Treatm 2001; 7: 266-274. 42 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

22. Taylor DC, Szatmari P, Boyle M H, Offord DR. Somatization and the vocabulary of everyday bodily experiences and concerns: A community study of adolescents. J Am Acad Child Adolesc Psychiatry 1996; 35: 491-499. 23. McDaniel S, Campbell T, Seaburn D. Somatic fixation in patients and physicians: a biopsychosocial approach. Family Syst Med 1989; 7: 5-16. 24. Eminson M. Somatising in children and adolescents. 2. Management and outcomes. Adv Psychiatr Treatm 2001; 7, 388-398. 25. Taylor S, Garralda E. The management of somatoform disorder in childhood. Curr Opin Psychiatry 2003; 16:227-231. 26. Garralda ME. Primary health care psychiatry. In: Rutter M, Taylor E (eds): Child Adolesc Psychiatry. Oxford: Blackwell 2002; 1090-1100. 27. Eccleston C, Malleson P. Managing chronic pain in children and adolescents BMJ 2003; 326: 1408-1409. 28. Chandrasekaran R, Goswami U, Sivakumar V, Chitralekha J, Hysterical neurosis: a follow-up study. Acta Psychiatr Scand 1994;89:78-80. 29. Garralda M E, Bailey D. Psychiatric disorders in general paediatrician referrals. Arch Dis Child 1989; 64:1727-1733. 30. Brown LK, Fritz GK, Herzog DB. Psychosomatic disorders. In: Wiener J, (ed): Textbook of Child and Adolescent Psychiatry. New York: American Psychiatric Press 1997:621-633. 31. Fearon P, Hotopf M. Relation between headache in childhood and physical and psychiatric symptoms in adulthood: National birth cohort study. BMJ 2001; 322: 1145-1148. 32. Hotopf M, Mayou R, Wadsworth M, et al. Temporal relationships between physical symptoms and psychiatric disorder. Results from a national birth cohort. Br J Psychiatry 1998;173:255-261. 33. Hotopf M, Wilson-Jones, C, Mayou R, et al. Childhood predictors of adult medically unexplained hospitalisations. Results from a National Birth Cohort Study. Br J Psychiatry 2000;176:273–280. 34. Borge AIH, Nordhagen BM, Botten G, Bakketeig LS. Prevalence and persistence of stomach ache and headache among children: Follow-up of a cohort of Norwegian children from 4 to 10 years of age. Acta Pediatr 1994;83:433-437. 35. Mulvaney S, Lambert EW, Garber J, Walker LS. Trajectories of Symptoms and Impairment for Pediatric Patients with Functional Abdominal Pain: A 5-Year- Longitudinal Study. J Am Acad Child Adolesc Psychiatry 2006;45:6,737-744. RAP Special Volume 19: Developmental and Behavioral Pediatrics 5 Eating Disorders

Shabari Dutta

INTRODUCTION The simplistic term, eating disorders, encompasses a complex group of psychiatric disorders with prominent physical, psychological and social dimensions. Differential diagnosis is important to distinguish one eating disorder from another, to assess psychiatric comorbidity and to rule out concomitant medical illnesses.

PLANNING TREATMENT PROTOCOL A treatment program that addresses each of the following must be planned: 1. Current nutritional status, 2. Abnormal eating behaviors and attitudes, and 3. The psychological meaning of the eating disorder for a particular patient. The DSM IV describes two eating disorders (anorexia nervosa and bulimia nervosa) which commonly begin in adolescence or young adulthood. Criteria for a new eating disorder called “binge eating disorder” appears in the Appendix B of DSM IV.1 Some screening questions for eating disorders:2 • What concerns do you have about your appearance and weight? • Is your current weight too high, too low or about right? • What is you ideal weight? • What are you worried about: gaining or losing weight? • How would you describe your appearance or weight? • What do you see when you look in the mirror? • What do your friends and family say about your looks and appearance? • What have you eaten today/yesterday/last week? 44 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• What kind of activities precede eating? • What were your thoughts/feelings while eating? • What are you doing to lose/maintain weight? • How much are you dieting/taking laxatives and other medications/ purging/binging/ exercising? • What kind of exercise do you do and how much? • When was your last menses? Have you missed any? • How often do you feel sad or worried recently? What were the circumstances? Some screening questions for interest in treatment: • What concerns do you have about your weight? • What have you tried about your weight problem? • What worked? Why? • What did not work? Why not? • What do you think would help this problem?

ANOREXIA NERVOSA Anorexia nervosa occurs in 0.5-1% of adolescent females in middle to upper socioeconomic groups in industrialized countries. Out of 20 cases of anorexia nervosa, only one is a male. Early recognition and treatment reduce the likelihood of a chronic and even life-threatening course. Physicians should be alert to children and adolescents who are losing weight but do not have appropriate concern for undue thinness. Anorexia nervosa has been defined by weight loss of at least 15% of the expected weight for height, age gender or by failure to gain weight so as to be within 85% of the expected weight. Along with a refusal to maintain body weight at a minimally normal weight, a disturbed perception of body image, i.e. an irrational fear of being too fat persistent weight loss in the absence of medical illness, questions related to fear of gaining weight and body image perception should be asked. Other consistent features include amenorrhea for three consecutive cycles. Boys with the disorder may have an analogous suppression of reproductive endocrine function. A decrease in gonadotropins leads to a decreased output of testosterone lack of sexual interest and a diminution of sexual functioning. In a small number of women amenorrhea begins long before sustained weight loss is evident but may persist even after normal weight has been restored, particularly if abnormal attitudes towards body size persisit.1 Eating Disorders 45

Physical findings include a reduction in vital signs (temperature, pulse and respiration) all reflecting the body’s need to conserve energy in the face of starvation.The patient may appear misleadingly healthy if weight loss has been gradual.

Differential Diagnosis Anorexia nervosa is not a diagnosis of exclusion. A patient who has lost a lot of weight for no apparent medical reason needs to be asked questions that specifically address characteristic attitudes of AN.

Other psychiatric conditions include:2 A. Severe depression in which appetite is truly lost B. Psychotic disorders, e.g. failure to eat owing to a persecutory delusion of being poisoned.2 Medical conditions include: A. Addison’s disease in which anorexia and weight los smay be accompanied by apathy and negativism.3 B. Crohn’s disease (regional enteritis) is often accompanied by symptoms of AN. Crohn’s disease patients show raised ESR. In AN, it is often less than 10. C. Hypotalamic lesions may occasionally mimic AN. Such patients often have headache and visual field cuts suggestive of raised intracranial tension. D. Turner’s syndrome (gonadal dysgenesis) in women has been associated with AN.4

Laboratory Anomalies Patients that binge eat and purge by emesis or laxative or diuretic abuse are prone to electrolyte imbalance. Low potassium can predispose individuals to cardiac arrhythmias. A prolonged QT interval on ECG may indicate the risk for sudden death. Low calcium with characteristic estrogen deficiency may result in bone thinning and even with subsequent weight gain patients maybe prone for stress fractures5 Low magnesium may be associated with cognitive difficulties. Severe hypoglycemia when glycogen stores are depleted can result in coma and death. 46 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Low phosphorus is an ominous finding. If carbohydrates are given too rapidly during the refeeding process, phosphorus sores can be depleted quickly with fatal outcome. Mild anemia may be caused by iron deficiency from malnutrition.

Low Albumin Reduced T3 and elevated reverse T3 reflects an adjustment to reduced calories and should not be treated other than attempts to improve patients caloric intake.

Treatment The treatment of anorexia nervosa needs to be nutritionally and psychologically driven. Dividing the treatment team into those concerned with nutrition and those concerned with psychotherapy is beneficial provided, that there is good communication between the two teams. An alternative approach uses family as a resource for recovery and puts the parents in charge of refeeding their affected child.

Goals of Nutritional Treatment2 1. To prevents the patient from starving. 2. To help the patient to slowly gain weight until she reaches the normal weight range. OPD management is the first choice. With the help of a dietician, an eating plan is worked out that brings weight loss to a halt and provides slow weight gain, approximately 200-400 g/day. Some patients may tolerate dietary supplements better than foods. Parents need to be encouraged to make food available but not get into struggles with their child.6 Weight monitoring, at least once a week, is necessary. Treatment team must take care not to be deceived by the patient. Patients often consume large amounts of water or conceal heavy objects in clothing before being weighed. Hospitalization becomes mandatory if weight loss persists despite therapy. This aids more intense supervision of eating and a behavior modification program may be implemented if necessary. Rewards such as visiting privileges, time out of bed or field trips can be made contingent on weight gain. Nasogastric feeding may be occasionally necessary, sympathetically with emphasis on nurturance. If patient’s life is threatened, parenteral alimentation may be necessary. However, this treatment carries Eating Disorders 47 the risks not only of sepsis but catastrophes related to rapid feeding such as depletion of phosphorus stores and heart failure Responsibility of adequate feeding rests with the treatment initially.

Goals of Psychotherapy 1. Identity formation 2. Increasing patients comfort with an age appropriate role. Identity being typically contracted, patients with AN are uncomfortable with the role of an adolescent or young adult, resulting in regression to a preadolescent stage. She feels that the only remaining way to feel effective in the environment is to control eating and weight. AN thus becomes the solution for the patient rather than the problem. Families sometimes discourage identity formation by failing to validate the patient’s ideas and feelings. Family therapy aimed at carving out an effective role for the patient is helpful. Marital conflict within he parents need to be addressed. Throughout individual, group and family sessions with the patient, the goal is to make the patient more assertive. Cognitive behavior therapy has been shown to be effective in posthospitalization care and relapse prevention7 A continuum of care, that includes partial hospitalization, day treatment and intensive outpatient treatment, may provide safe and effective care. Families can, thus, gradually assume responsibility for maintaining patients’ nutritional status in a healthy range.

Pharmacotherapy No specific medication, targets the irrational fear of weight gain. However, a retrospective study of treatment of AN with the atypical antipsychotic olanzapine, found significant reduction in anxiety, difficulty in eating and core eating disorder symptoms.8 A placebo controlled study indicates fluoxetine may help prevent relapse in patients with AN who are weight recovered.

BULIMIA NERVOSA Bulimia nervosa is more common than AN. It affects 3-4% of young women in middle to upper socioeconomic class but it is also seen in some males and adolescents. The discriminating features are recurrent episodes of “binge eating” with compensating behaviors to affect the large amounts of food that has 48 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics been consumed, so that the net effect is such that the patient is usually of normal weight. The disorder may be easily missed unless the physician specifically asks about eating habits and body image. Most research studies have focused upon the purging subtype in which binges are followed by self-induced vomiting, often concurrent to laxative or diuretic use to get rid of some of the calories. The nonpurging type is characterized by the patients fasting possibly with exercise as to compensate for the binge. Consistent features of BN include a sense of lack of control over eating, food craving and an absence of AN or a physiological condition that explain the symptoms, e.g. Klein-Levin syndrome. The most common physical finding is erosion of dental enamel resulting from the presence of stomach acid in the mouth particularly the upper teeth as lower ones are protected by the tongue during vomiting. If laxatives are used to assist purgation, electrolyte disturbances may occur, consisting of metabolic alkalosis and hypokalemia. The latter may predispose to cardiac arrhythmias. Emetic related toxicity can result in cardiac failure and death. Occasionally, violent vomiting can result in esophageal or gastric tears. Enlargement of salivary glands-giving a “chipmunk-like” appearance, probably results from recurrent vomiting . A telltale sign of BN is Russell’s sign, i.e. the presence of callous marks on the back of the hand produced by the upper teeth when the hand is pushed down the mouth to induce vomiting. It seems helpful to treat BN as a kind of addiction where food is the abused substance. Patients often recall the time that abnormal behaviors were no longer under their control, but rather had assumed a life of their own. The abnormal behavior is craved. The patient may in severe cases spend all day focused on the next indulgence in the activity. The behavior is often followed by a terrible sense of remorse and a firm intention to do better next time, and the patient experiences a sense of total failure when the behavior recurs. Outward physical signs are few, it is incumbent upon the primary care physician to ask about problems in the area of eating to facilitate early diagnosis. Patients are inclined to be secretive about the matter foregoing treatment for the concern that it might not be possible to exist without the disorder. Eating Disorders 49

Differential Diagnosis Binge eating and vomiting in bulimia are conscious voluntary activities. A. A brain tumor would produce vomiting in response to nausea and compulsive eating and would not be so episodic. Other neurological signs and personality changes may be evident. B. Kleine-Levine syndrome, typically fond in adolescent boys, in which episodes of uncontrolled eating and hypersexuality are associated with periodic hypersomnolence as a result of a hypothalamic lesion C. Prader-Willi syndrome, a genetic condition in which binge eating and obesity apparently resulting from an absence or deletion of the long arm of chromosome 15 D. Klüver-Bucy syndrome in which binge eating is associated with visual disturbances and sexual behavior alterations with diverse causes. E. Achalasia in which the esophagogastric sphincter fails to relax with swallowing and may be mistaken for bulimia. F. Insulin dependent diabetes mellitus and hyperthyroidism may predispose adolescents to bulimia.

Treatment Two types of interventions have been found useful in young girls 1. Cognitive behavior therapy Stage 1 substitutes a pattern of regular eating in place of habitual binge eating, self induced vomiting and laxative abuse. After a period of self-monitoring, the patient is provided with specific behavioral strategies that prevent overeating. Stage 2 involves identification and challenging of thoughts, beliefs and values that are perpetuating the eating problem. Stage 3 maintains progress and anticipates future difficulties. 2. Antidepressants tine, based on the premise of increased incidences of past personal and family history of depression Diagnostic criteria for depression may be met during assessment of BN, doses of fluoxetine may be as high as 60 mg for BN. A combination of antidepressants and cognitive behavior therapy may be especially helpful. Adolescents with BN who are brought for help by their parents may be very challenging because they are extremely ambivalent about their eating problem and working towards its resolution. Typically there are disagreements between parents and the child with 50 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics conflicting views about bulimia. It may be more productive to start with other differences that can be addressed in the family meetings that do not focus solely on the child’s eating behavior. The outcome of BN is that one-third subjects still continue to have clinically severe BN 5-10 years later.

KEY LEARNING POINTS • The term encompasses a complex group of psychiatric disorders with prominent physical, psychological and social dimensions. • Anorexia nervosa and bulimia are the two major eating disorders which usually begin in adolescence or young adulthood. Binge eating is a relatively new eating disorder. • Anorexia nervosa has been defined by weight loss of at least 15% of the expected weight for height, age gender or by failure to gain weight so as to be within 85% of the expected weight. • Differential diagnosis of anorexia nervosa includes other psychiatric conditions (severe depression) and medical conditions (Addison’s disease, Crohn’s disease, hypothalamic lesions, Turner’s syndrome). • Physical findings in anorexia nervosa include remarkable weight loss with a reduction in vital signs-temperature, pulse and respiration, all reflecting the body’s need to conserve energy in the face of starvation. • The treatment of anorexia nervosa needs to be nutritionally and psychologically driven. • Bulimia disorders with prominent physical, psychological and social dimensions. • Differential diagnosis of bulimia includes brain tumor, Kleine Levine syndrome, Prader Willi syndrome, Kluver Bucy syndrome, achalasia, insulin-dependent diabetes mellitus and hyperthyroidism. • Adolescents with BN who are brought for help by their parents may be very challenging because they are extremely ambivalent about their eating problem and working towards its resolution. • A combination of antidepressants and cognitive behavior therapy may be especially helpful. • Generally speaking, for management of eating disorders, a treatment program that addresses each of the following should be chalked out: 1. Current nutritional status 2. Abnormal eating behaviors and attitudes, and 3. The psychological meaning of the eating disorder for a particular patient. Eating Disorders 51

REFERENCES 1. American Psychiartic Association. Diagnostic and Statistical Manual of Mental Disorders, 4th edn. Washington DC: American Psychiatric Press 1994. 2. Waller DA. Eating disorders. In: Sexon BS (ed): Child and Adolescent Psychiatry, 2nd edn. Massachussets: Blackwell 2005;154-162. 3. Vardaraj R, Cooper AJ. Addison’s disease presenting with psychiatric symptoms(letter). Am J Psychiatry 1986;143:553-554. 4. Kron L Anorexia nervosa and gonadal dysgenesis: Further evidence of a relationship. Archiv Gen Psychiatry1977;34:332-335. 5. Mehler PS. Osteporesis in anorexia nervosa. Int J Eating Dis 2003;.33:113-126. 6. Lock J. Treating adolescents with eating disorders in the family context: Empirical and theoretical considerations. Child Adolesc Clin North Am 2002:11:331-342. 7. Pike KM. Cognitive behavior therapy in the post-hospitalization treatment of anorexia nervosa. Am J Psychiatry 1990;144:2046-2049. 8. Malina A. Olanzapine treatment of anorexia nervosa: A retrospective study Int J Eating Dis 2003.33:234-237. 9. Fairburn CG, Marcus MD, Wilson GT. Cognitive behavioral Therapy for Binge Eating and Bulimia Nervosa: A Comprehensive Treatment Manual. In: Fairburn CG, Wilson GT (eds): Binge Eating. New York: Guilford Press 2004;100-110. RAP Special Volume 19: Developmental and Behavioral Pediatrics

6 Adolescent Depression

Samir Parekh, Khyati Mehtalia

INTRODUCTION Major depression is common in adolescence and account for a significant proportion of all adolescent referrals and admissions for treatment. It has a wide array of symptoms affecting somatic, cognitive, affective, and social processes. Academic failure, poor peer relationships, behavioral problems, conflict with parents and other authority figures, and substance abuse are some of the consequences of major depressive disorder in this age group.1 As per DSM-IV-TR, major depressive episode is defined as a period of at least two weeks during which there is persistent depressed mood or diminished interest or pleasure in all activities and the additional symptoms include sleep disturbance, appetite or weight changes, psychomotor agitation or retardation, decreased energy, feelings of guilt or worthlessness, impaired concentration or difficulty in making decisions and thoughts of death. The only distinction between adolescent and adult criteria is the allowance of irritable mood or depressed mood in adolescent as compared to only depressed mood in adults.2 Also, the criteria of weight loss in absence of dieting can also be met in the young by failure to make expected weight gain. Dysthymic disorder is characterized by a chronic low-grade disorder and, in adolescents, the essential feature is persistent depressed or irritable mood for at least one year as against adults where the mood must be depressed and duration 2 years.2 EPIDEMIOLOGY A 2004 report from the American Academy of Child and Adolescent Psychiatry documented that suicide is the third leading cause of death for 15 to 24-year-olds, and the sixth leading cause of death for 5 to 14-year- olds. In fact, in 2001, nearly 4,000 teenagers between the ages of 15 and 24 killed themselves.3 Adolescent Depression 53

The point prevalence of major depression (DSM-IV TR) is between 1 and 6 percent for adolescents. Estimates of cumulative prevalence by the end of teens runs between 14 and 25 percent. The National Youth Risk Behavior Survey reported that almost one-third of high school students felt “so sad or hopeless almost everyday for at least two weeks in a row that they stopped doing some usual activities”.4 Depression is twice as common in girls as in boys. In a study amongst depressed adolescents in 14-16-year-group, girls outnumbered boys by a ratio of 5:1.5 In high school dropouts and institutionalized adolescents there are higher rates of mental illness.6

ETIOLOGY Recent scientific advances from neuroscience, genetics and clinical trials have demonstrated that the brain is the primary organ affected in depression. Modern brain imaging technologies have revealed neural circuits responsible for the regulation of moods, thought, sleep, appetite and behavior. There are documented changes seen in hypothalamic-pituitary- adrenal [HPA], hypothalamic-pituitary-thyroid [HPT] and hypothalamic- pituitary-growth hormone [HPGH] axes in depressed individuals.7 Recent work has delineated several neurotransmitter and other neurochemical systems that are involved in mood disorders, including systems that modu- late gene transcription. The biogenic amines norepinephrine, serotonin are most implicated in depression. Sleep abnormalities, like decrease in REM latency, are seen in depressed adolescents.8 In one study, circadian activity rhythms were found to be dysregulated in pediatric patients with major depression. These findings may have etiological significance.9 Mood disorders tend to cluster in families. Having one depressed parent doubles the risk and having two depressed parents quadruples a child having a mood disorder before 18 years as compared to the risk in children with unaffected parents.10 Genetics research indicates that vulnerabilities to depression often result from the interaction of multiple genes and environmental factors. Recent studies support passive gene environment correlate, i.e. a depressed parent, as a consequence of his or her genetic makeup, establishes an environment that is depression causing for his or her children with that genetic makeup.4 It has been theorized that aggression following loss of a loved object results in depression.11,12 The psychoanalytical model postulates “object loss” for depression.7 54 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Beck13 described distorted, negative thoughts as features of depressive individuals and this cognitive model was supported with research evidence.14, 15 Seligman and Peterson16 described learned helplessness as a factor for depression. Depressed individuals lack social skills needed to get positive reinforcement from others17 and that depressed adolescents had significantly more negative self concepts and low social self confidence.18

CLINICAL FEATURES Adolescents exhibit same core symptoms of major depressive disorder but there can exist important phenomenological characteristics which are typical of depression at this stage of life. Depressed adolescents are not always sad; they seem primarily irritated and unstable, and can have emotional outbursts and anger in their behavior. According to Kazdin and Marciano,19 more than 80% of depressed youths present irritable mood. In addition to depression, which is present in nearly all instances, symptoms occurring in more than two-thirds of cases include tearfulness, brooding about a past experience, feeling pessimistic, having difficulty concentrating, feeling worthless and fatigue. Weight and appetite changes affect three-fourths, with anorexia and weight loss being considerably more common than overeating and weight gain. Insomnia affects four-fifths and is most commonly experience when trying to fall asleep. Early morning awakening is less common.4 Another study reports that adolescents are more likely to have symptoms of anhedonia, hopelessness, hypersomnia, weight change and drug abuse, as compared to children.20 Other unique characteristics of this group are poor school performance, low self-esteem, suicidal ideation and attempts, and serious behavior problems, especially abusive alcohol and drug use. It has been indicated that even after recovery from depressive episodes, social skill deficits and psychological morbidity associated with it may continue to exist.21 Some authors such as Sadler22 and Baron and Campbell23 call attention to the difference between the manifestation of depression in female and male adolescents, emphasizing that girls report more subjective symptoms such as feelings of sadness, emptiness, boredom, anger and anxiety. Girls normally are also more concerned with popularity, less satisfied with their appearance, more self-conscious and have lower self-esteem, while boys report more feelings of contempt, defiance and disdain, and show conduct Adolescent Depression 55 problems like missing classes, running away from home, physical violence, robberies and substance abuse. They emphasize that alcohol abuse can be a strong indicator of depression. • As guidance for physicians, the following should not be considered normal • Alerting to the probable presence of depression during adolescence. • States of irritability or long-lasting and/or excessive depression • Prolonged periods of isolation from or hostility towards family and friends • Distancing oneself from school or a significant decline in school performance • Distancing oneself from group activities, and • Behaviors such as substance abuse (alcohol and drugs), physical violence, promiscuous sexual activity and running away from home.22 Migraine headaches which frequently begin during adolescence may be associated with shifts in mood, depression and irritability.24 More than 90% of youths committing suicide have a psychiatric disorder.7 Diagnosing major depression is considered an important preventive method for young suicides.25

COMORBIDITY Depressed adolescents normally present high rates of comorbidity with other psychiatric disorders, encountered more commonly than in depressed adults. Comorbidity with an anxiety disorder ranges from 20 to 50%. Studies suggest that not only is adolescent depression often preceded by a period of anxiety, but also that anxiety might return after depressive features abate.4 Comorbidity with one of the externalizing disorders is also common, with between one-third and one-fifth of teens with conduct disorder and a similar proportion among those with ADHD experiencing depression.4 Depression is often found to be comorbid with substance use. In presentation of mood disorders among adolescents, 20% of them present the symptom of drug abuse.26 56 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

TREATMENT The various psychosocial treatments for management of adolescent depression are cognitive, behavioral, psychodynamic, group, and family therapies.27 More than a dozen clinical trials have demonstrated that both cognitive- behavioral therapies (CBT) and interpersonal therapies (IPT) are effective modalities for adolescents with depression. Studies have also documented that nonspecific supportive psychotherapy, on the other hand, is not effective in reducing depression. Delivered by trained professionals, CBT and IPT are structured clinical interventions, that target specific factors associated with depressive thinking or behaviors. For example, active problem solving, social skills development, activity scheduling, and self- monitoring are often targeted and taught in these structured treatments. Nonspecific supportive psychotherapies, on the other hand, are unstructured “talking” therapies, often characterized by supportive listening, play or other nondirective activities primarily designed to provide emotional “support” to the individual.28 Over the past few years, there has been an increasing concern that antidepressants may increase suicidal thinking and behavior (not completed suicide) in depressed adolescents. On October 15, 2004, The United States Food and Drug Administration has issued a black box warning asking providers to use caution when prescribing antidepressants in children under the age of 18.29 Recent studies of antidepressant use in adolescents have demonstrated variable efficacy and an increased risk of adverse events, including suicidality. The evidence is greatest to support the efficacy of fluoxetine. Thus, it remains the only selective serotonin reuptake inhibitor approved by the United States Food and Drug Administration for the treatment of depression in children and adolescents.29 It is clear that, unlike adults with acute depression, the older antidepressants (e.g. tricyclic antidepressants) do not work well for childhood depression. Available studies have not found superior efficacy of TCAs over placebo for depression in this young population.30 The controlled published studies indicate that SSRIs are generally effective and well tolerated in the treatment of major depression in children and adolescents. The addition of unpublished data, however, suggested that the risks of most SSRIs (except fluoxetine) could outweigh the benefits because of little evidence of efficacy and possible risk of suicidal ideation Adolescent Depression 57 or attempts. Concomitant CBT may protect against development of suicidal ideation, though more studies of its effects are needed.31 Suicidality should be monitored carefully in every child and adolescent with depression, especially in high-risk patients and during initial antidepressant treatment.32 After starting an SSRI, the FDA recommends that clinicians see patients or their family members or caregivers weekly for the first month, every other week for the next 4 weeks, and again at 12 weeks. They may be seen as clinically indicated after 12 weeks (FDA website).29 Deaths have been reported in SSRI overdose cases, as well as the death of a child who undermetabolized fluoxetine.33 Antidepressant adverse effects such as disinhibition and impulsivity and manic switching require careful parental supervision and history and symptom clarification in clinical settings. Clinicians should also inquire at some length about bipolar family history and previous or present manic symptoms before prescribing antidepressants without concomitant mood stabilizers.34 Other adverse effects may be minimized by starting with lower doses. Common physical adverse effects in the studies on use of SSRIs in adolescent depression were dry mouth, vomiting, nausea, diarrhea, somnolence, insomnia, dizziness, tremors and agitation.31 The arguments that support prescribing antidepressants to adolescents with depression are: 1. Well studied efficacy of antidepressants in adult treatment 2. Evidence of continuity between adolescent and adult depression, and 3. Significant morbidity and mortality of adult depression.7 New research in the treatment of adolescent depression (i.e. the Treatment of Adolescents with Depression Study) demonstrates that the combination of CBT therapy and antidepressant medication (fluoxetine) results in successful treatment (71% of adolescents who were depressed responded positively to the combination treatment compared to 35% taking a placebo).28 In spite of the “black box” warning, the FDA has not taken a position that SSRIs and other new antidepressants are contraindicated in children and adolescents. Therefore, six medications (citalopram, escitalopram, fluoxetine, fluvoxamine, paroxetine and sertraline ; three others, including bupropion, mirtazapine, and venlafaxine); and MAO inhibitors and tricyclic antidepressants can continue to be prescribed for children and adolescents if rational prescribing principles are followed.28 58 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

KEY LEARNING POINTS 1. Major depression is common in adolescence and if not treated, may lead to devastating consequences in adult life. 2. Unlike adult depression it may primarily present with irritability, feeling, pessimistic, poor school performance, behavioral disturbance, drug abuse or suicidal ideation. 3. SSRIs, considered first-line treatment for adolescent depression, are generally effective and well tolerated, but should be used with caution because of the risk of increasing suicidal thoughts and behavior. 4. Pharmacotherapy should be accompanied by concomitant psychotherapy, mainly CBT or IPT.

REFERENCES 1. Sobti PC, Biswas G. Depression in adolescents. In: Gupte S, Bhave SY (Eds): Recent Advanced in Pediatrics (Special Vol 17: Adolescence), New Delhi: Jaypee Brothers 2006;205-223. 2. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th edn. Washington DC: American Psychiatric Association 2000. 3. National Center for Injury and Prevention Control. Suicide. Fact sheet. Accessed online January 12, 2004, at http://www.cdc.gov/ncipc/factsheets/suifacts.htm. 4. David S. Depressive disorders and suicide in children and adolescents. In: Sadock BJ, Sadock VA (eds): Kaplan and Sadock’s Comprehensive Textbook of Psychiatry, 8th edn. Philadelphia: Lippincott Williams and Wilkins 2005: 3262-3274. 5. Kashani JH, Beck NC, Hoeper EW, et al. Psychiatric disorders in Community Samples of adolescents. Am J Psychiatry 1987; 144: 584-589. 6. Whitaker A, Johnson J, Shaffer D, et al. Uncommon troubles in young people: Prevalence estimates of selected psychiatric disorders in a nonreferred adolescent population. Arch Gen psychiatry 1990; 47: 487-496. 7. Kashani JH, McNaul JP. Mood disorders in adolescents. In: Weiner JM, eds. Textbook of Child and Adolescent Psychiatry. Washington DC: American Psychiatric Press 1999. 343-385. 8. Dahl RE, Ryan ND, Williamson DE, et al. Regulation of sleep and growth hormone in adolescent depression. J Am Acad Child Adolesc Psychiatry 1992; 31: 615-621. 9. Teicher MH, Glod CA, Harper D, et al. Locomotor activity in depressed children and adolescents: I. Circadian dysregulation. J Am Acad Child Adolesc Psychiatry 1993; 4 : 760-769. 10. Saddock BJ, Saddock VA. Synopsis of Psychiatry ( 9th edn). Philadelphia: Lippincott Williams and Wilkins 2003:1274-1281. 11. Klerman GL, Weissman MM. Increasing rates of depression. JAMA 1989; 261: 2229- 2235. 12. Downey G, Coyney JC. Children of depressed parents: An integrative review. Psychol Bul 1990; 180: 50-76. 13. Beck AT. Depression: Clinical, Experimental and Theoretical Aspects. New York: Harper 1967. 14. Deal SL, Williams JE. Cognitive distortions as mediators between life stress and depression in adolescents. Adolescence 1988; 23: 477-490. 15. Garber J, Weiss B, Shanley N. Cognitions, depressive Symptoms, and development in adolescents. J Abnorm Psychol 1993; 102: 47-57. Adolescent Depression 59

16. Seligman M, Peterson C. A learned helplessness perspective on child depression: theory and research. In: Rutter M, Izard E, Read PB, (eds): Depression in Young Children. New York: Guilford 1986. 223-249. 17. Lewinsohn PM. A behavioral approach to depression. In: Friedman RJ, Katz MM (eds): The Psychology of Depression: Contemporary Theory and Research. New York: Wiley 1974;157-178. 18. Marton P, Connolly J, Kutcher S, et al. Cognitive social skills and social self- appraisal in depressed adolescents. J Am Acad Child Adolesc Psychiatry 1993; 33: 729-744. 19. Kazdin AE, Marciano PL. Childhood and adolescent depression. In: Mash E, Barkley R (eds): Treatment of Childhood Disorders, 2nd edn. New York: Guilford 1998: 100-105. 20. Ryan ND, Puig-Antich J, Ambrosini P, et al. The clinical picture of major depression in children and adolescents. Arch Gen Psychiatry 1987; 44: 854-861. 21. Rao V, Ryan ND, Birmaker B, et al. Unipolar depression in adolescents: Clinical outcome in adulthood. J Am Acad Child Adolesc Psychiatry 1995; 34: 566-578. 22. Sadler LS. 1991. Depression in adolescents. Context, manifestations, and clinical management. Nurs Clin North Am 26: 559-572. 23. Baron P, Campbell TL. Gender differences in the expression of depressive symptoms in middle adolescents: An extension of early findings. Adolescence 1993;28:903-911. 24. Fogel BS, Stone AB. Practical pathophysiology in neuropsychiatry: A clinical approach to depression and impulsive behavior in neurological patients. In: Yudofsky SC, Hales RE (eds): The American Psychiatric Press Textbook of Neuropsychiatry, 2nd edn. Washington DC: American Psychiatric Press 1992: 329- 344. 25. Bhatara VS. Early detection of adolescent mood disorders. SDJ Med 1992; 45: 75- 78. 26. Garland EJ, Weiss M. Subgroups of adolescent depression (letter). J Am Acad Child Adolesc Psychiatry 1995; 34:831. 27. McCraken JT, Cantwell DP. Management of Child and adolescent mood disorder. Child Adoles Psychiatr Clin North Am 1992; 1: 229-255. 28. Carpileno S. Children and Depression, 2005-2009 Statewide Comprehensive Plan for Mental Health Service Services 81-88 Available from http://www.omh.state.ny.us/ omhweb/statewideplan/2005/ Accessed last 29th July 2006. 29. Food and Drug Administration Website. Worsening depression and suicidality in patients being treated with antidepressants medications [online]. Available from URL: http://www.fda.gov/cder/drug/antidepressants. [Accessed 11th Apr 2006]. 30. Hazell P, O’Connell D, Heathcote D, et al. Efficacy of tricyclic drugs in treating child and adolescent depression: A meta-analysis. BMJ 1995; 310: 897-901. 31. Sharp SC, Hellings JA. Efficacy and Safety of Selective Serotonin Reuptake Inhibitors in the Treatment of Depression in Children and Adolescents, Posted 05/31/2006, Efficacy and Safety of Selective Serotonin Reuptake Inhibitors in the Treatment of Depression in Children and Adolescents, Posted 05/31/2006, Clinical Drug Investigation TM vol 26, no 2, available from http://www.medscape.com/ viewpublication/64_index 6 last accessed 29th July, 2006. 32. Wagner KD, Ambrosini P, Rynn M, et al. Efficacy of sertraline in the treatment of children and adolescents with major depressive disorder. JAMA 2003; 290: 1022- 1041. 33. Sallee FR, DeVane CL, Ferrell RE. Fluoxetine-related death in a child with cytochrome P-450 2D6 genetic deficiency. J Child Adolesc Psychopharmacol 2000 ;10: 27-34. 34. Sood AB, Weller E, Weller R. SSRIs in children and adolescents. Curr Psychiatry 2004;3:83-89. RAP Special Volume 19: Developmental and Behavioral Pediatrics Attention Deficit Hyperactivity Disorder in Adolescents: Evaluation and Psychological 7 Management Helen D Pratt

INTRODUCTION Attention-deficit disorder (ADD) or attention-deficit/hyperactivity disorder (ADHD) is a complex behavioral disorder that has symptoms that range from mild to severe. The impact of this disorder on the lives of affected youth can be minimal or so severe as to cause academic failure, as well as serious emotional problems. These adolescents are at increased risk of physical injury, psychosocial dysfunction, depressive and anxiety disorders, conduct disorder, and drug abuse. ADHD is noted in 3 to 9% of youth and adolescents, is three times more common in males than females, and is one of the most common of the psychiatric disorders that appear in childhood. Symptoms of ADHD usually become evident in preschool or early elementary years. The disorder frequently persists into adolescence and occasionally into adulthood. Youth with ADHD who receive help, guidance, and understanding from parents, guidance counselors, and the public education system can reach their full potential.1

DIAGNOSIS1 Effective treatment depends on appropriate diagnosis of ADHD. A comprehensive evaluation should include: a. A medical exam to rule out physical cause of symptoms or a disease process, brain trauma, infections or seizures b. And psychosocial to rule out, family distress, abuse, or trauma c. Behavioral assessment to measure presenting symptoms against age, developmental, and gender norms d. Psychological exam to rule out learning, intellectual, depression, post- traumatic stress disorders, psychosis, or other mental disorders. Ideally, Attention Deficit Hyperactivity Disorder: Evaluation and Management 61

a health care practitioner making a diagnosis should include input from both parents and teachers, and other care providers. The assessment should include questions about the adolescents ability function by exhibiting the following behaviors at home, school, and work: Compliance to socially appropriate behavioral norms, rules and values of his parents, school and community, show initiative, complete tasks, resolve conflict and engage in productive problem solving, demonstrate mental flexibility, be resilient, be on time, demonstrate endurance, establish and maintain friendships, engage in mutually beneficial interpersonal relationships, etc. Health practitioners who diagnose ADHD without all this information tend to either over diagnose the disorder or underdiagnose it.1, 3 Several resources for diagnosing ADHD have been published for clinicians. The most common are the Diagnostic and Statistical Manual of Mental Disorders, 4th edition, Text Revision (DSM-IV-TR),4 Classification of Child and Adolescent Mental Diagnosis in Primary Care, Diagnostic and Statistical Manual for Primary Care (DSM-PC), Child and Adolescent Version (DSM-PC)2 and the International Classification of Diseases, 9th revision: Clinical Modifications, 6th Revision.6 These manuals provide the criteria for mental health diagnosis and set the codes used by insurance companies to reimburse clinicians. Each of the above mentioned classification manuals follow DSM-IV- TR criteria for diagnosis. The impairments must a. Exhibit six or more symptoms of either inattention or hyperactivity- impulsivity b. Be present before age seven and c. Symptoms must be present in two or more settings.4 Behavioral assessment of the adolescents can be facilitated by the use of various parent—and self report-instruments designed to address the problems symptoms of attention and hyperactivity. These tools allow the clinician to gather much of this information in an efficient manner. Data from the adolescent, parents or care providers, and teachers should include the presenting symptoms, other related symptoms, and, possible co morbid conditions (Tables 7.1 to 7.3). Understanding why the family is seeking help at this point also provides clues regarding current stressors in the lives of the family. In addition to screening for ADHD symptoms, it is essential that the adolescent be screened for psychosis, mood disorders (anxiety and depression) and trauma.1 62 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 7.1: Other disorders that can cause inattention, hyperactivity, impulsivity Inattention • Mood disorders: Major depression [both manic and hypomanic episode of depression], dysthymic disorder; bipolar disorder; cyclothymic disorder • Pervasive developmental disorder • Schizophrenia, or other psychotic disorder • Dissociative disorder • Personality autistic disorder • Generalized anxiety disorder, separation anxiety disorder • Avoidant personality disorder • Substance abuse (cocaine phencyclidine, stimulants) • Mental retardation unless symptoms are in excess of those associated with MR Hyperactivity, and mental • Anxiety disorders (panic attack, agoraphobia, panic disorder without agoraphobia Specific and social phobias, acute stress disorder, post-traumatic stress disorder • Pervasive developmental disorder • Schizophrenia, or other psychotic disorder • Dissociative disorder • Personality autistic disorder • Generalized anxiety disorder, separation anxiety disorder • Avoidant personality disorder • Substance abuse (cocaine phencyclidine, stimulants) • Retardation unless symptoms are in excess of those associated with MR. Impulsivity • Impulse-control disorders • Pervasive developmental disorder • Schizophrenia, or other psychotic disorder • Dissociative disorder • Personality disorder • Autistic disorder • Separation anxiety disorder • Generalized anxiety disorder • Avoidant personality disorder • Substance abuse (cocaine phencyclidine, stimulants) • Mental retardation unless symptoms are in excess of those associated with MR. Reprinted with permission from Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics 2nd edn.New York: Universe 2006;107-142.

Educational Issues Physicians and parents should be aware that schools in the United States are federally mandated to perform an appropriate evaluation if an adolescent is suspected of having a disability that impairs academic functioning. This policy was recently strengthened by regulations implementing the 1997 reauthorization of the Individuals with Disabilities Act [IDEA],7 which guarantees appropriate services and a public education Attention Deficit Hyperactivity Disorder: Evaluation and Management 63

Table 7.2: Differential diagnosis for ADHD • Anxiety disorders (generalized anxiety disorder, separation anxiety) • Communication disorders • Conduct disorder • Impulse-control disorders • Learning disorders (mathematic disorders; reading disorders; • Written expression disorders) • Mental retardation • Mood disorders • Oppositional defiant disorder • Pervasive developmental disorders; autism • Psychotic disorders: Schizophrenia or other dissociative disorders • Personality disorders: Avoidant personality disorder • Substance abuse: Cocaine phencyclidine, stimulants • Tourette’s disorder • Miscellaneous medical conditions Reprinted with permission from Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics 2nd edn.New York: Universe 2006:107-142.

Table 7.3: Disorders that sometimes accompany ADHD • Learning disabilities • Tourette’s syndrome • Oppositional defiant disorder • Conduct disorder • Anxiety and depression • Bipolar disorder Reprinted with permission from Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics 2nd edn.New York: Universe 2006: 107-142. to youth with disabilities from ages 3 to 21. For the first time, IDEA specifically lists ADHD as a qualifying condition for special education services. If the assessment performed by the school is inadequate or inappropriate, parents may request that an independent evaluation be conducted at the school’s expense. Furthermore, some youth with ADHD qualify for special education services within the public schools, under the category of “Other Health Impaired.” In these cases, the special education teacher, school psychologist, school administrators, classroom teachers, and parents, must assess the adolescent’s strengths and weaknesses and design an Individualized Education Program. These special education services for youth with ADHD are available though IDEA.1 These assessments can be invaluable to the physician when making a diagnostic decision. Additionally, the physician’s diagnosis and documentation can 64 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics help the school determine if the adolescent is eligible for evaluation under IDEA. The diagnosis of ADHD should be made by a professional with training in ADHD or in the diagnosis of mental disorders. Child psychiatrists and psychologists, developmental/behavioral pediatricians, or behavioral neurologists are most often trained in differential diagnosis. Clinical social workers may also have such training.2,3 A referral to a psychologist is warranted if the physician suspects the adolescent has learning problems, has a comorbid disorder or does not achieve success with one or two medication adjustments. A referral to a child psychiatrist is warranted if the adolescent is suspected of also having a manic, depressive or bipolar disorder or in the adolescent exhibits symptoms of psychosis. Clinicians are reminded that assigning a label of ADHD is a serious diagnosis that can have adverse consequences for youth when they reach adulthood. Clinicians should be aware that there are some careers that may be off limits to even the most accomplished youth who have the mention of ADHD in their medical files. For example, very gifted individuals who have been diagnosed with ADHD during early childhood and taken medication to treat the disorder, then “outgrow” the symptoms and do not take medications during adolescence can be denied acceptance into high level military careers or any other career where deficits in attention and impulsivity are considered as undesirable characteristics. Very few clinicians envision this type of negative outcome for their patients, especially when their interventions are meant to improve the health care and quality of life for their patients.1

Symptom2,3 The principle characteristics of ADHD are inattention, hyperactivity, and impulsivity. Symptoms of ADHD appear over the course of many months with symptom presentation varying by setting, demands of the situation and intellectual abilities of the adolescent. Those youth who have above average intellectual functioning may be able to employ other skills to compensate for deficits in attention, self-regulation and self-control. An adolescent who has ADHD, combined type or with hyperactivity may be disruptive or engage in inappropriate behaviors while the adolescent ADHD primarily inattentive type may daydream, or struggle quietly with focusing on his or her tasks. The former often comes to the attention of Attention Deficit Hyperactivity Disorder: Evaluation and Management 65 teacher at school, parents at home, and adult authority figures in other settings. The impulsive adolescent who acts before thinking may be considered just a “discipline problem,” while the adolescent who is passive or sluggish may be viewed as merely unmotivated and overlooked by same settings. When symptoms affect performance in school, social relationships with other youth, or behavior at home, ADHD may be suspected. But because the symptoms vary so much across settings, ADHD is not easy to diagnose. This is especially true when inattentiveness is the primary symptom. According to the most recent version of the Diagnostic and Statistical Manual of Mental Disorders 2 (DSM-IV-TR), there are three patterns of behavior that indicate ADHD. People with ADHD may show several signs of being consistently inattentive. They may have a pattern of being hyperactive and impulsive far more than others of their age. Or they may show all three types of behavior. This means that there are three subtypes of ADHD recognized by professionals. These are the predominantly hyperactive-impulsive type (that does not show significant inattention); the predominantly inattentive type (that does not show significant hyperactive-impulsive behavior) sometimes called ADD—an outdated term for this entire disorder; and the combined type (that displays both inattentive and hyperactive-impulsive symptoms). Hyperactivity-Impulsivity Hyperactive youth always seem to be “on the go” or constantly in motion. They dash around touching or playing with whatever is in sight, or talk incessantly. Sitting still at dinner or during a school lesson or story can be a difficult task. They squirm and fidget in their seats or roam around the room. Or they may wiggle their feet, touch everything, or noisily tap their pencil. Hyperactive teenagers may feel internally restless. They often report needing to stay busy and may try to do several things at once. Impulsive youth seem unable to curb their immediate reactions or think before they act. They often blurt out inappropriate comments, display their emotions without restraint, and act without regard for the later consequences of their conduct. Their impulsivity may make it hard for them to wait for things they want or to take their turn in games. They may grab an object from another adolescent or hit when they’re upset. They may impulsively choose to do things that have an immediate but small pay off rather than engage in activities that may take more effort yet provide much greater but delayed rewards. 66 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Some signs of hyperactivity-impulsivity are: a. Feeling restless, often fidgeting with hands or feet, or squirming while seated; b. Running, climbing, or leaving a seat in situations where sitting or quiet behavior is expected; c. Blurting out answers before hearing the whole question; d. Having difficulty waiting in line or taking turns.

Inattention Youth who are inattentive have a hard time keeping their minds on any one thing and may get bored with a task after only a few minutes. If they are doing something they really enjoy, they have no trouble paying attention. But focusing deliberate, conscious attention to organizing and completing a task or learning something new is difficult. Homework is particularly hard for these youth. They will forget to write down an assignment, or leave it at school. They will forget to bring a book home, or bring the wrong one. The homework, if finally finished, is full of errors and erasures. Homework is often accompanied by frustration for both parent and adolescent. The DSM-IV-TR gives these signs of inattention: a) Often becoming easily distracted by irrelevant sights and sounds; b) often failing to pay attention to details and making careless mistakes; c) rarely following instructions carefully and completely losing or forgetting things like toys, or pencils, books, and tools needed for a task; and d) often skipping from one uncompleted activity to another.4 Youth diagnosed with the predominantly inattentive type of ADHD are seldom impulsive or hyperactive, yet they have significant problems paying attention. They appear to be daydreaming, “spacey,” easily confused, slow moving, and lethargic. They may have difficulty processing information as quickly and accurately as other youth. When the teacher gives oral or even written instructions, this adolescent has a hard time understanding what he or she is supposed to do and makes frequent mistakes. Yet the adolescent may sit quietly, unobtrusively, and even appear to be working but not fully attending to or understanding the task and the instructions. These youth don’t show significant problems with impulsivity and overactivity in the classroom, on the school ground, or at home. They may get along better with other youth than the more impulsive and Attention Deficit Hyperactivity Disorder: Evaluation and Management 67 hyperactive types of ADHD, and they may not have the same sorts of social problems so common with the combined type of ADHD. So often their problems with inattention are overlooked. But they need help just as much as youth with other types of ADHD, who cause more obvious problems in the classroom.1

PSYCHOLOGICAL MANAGEMENT1, 3 Most experts agree that that management of ADD/ADHD should include a multi-modal approach, involving appropriate educational interventions, psychological management of the patient, family focused interventions, combined with pharmacological management. This multi-modal approach must include the active involvement of the patient, parents, school officials, social workers, psychologists, and physicians (primary care and behavioral pediatricians or psychiatrists). These clinicians must work together to help all youth diagnosed with ADD/ADHD achieve their maximum potential The literature on treating ADHD describes a wide variety of psychological treatments used to treat the symptoms and problems associated with youth diagnosed with ADHD: psychotherapy, cognitive- behavior therapy, support groups, parent training, educator/teacher training, biofeedback, meditation, and social skills training. The empirical evidence regarding these interventions is uneven, ranging from no data to well-controlled trials. 1-3, 8-10 However, some researchers contend that youth who actually receive these treatments may be in the minority of those with ADD/ADHD.10

Treatment Modalities Psychotherapy This therapeutic modality is designed to help those with ADHD learn to like and accept themselves despite their disorder, to understand their strengths as well as weaknesses, and to develop strategies to minimize the negative impact of their disorder. In psychotherapy, youth talk with their therapist about upsetting thoughts and feelings, explore self-defeating patterns of behavior, and learn alternative ways to handle their emotions. As they talk, the therapist tries to help them understand how they can change. However, those dealing with ADHD usually want to gain control of their symptomatic behaviors more directly. If so, more direct kinds of intervention are needed.2,3,9 Psychotherapy is most effective with 68 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics individuals who are of average to above average intelligence and who have well developed abstract thinking and communication skills. Young youth, school age youth, pre-adolescents, and youth who are in the early stage (11-13 years of age) of their adolescent development generally do not have well developed abstract thinking and communication skills.1 Behavior therapy (BT) The focus of this treatment modality is to teach authority figures or patients how to increase appropriate (wanted behavior) and decrease inappropriate (unwanted behavior) by manipulating the patient’s behavior and environment. 2,3, 9-11 BT generally requires these major components: a functional analysis of the problem, contingency management, maintenance measures, and generalization procedures that include functional analysis of contingency management, self-management. 12, 13 Functional analysis During a functional analysis of the problem several steps are employed: Identification of problem behavior, determining the antecedents, consequences and maintaining variables controlling the behavior; selecting or targeting behavior to be changed; identifying and selecting potential rewards and punishers; goal setting, developing specific criteria for determining when goals are met; identifying consequence [rewards and punishers], fading or thinning of consequence to maintain gains, and planning for the termination of the program.1-3 Contingency management Contingency management is another essential component of behavior therapy. Interventions are delivered in classroom and home settings. Contingency management involves the development a detailed reward and punishment system where techniques such as token economies, timeout, and response cost might be employed. An effective way to modify a child’s behavior is through a system of rewards and penalties. Parents or teacher identify a few behaviors they consider desirable (appropriate and adaptive) and want to encourage in the child— such as prosocial behavior. They then structure situations in ways that will allow their child/adolescent to engage in those behaviors and set the stage for them to succeed. The parent or teacher works to keep the child/ adolescent from becoming over stimulated. The tasks are divided into small steps and then small successes are praised. Major objectives are rewarded until the child/adolescent achieves the major goal—the desired response/behavior. Parents and teachers use behavioral techniques Attention Deficit Hyperactivity Disorder: Evaluation and Management 69

(strategic attending, active ignoring, prompting, instructive feedback, pairing delivery of rewards with genuine praise delivered in a warm tone of voice and varying the content verbal praise that is appropriate to the developmental level of the child) during the structured situations. Parents and teachers learn to deliver consequences immediately following either appropriate or inappropriate behavior. Youth are rewarded for rule following and other prosocial behavior. An array of techniques ( e.g. time out, “quality time” each day, in which parents or teachers share a pleasurable or relaxed activity; “catching them being good” where parents and teachers look for opportunities to notice and point out what the child/ adolescent does well, and praise his or her strengths and abilities) are all part of reinforcement and punishment procedures.1-3

Self-management Individual patients/students are taught to use behavioral techniques to increase their awareness and control of their own behaviors (e.g. self-monitoring, verbal self-instruction, problem-solving strategies, self-reinforcement, self punishment, and self evaluation). The techniques described above are generally taught using teacher, parent, or patient/student training with an individual or group in school, home, or office settings. Research supporting the efficacy of behavioral therapy has existed for several decades. Intensive direct interventions in youth with ADHD have produced improvements in key areas of functioning, such as controlling activity levels, decreasing impulsiveness, paying attention, and focusing on required tasks.2,3 When BT components are included in treatment, families and teachers reported somewhat higher levels of satisfaction.1-3 Cognitive-behavioral therapy (CBT) As a treatment modality, CBT is designed to help youth work on immediate issues and employs interventions that are intended to directly change behavior. CBT combines components of psychotherapy and behavior therapy to help people rethink and restructure how they feel and think about their actions to initiate behavior change.2, 3 Youth learn how to think through tasks and organize work as well as use such techniques as problem-solving, planning, and time management. Therapists encourage new behaviors by using contingency management procedures to teach an individual how to control fighting, impulsive behaviors, anger, and frustration. Cognitive-behavioral treatment has not been found to yield beneficial effects in youth with ADHD. There is little empirical support for the use of CBT to treat youth 70 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics with ADHD. Research shows that cognitive therapies often create greater demands on the target youth than on their significant others, requiring not only that they participate in and contribute to training sessions but also that they apply the tactics learned across settings and activities, thus making empirical study difficult.2,3,10 CBT interventions are often labor intensive; when one considers the fact that several BT components are often combined with CBT in the treatment of ADHD, the labor intensiveness of these approaches becomes even more apparent.14 Biofeedback This treatment modality combines physiological, cognitive, and behavioral techniques to teach an individual how to control physiological responses to stressors as well as contract and relax muscles that increase or decrease tension, anxiety, and stress. Biofeedback is a controversial treatment and has not been scientifically shown to be effective in treating the majority of youth or adults with ADHD.2,3 One research study found that biofeedback (used to teach control of muscles involved in urination by responding to physiological cues) is an effective treatment for urination and bowel problems in youth with ADHD.1,15 Psychosocial treatments Treatments that address social, emotional, and behavioral functioning are labeled as psychosocial interventions and include social skills training, support groups, parent training, teacher training and peer mediation.1 Social skills training is often used as a component of treatment packages as a means of teaching or increasing a child’s prosocial behavior. In social skills training, modeling of prosocial behaviors is provided for the learner. These behaviors include such tasks as waiting for a turn, sharing toys, asking for help, or responding to teasing. The child or adolescent is then required to imitate the appropriate models and discuss why such behavior is acceptable. Next a discussion regarding models of antisocial behaviors occurs. The child or adolescent is also asked to explain why the inappropriate models are not acceptable. Also, participants are given chances to practise and receive corrective feedback. Trainers then use contingency management techniques to slowly help the student engage in prosocial behavior in relevant situations. Social skills training helps these youth and adolescents learn to join in group activities, make appropriate comments, ask for help and possibly learn to view how personal behavior affects others as well as develop new ways to respond when angry, pushed, frustrated, scared, afraid or sad.1-3,9 Attention Deficit Hyperactivity Disorder: Evaluation and Management 71

Social skills training programs are most effective if they have goals of teaching skills of social significance, using motivational systems to reduce performance problems, increasing awareness as well as understanding of verbal and nonverbal social cues (via didactic, role play, and video feedback), and promoting generalization and maintenance by incorporating significant others.16 Medication and behavioral treatment may improve different aspects of social impairment (function and normalization) and may complement each other when used together. Social skills training is most effective when youth and adolescents learn to use their new skills across the settings in which they live and under a variety of conditions.16,17 However, the positive outcomes do not hold true for helping youth diagnosed with ADHD develop social competence. However, social skills training is especially difficult for youth diagnosed with co-morbid emotional disturbance, mental retardation, and specific learning disabilities. Based on their analysis of the literature, some scholars have concluded that social skills training has not produced large, socially important, long-term, or generalized changes in social competence of these youth.18 Social skills training groups for youth use informal social activities. During these sessions the leader outlines the session plan, reviews home work successes, conducts large group discussion of homework and shows the large group video taped models of inappropriate and appropriate coping. Next, the group is divided into subgroups, which are charged with formulating solutions to the video taped coping models. Subgroup members discuss proposed solutions and then the large group reconvenes to brainstorm applications. Dyads are then formed to rehearse strategies after which the large group reconvenes and processes learned information. Prior to closure, group members are assigned homework and the leader then closes the session. Studies supporting the efficacy of these groups are limited.15 Parenting training is designed to provide participants with tools and techniques for managing their child’s behavior.1-3 Parents are taught about child development, child management skills, concepts of ADHD, and associated behavior or learning problems youth might face. Contingency management and techniques for coping with having a child diagnosed with ADHD are taught to improve their effectiveness as parents and subsequently improve the quality of life for their families. Specific issues addressed during this training include: identifying specific information 72 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics on why youth misbehave, how to pay attention, how to increase compliance and independent play, token economies, punishment (i.e. time out), and anticipating problems such as how to manage youth in public places. Additionally, parents are taught techniques for rewarding positive school behaviors (i.e., using the daily school behavior report cards to identify positive school behavior), and how to handle future behavior problems. Follow-up sessions are conducted to trouble shoot and support parents combined with follow-up parent meetings.1-3 This form of therapy is also useful as an adjunctive therapy or a primary form of therapy for parents who do not wish to have their youth take stimulant medications. However, it works best with parents who are mentally healthy, have a healthy marriage, and share similar beliefs about how youth should be raised. The program can be adjusted to meet the needs of parents who have limited intellectual functioning or are undergoing concurrent psychotherapy.1-3 Cunningham15 also concludes that community-based parent training is especially useful for parents of youth ages 4 -12 years. They frequently employ the use of social learning, cognitive and social psychology, family systems theory, and group theory. Sessions include an introduction (facts about ADHD and structure of the group) and lectures on miscellaneous topics, including attending and balancing attending among siblings, rewards, planned ignoring, transitional warnings, point systems, planning ahead, time out from positive reinforcement, problem solving and monthly booster groups.15 Robin18 describes a parent training model from a bio-behavioral family- systems approach. He states that fostering realistic beliefs and expectations is a very important starting point in treating adolescents diagnosed with ADHD. Psycho-education, cognitive restructuring, setting realistic expectations for the adolescent’s behavior and collaboration with the adolescent (as well as with the teachers) are all key skills parents need in order to improve their parenting effectiveness. Parents are taught principles of parenting the adolescent diagnosed with ADHD. Other researchers teach additional strategies that involve stress management, employing techniques such as meditation, relaxation techniques and exercise. This is designed to help parents increase their own tolerance for frustration and respond more calmly to their child’s/adolescent’s behavior. [National Institute of Neurological Disorders and Stroke, 2001]. Parent Attention Deficit Hyperactivity Disorder: Evaluation and Management 73 training is an effective method to teach positive parenting and to teach parents how to control family stress. 21

Teacher training Based on their research and literature review, Pfiffner and Barkley 22 concluded that teachers are most effective when they involve youth by teaching them self-management techniques (such as recording their own appropriate or inappropriate behaviors), combined with teacher observation and feedback on the accuracy of the child’s self-monitoring/ teacher monitoring recordings. Teachers are taught to use tangible rewards (tokens, check marks), visual aides, lotteries and auctions to motivate appropriate behavior. Individual, team and class contingences such as class movies and theme parties can be used as rewards for good behavior. Negative consequences (such as reprimands, corrective statements, time out and suspension in and out of school) can be used as punishers. Caution must be taken to minimize the effects of aversive consequences by using them sparingly, as well as teaching and rewarding alternative behaviors. Corporal punishment is not recommended as a method to control the student.23 It is important to promote the maintenance and generalization of appropriate behaviors across different settings by teaching youth self- monitoring, self evaluation and self reward. It is also helpful to reward positive behavior with verbal compliments and smiles. Use of peer interventions to encourage attention to positive prosocial behavior can be effective. Peers must have ability and motivation to learn this program and be able to accurately implement the program; however, they should not be included in implementing its punishment portion. Home-based interventions should direct parents to reward prosocial behaviors, rule following, and positive academic performance (i.e. completing assignments, playing well with peers and following rules at school). The authors further state that the success of the teacher implemented intervention programs requires a clear, consistent system for translating teacher reports into consequences at home.22 It is suggested that this approach is also effective for adolescents.

School based interventions According to the National Institute of Neurological Disorders and Stroke [NINDS], treatment of youth diagnosed with ADHD should include structured classroom management, teacher and parent education and collaboration (to address discipline and limit- 74 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics setting), tutoring and/or behavioral therapy for the individual in treatment.21 Education about the facts regarding ADHD and the special needs of these youth begins the process and should include specific recommendations for teachers on managing students diagnosed with ADHD. Effective teachers learn to incorporate these behavioral (contingency management) techniques into their teaching regime. School based interventions should also include peer mediation to help students resolve conflict, learn prosocial skills and teach them conflict management skills.18 Peer mediation programs in schools address a wider range of youth than are affected with traditional treatment programs. They overcome obstacles to treatment such as lack of access, financial constraints, refusal of parents to participate and adolescent resistance to psychotherapy.18 Student- mediated conflict resolution programs consist of trained teams of older youth helping peers solve conflicts and have occurred in two settings: schools and private offices. Successful programs must have administrative support and commitment to the goals and practices of the program; there must be adequate administrative policy support, majority teacher decision support and parent organizational support. The content of the program includes student-mediation teams of well- trained members who receive 12-15 hours of skill focused training. Successful programs have adequate numbers of team members, designated team captains, and develop meaningful consequences for harassing mediators. Further, there must be a supportive infrastructure that includes an assembly launching program, morning announcements, centrally- posted team bulletin board, two active playground supervisors and two mediation team champions combined with weekly team meetings.18 Peer mediators receive training that requires them to engage in component skill building, mastery of the mediation process, solving common problems, communication skills building, and field simulation exercises. Active ongoing recruitment must occur to sustain the next generation of peer mediators. Transition of new and old members into new teams is facilitated if there are planned sessions to introduce new teams. The inclusion of parents and high-risk students as trained mediators improves the overall environment.18 Attention Deficit Hyperactivity Disorder: Evaluation and Management 75

Support groups provide individuals with common concerns and ways to come together to learn and share their issues, experiences, strengths, weaknesses, frustrations and successes; they also discuss information about what works, as well as share their hopes for themselves and their youth. Supports groups are useful with youth, adolescents and adults. They can be used as interventions in the school, community, or private office. Such groups can be organized around any topic, such as parent, adolescent or teacher support and can include peer mediation groups. Support, groups can be local, regional, state, and national. In many programs social skills activity groups are often held concurrent with parent training/support groups.15

Psychological vs Psychopharmacological Treatment Some scholars13,23,24 argue that despite the impressive track record established by stimulant therapies over the past two decades, this treatment has serious limitations, including short treatment duration, positive changes occurring in only a few functional problem domains, and the observation that some youth suffer physiological side effects.14 Not all youth can be given these medications, nor do all take them. Many experts believe that the most significant, long-lasting gains appear when medication is combined with behavioral therapy, emotional counseling, and practical support.2,3 Even when pharmacologic intervention (particularly stimulant medication) has a positive influence on achievement-related measures, the benefits usually occur in the presence of intervention that emphasizes direct teaching of the academic curriculum. Overall, reducing problem behavior is not a sufficient intervention for youngsters with overlapping achievement and behavior problems. The promotion of academic success is critical for these youth. Youth with comorbid learning disabilities also need behavioral interventions designed to enhance motivation and direct instruction focusing on specific skill deficits.22,25 Pelham and Fabiano24 support the thesis that behavior therapy should be the first line of defense in the treatment of ADHD.24

SUMMARY Attention-deficit disorder (ADD) or attention-deficit/hyperactivity disorder (ADHD) is a complex behavioral disorder with symptoms that range from mild to severe. The impact of this disorder on the lives of 76 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics affected youth can be minimal or so severe as to cause academic failure, as well as serious emotional problems. These adolescents are at increased risk of physical injury, psychosocial dysfunction, depressive and anxiety disorders, conduct disorder, and drug abuse. Youth with ADHD who receive help, guidance, and understanding from parents, guidance counselors, and the public education system can reach their full potential.1 Adolescents who exhibit symptoms of ADHD require an evaluation that examines their individual (their physical, cognitive social, emotional, exposure to substance and violence, socioeconomic status), academic, family and environmental characteristics. The results of the evaluation should guide treatment. Research supports that a combination of pharmacological and behavioral therapy attains the best outcome.

KEY LEARNING POINTS • Adolescents who exhibit symptoms of ADHD require an evaluation that examines their individual (their physical, cognitive social, emotional, exposure to substance and violence, socioeconomic status), academic, family, environmental characteristics. • The results of the evaluation should guide treatment. • Although the preferred treatment modality is stimulant medication, adolescents with ADHD often have comorbid psychosocial problems that are the results of their struggles prior to treatment. Those problems require interventions ranging from extramentoring, peer support to psychotherapy. • The most effective psychotherapy has been shown to be behavior therapy, specifically cognitive behavior therapy. • The NIMH MTA study supports that a combination of pharmacological and behavior therapies are most effective. The addition of parent training, and support groups serves to enhance the adolescent’s ability to manage problems and prevent major setbacks. • Youth who receive appropriate treatment can reach their full potential.

REFERENCES 1. Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder, In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. New York:Universe. 2006 :107-142. Attention Deficit Hyperactivity Disorder: Evaluation and Management 77

2. National Institute of Mental Health (NIMH). Attention deficit hyperactivity disorder. Bethesda (MD): National Institute of Mental Health, National Institutes of Health, US Department of Health and Human Services; NIH Publication No. 3572. (2003, Revision) http://www.nimh.nih.gov/publicat/adhd.cfm#symptoms Accessed 7/30/2006. 3. National Institute of Mental Health (NIMH): Attention Deficit Hyperactivity Disorder (Overview). Bethesda (MD): National Institute of Mental Health, National Institutes of Health, US Department of Health and Human Services; NIH Publication No. 01-4589. (2001, Updated 2/17/2006) http://www.nimh.nih.gov/ publicat/helpchild.cfm Accessed 7/30/2006. 4. American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th edn. Text Revision. Washington, DC: American Psychiatric Association 2000: 85–93. 5. Practice Management Information Corporation. Mental Disorders: International Classification of Diseases, 9th Revision, 6th edn. Los Angeles, CA: Practice Management Information Corporation 2001: 223. 6. Wolraich ML, Felice ME. The Classification of Child and Adolescent Mental Diagnoses in Primary Care: Diagnostic and Statistical Manual for Primary Care (DSM-PC): Child and Adolescent Version. Elk Grove Village, IL: American Academy of Pediatrics 1986: 93–110. 7. Hardman ML, Drew CJ, Egan, MW. Human Exceptionality:Society,School, and Family. Boston: Allyn and Bacon 2002. 8. MTA Cooperative Group. National Institute of Mental Health Multimodal Treatment Study of ADHD follow-up: Changes in effectiveness and growth after the end of treatment. Pediatrics 2004; 113: 762-769. 9. Reiff MI, Stein MT. ADHD evaluation and diagnosis: A practical approach to office practice. Pediatr Clin North Am 2003;50:1019–1048. 10. Johnston C, Leung DW. Effects of medication, behavioral, and combined treatments on parents’ and youth’s attributions for the behavior of youth with attention-deficit hyperactivity disorder. J Consult Clin Psycholog, 2001; 69: 67-76. 11. Barkley RA, Edwards G, Laneri M, Fletcher K, Metevia L. The efficacy of problem- solving communication training alone, behavior management training alone, and their combination for parent-adolescent conflict in teenagers with ADHD and ODD. J Consult Clin Psycholog 2001; 69: 926-941. 12. Malott RW, Malott, ME, Trojan, EA. Elementary Principles of Behavior, 4th edn. Upper Saddle River: Prentice Hall 2000. 13. Whalen CK, Henker B. Therapies for hyperactive youth: Comparisons, combinationa and compromises. J Consult Clin Psycholog 1991;59:126-137. 14. Reuters Health : ADHD: Kids with Attention Problems More Likely to Wet Bed. Bethesda, MD: U.S. National Library of Medicine, Wednesday, October 23, 2002 http:/ /www.nlm.nih.gov/medlineplus/news/fullstory_10009.html. 15. Cunningham CE, Cunningham LJ. Bench marks of effective programs. Student mediated conflict resolution programs: In: Barkley RA (Ed.) Attention-Deficit Hyperactivity Disorder: A handbook for diagnosis and treatment, 2nd edn. New York: Guilford 1998:.491-509. 16. Pfiffner LJ, Calzada E, McBurnett K. Interventions to enhance social competence. Child Adolesc Psychiatr Clin N Am 2000; 9: 689-709. 17. Embregts PJ. Effect of resident and direct-care staff training on responding during social interactions. Res Dev Disabil 2002; 23: 353-366. 18. Robin AL. Training families with ADHD adolescents: In: Barkley RA (ed): Attention-Deficit Hyperactivity Disorder: A Handbook for Diagnosis and Treatment, 2nd edn New York: Guilford Press 1998: 413-457. 78 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

19. Gresham FM, Sugai G, Horner RH. Interpreting outcomes of social skills training for students with high-incidence disabilities. Except Child 2001; 67: 331-344. 20. Anastopoulos AD, Smith JM, Wien EE. Counseling and training parents In: Barkley RA (ed): Attention-Deficit Hyperactivity Disorder: A Handbook for Diagnosis and Treatment, 2nd edn. New York: Guilford 1998:373-393. 21. March JS, Swanson JM, Arnold LE, Hoza B, Conners CK, et al. Anxiety as a predictor and outcome variable in the multimodal treatment study of youth with ADHD (MTA). J Abnorm Child Psychol 2000; 28:527-542. 22. Pfiffner LJ, Barkley RA. Treatment of ADHD in school settings. In: Barkley RA (ed): Attention-Deficit Hyperactivity Disorder: A Handbook for Diagnosis and Treatment 2nd edn New York: Guilford 1998: 458-490. 23. Hinshaw SP. Academic underachievement, attention deficits, and aggression: Comorbidity and implications for intervention. J Consult Clin Psychology 1992; 60: 893-903. 24. Pelham W, Fabriano GA. Behavior modification. Child Adolesc Psychiatr Clin North Am 2000; 9:671-688. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Attention Deficit Hyperactivity Disorder in Adolescent: 8 Psychopharmacology

Donald E Greydanus, Daniel Olson

INTRODUCTION Attention-deficit/hyperactivity disorder (ADHD) is noted in 3 to 12% of children and adolescents. It is three times more common in males than in females.1, 2 ADHD is a complex, neurobehavioral disorder that presents with attentional dysfunction, sometimes in association with various degrees of impulsivity and hyperactivity. It is a lifelong condition and the individual must learn to adapt to the limitations it may present.3 Basic principles of management include obtaining a correct diagnosis as early as possible (preferably in childhood), developing an appropriate educational plan to allow maximum learning, correcting any comorbidities (Table 8.1) that may be present, employing various psychological management strategies (Table 8.2), and, in some cases, using medications.1 This article focuses on the psychopharmacologic approach to an adolescent with ADHD. 4-6 Table 8.1: Comorbidities of ADHD Depression disorders Anxiety disorders Substance abuse disorders Conduct disorder Oppositional defiant disorder Learning disorders (i.e. math, reading, others) Others

PSYCHOPHARMACOLOGIC MANAGEMENT There are over 60 years of research showing that medication can ameliorate ADHD symptoms in children, adolescents, and adults.1,7 The beneficial 80 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 8.2: Psychological management of ADHD in youth Psychotherapy Cognitive-behavioral therapy (CBT) Behavioral therapy (BT) Psychosocial interventions Support groups Social skills training Biofeedback training Peer mediation to resolve interpersonal conflicts Family therapy Self management training role of psychopharmacology is due to its effects on the central nervous system noradrenergic and dopaminergic pathways. In the United States, 4.3% (nearly 2.5 million) of children age 4-17 years were on medication for ADHD in 2003; approximately 56.3% of children with a diagnosis of ADHD were on anti-ADHD medication.8 Table 8.3 lists medications with research evidence of benefit for ADHD: stimulants, antidepressants, alpha2 agonists, and norepinephrine reuptake inhibitors. These medications are now reviewed. Principles of ADHD psychopharmacology are listed in Table 8.4.

Stimulant Medication Hundreds of research studies on patients with ADHD have noted the beneficial effects of stimulant medication, say methylphenidate, amphetamine, and pemoline (Table 8.3).1 Since the late 1930s, it has been observed that stimulants improve attention-span impairment, and sometimes, hyperactivity and impulsivity.9 Approximately 75% or more of those with ADHD demonstrate some benefit, and the use of medication has become a standard part of management for these patients by many clinicians.8 The most comprehensive study today was arranged by the National Institute of Mental Health (NIMH) in the United States. This research was named the NIMH Collaborative Multisite Multimodal Treatment Study of Children with Attention-Deficit/Hyperactivity Disorder (or the MTA study).10-13 It involved almost 600 children between 7-9 years of age and documented the efficacy of methylphenidate (MPH) for these children above that of psychological therapies. The second best management strategy was the combination of MPH plus cognitive behavioral therapy.10-13 This discussion now considers methylphenidate (MPH), amphetamine, and pemoline. Attention Deficit Hyperactivity Disorder: Psychopharmacology 81

Table 8.3: Medications used in a attention disorders Medication Daily dose schedule (mg/kg) Common adverse side (unless otherwise indicated) effects Stimulants MPH (Methylphenidate) 0.3-2.0 (10-80 mg/day) in Insomnia, decreased 2-4 divided doses appetite, abdominal pain, headache, depression, weight loss, rebound symptoms, decreased velocity vs. growth delay

Magnesium pemoline 0.5-3.0 (37.5-131.25 mg/day) Same as MPH + possible in 1-2 divided doses liver toxicity (new FDA “black box” warning) Dextroamphetamine 0.1-1.5 (5-80 mg/day) in Same as MPH but possibly 2-4 divided doses more depression Antidepressants TCAs 1-5 Anticholinergic effects, others. See text. Imipramine desipramine 1-5 Nortriptyline 0.5-3 Bupropion 3-6 (50-300 mg/day) in Insomnia, irritability, drug- 2-3 divided doses induced seizures (with doses > 6 mg/kg); contraindicated in bulimic patients

Alpha2-agonists Clonidine 3-10 µg/kg (0.05-0.4 mg/day) Sedation (very frequent), in 2-4 divided doses depression, dry mouth, rebound hypertension, hypotension (rare), confusion (with high doses), localized irritation with transdermal preparation Guanfacine 15-43 µg/kg (0.5-4.0 mg/day) Same as clonidine but in 1-2 divided doses much less sedation, less hypotension Norepinephrine reuptake inhibitors Atomoxetine 0.5-1.4 mg/kg/day in 1-2 Decreased appetite, divided doses dyspepsia, dizziness, fatigue, sedation, nausea emesis, mood swings, growth delay Source: Modified with permission from: Greydanus DE, Pratt HD et al. Psychopharmacology of ADHD in adolescents. Adolesc Med 2002;13:600. 4 82 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 8.4: Principles of psychopharmacologic management for ADHD

1. Educate the patient and parents (“family”) about the purpose of these medications; clarify the goals of medications (improving concentration, decreasing impulsivity, others). The clinician should avoid focusing only on medication in the clinical encounter. This implies to families that medication use alone should be the remedy to all problems. It further implies that when things are not going well, the problem must be with the choice or dose of medication. This shifts responsibility for problems completely to the clinician who must then urgently find the right medication and dosage. 2. Be sure the patient and parents understand that medications are not curative. 3. Correct any “myths” about medication the family may have. For example, medication will not correct family problems (i.e. alcoholism in a parent, contentious custody battles). 4. Wait for the patient/family to approve of a trial medication period before embarking on medication management. Do not force medication on a child or adolescent. 5. Educate the patient/family about potential side effects of medications and how you will deal with them; follow these patients on a regular basis to monitor efficacy and adverse effects. 6. Provide a thorough evaluation of the patient and family to determine possible comorbidities that may benefit from other medications and/or therapy. 7. Be supportive of other management tools (i.e. psychoeducation strategies, behavioral therapy). 8. Begin with a low dose and increase slowly until identified target symptoms are sufficiently improved; stop the medication(s) if side effects are unacceptable or upper medication levels are reached without amelioration of target symptoms. 9. Specific medications and doses may vary from patient to patient and are identified by careful trial and error. Medication(s) that are helpful may change as the child emerges to adolescence and adulthood. 10. Adolescents may require a medication dose higher than needed for adults because of increased renal clearance of drugs, lower body fat percentage, increased liver metabolism, or idiosyncratic medication metabolism. 11. Strive to achieve complete syndrome remission if feasible (rather than settling for symptom improvement). 12. Share responsibility explicitly by clearly stating what issues the family must work on, the school must work on, the child or adolescent must work on, and the physician must work on. Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Attention-deficit/ hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1061-1062. 7

Methylphenidate Methylphenidate (MPH) is the most common prescribed stimulant for patients with ADHD since its production started in 1957. Its beneficial effect on attention span dysfunction is based on selective binding of the presynaptic dopamine transporter in the CNS striatal and prefrontal areas, leading to a rise in extracellular dopamine. It also causes a blockade of the CNS norepinephrine transporter in the norepinephrine system.14-16 MPH Attention Deficit Hyperactivity Disorder: Psychopharmacology 83

(Ritalin and the generic form as well) is taken orally with peak pharmacologic action occurring in 1-2 hours, disappearing in 4 hours, and requiring another dose if additional attention span benefit is desired. Beneficial effects of stimulant medication may include enhanced concentration, reduced hyperarousal, reduced impulsivity, reduced motor restlessness (i.e. less gross/fine motor movement and/or work performance), and less aggressive and/or antisocial behavior. A common method of prescription is to have the youth take MPH in the early morning, noon, and, if necessary, late afternoon.4,5 The youth and clinician can decide together the schedule that works best for the adolescent. A low dose (i.e. 2.5 to 5 mg) can be started initially, with gradual titration upwards, finding the optimal dose for each individual patient. The dosage range is 0.3 to 2.0 mg/kg/day with single doses usually not exceeding 20 mg and daily doses not exceeding 60-80 mg. There is no correlation between weight of the youth and optimal MPH dose, and plasma levels of MPH are not useful in determining the correct dosage. A number of clinical tools are used to verify effectiveness, such as patient/ family interviews, parent ratings, and school grades or reports. Reasons for failure of MPH to be effective are listed in Table 8.5.4 Table 8.5: Reasons for failure of methylphenidate Inaccurate diagnosis Comorbid disorders that overshadow the ADHD Medication doses that are too high or not high enough Medication is diverted to others in or outside the family Intolerable medication side effects Medication is used as a drug of abuse for its euphoric effects Patient and/or family not accepting of medication Patient does not respond to MPH but does to other stimulants or alternative medications Patient does not respond to medications of any kind Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Psycho- pharmacology of ADHD in adolescents. Adolesc Med 2002;13:604.4

MPH Side Effects Stimulant (including MPH) side effects are listed in Table 8.6, some of which are transient, and can be reduced if the patient starts with a low dose and slowly increases the dosage to maximize benefit with reduced side effects.1,17 Contraindications to stimulants (including MPH) include drug dependence, uncontrolled hypertension, glaucoma, symptomatic cardiovascular disorder, psychosis, and hyperthyroidism. Cardiovascular 84 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics side effects of stimulants are well-known, including increased blood pressure as well as pulse, and the very rare event of sudden death.18,19 Stimulants should not be combined with monoamine oxidase inhibitors (MAOIs) because this mixture may lead to a hypertensive crisis. In rare cases, mixing a stimulant with a tricylic antidepressant may lead to sudden death from cardiac arrhythmia. MPH can interfere with the metabolism of some anticonvulsant drugs, such as ethosuximide, phenytoin, and phenobarbital. Stimulant effectiveness can be reduced by mixing stimulants with antihistamine drugs.

Table 8.6: Potential side effects of stimulants (including methylphenidate) Headache* Abdominal pain* Jitteriness* Dizziness Anorexia* Insomnia (delayed onset of sleep)* Social withdrawal* Weight loss (due to decreased appetite)* Moodiness (irritability) Nausea Dry mouth Constipation Increase in heart rate, blood pressure, and palpitations “Unmasking” of Tourettes syndrome (TS) Appearance of being “dazed or drugged;” perseveration and withdrawal Rebound phenomenon Increased hyperactivity Appearance of psychosis or psychotic features Personality change Tolerance Skin rash (rare) *Commonly seen side effects Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Psycho- pharmacology of ADHD in adolescents. Adolesc Med 2002;13:607. 4 Nausea or emesis that may occur while the patient is on stimulants often improves if the medication is taken with meals. If the cardiovascular system is normal, the mild rise in heart rate and blood pressure noted with stimulants is not a problem. Dizziness occurs in some patients, worse with short-acting stimulants versus long-acting types (see below under MPH preparations); if this occurs, look for dehydration or blood pressure changes and treat as necessary. Headaches may develop while taking stimulants, sometimes related to peaking MPH plasma levels, and some- Attention Deficit Hyperactivity Disorder: Psychopharmacology 85 times related to drug withdrawal. Prescribing a different stimulant may improve the headache issue. There is no indication that the use of stimulant medication increases the risk of substance abuse in youth.20 The phenomenon of delayed growth in children or young adolescents on stimulants remains controversial and seems to be, in part, related to appetite suppression with decreased nutrition. It appears to be a transient effect, though more research in this area is on-going.1 Youth on stimulants who are not growing properly need careful supervision. If the appetite is blunted while on stimulants, a number of measures can be considered, including taking food when the stimulant wears off (as in the evening), using high-caloric foods, taking the patient off stimulants when not in school (such as during vacation or on weekends), and trying other stimulants or anti-ADHD medications. Tolerance may develop in some youth on high stimulant doses and does not indicate any type of addiction. Management involves lowering the stimulant dose or trying a different anti-ADHD drug.1,4 Rebound can occur in which increased ADHD symptoms (as rage episodes and irritability) develop as the stimulant effect wears off, such as in late morning or late afternoon for short-acting MPH or late afternoon for the long-acting stimulant. Because MPH may interfere with sleep, the addition of other medications causing sedation may be beneficial, such as bupropion, tricyclic antidepressants, alpha2 agonists (i.e. clonidine), mirtazepine, trazodone, or melatonin. ADHD is found in 50 to 75% of patients with Tourette’s syndrome (TS) and TS may be officially diagnosed in some children or adolescents after starting stimulant drugs.1,4 Research does not suggest that stimulant medications cause TS and the presence of tics is a relative, not absolute contraindication to stimulant medication. Youth with both ADHD and TS may be given both stimulant medications (if effective) and anti-tic medication (such as risperidone, haloperidol, or pimozide).21 If the tics are worsened by the stimulant drugs, other anti-ADHD medications that do not typically worsen tics may be prescribed. These include alpha2 agonists (as clonidine or guanfacine) or atomoxetine. Bupropion may improve ADHD but worsen tics. 86 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

MPH Preparations Until recently Ritalin, generic MPH, and Ritalin-SR have been very popular. Now, a number of longer acting MPH products have been developed and are being used.1,4-6 Ritalin-SR is a longer-acting version of Ritalin, delivering 7 mg of MPH over several hours. However, its absorption is erratic in half or more of patients. It is only is produced in a 20 mg form. Pharmaceutical companies have now developed a variety of alternative MPH preparations over the past decade (Table 8.7) and have encouraged clinicians to use them. A recent MPH formulation is the transdermal methyphenidate patch which lasts as long as oral formulations and can be used for those not tolerating pills or other anti-ADHD medication.22 The patch allows early removal for those with MPH-induced insomnia, but patch-induced dermatitis may be bothersome for some.22 Unfortunately there are no unbiased studies available at this time to tell the clinician and patient which of these products are superior, and patients must simply find out which product is best for them. If a patient has difficulty swallowing pills, some of the longer-acting products can be opened and added to food; these products include Ritalin LA and Metadate CD. Longer-acting amphetamine products that can be opened and added to food include Dexedrine Spansule, and Adderall XR. Table 8.8 compares the relative duration of action of these compounds. The reader is referred to recent articles reviewing these newer MPH preparations.1,4-7,23-26

Table 8.7: Available methylphenidate medications Ritalin (MPH immediate-release; 5, 10, 20 mg tablets) (Novartis) Ritalin-SR (20 mg MPH tablet, sustained-release) (Novartis) Generic MPH (both immediate-release and sustained-release; tablet options same as Ritalin) (Geneva) Ritalin LA (Ritalin developed for 8-9 h duration; 20, 30, 40 mg capsules) (Novartis) Metadate ER (sustained-release [8 h] MPH; 20 mg tablets) (Celltech) Methylin ER (sustained-release [8 h] MPH; 10, 20 mg tablets) (Mallinckrodt) Concerta (MPH HCl: extended-release tablets: 18, 36, 54 mg; up to 12 h MPH duration) (Alza-McNeil) Metadate CD (MPH HCl: Extended-release 20 mg capsules; 8-9 h of MPH duration) (Celltech) Focalin (dexmethylphenidate [purifed D-methylphenidate] to last 4-6 h at half the usual dose; 2.5, 5, 10 mg tablets) (Novartis) Daytrana (Transdermal methylphenidate; once a day MPH patch) (Noven/Shiren) Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Attention-deficit/ hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1063. 7 Attention Deficit Hyperactivity Disorder: Psychopharmacology 87

Table 8.8: Stimulants: Duration of action A. Short-acting (3-6 h) Ritalin? Methylene? Dexadrine? Dextrostat? Focalin? B. Intermediate-acting (6-8 h) Ritalin-SR? Methylene ER? Adderall? Metadate ER? C. Once-daily (8+ h) Dexedrine spansules Concreta? Adderall XR? Metadate CD? Ritalin LA? Source: Modified with permission from: Greydanus DE, Pratt HD,et al. Attention-deficit/ hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1065.7

Amphetamine Products A well-known alternative to MPH is dextroamphetamine (Dexedrine; Dextrostat) which can be used if MPH is not acceptable or helpful to the patient with ADHD. However, the potential adverse effects of dextroamphetamine are the same as MPH (Table 8.6).1,20 The known beneficial effects of amphetamine on ADHD dates back to 1937.9 Dextroamphetamine is effective for 3 to 6 hours, and its mechanism of action includes norepinephrine reuptake inhibition and selective binding to the dopamine transporter with increased dopamine release; weak effects on the serotonin system are also noted.1,16 Amphetamine products that are available are listed in Table 8.9. Dextroamphetamine is taken 1 to 3 times a day with gradual titration to 0.15 to 0.5 mg/kg/day, up to 40 to 50 mg a day. A generic version is also available. Dexedrine spansule is given once a day and produces anti-ADHD effects for 8 to 10 hours. Amphetamines may induce more depression than MPH and the abuse potential of amphetamine is well-known. Amphetamine and MPH should not be combined. Adderall and Adderall XR are long-acting amphetamine products that last up to 10 and 12 hours respectively. 88 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 8.9: Available amphetamine stimulant medications Dexedrine (dextroamphetamine tablets; short-acting) (GlaxoSmithKline) Dexedrine Spansule (long-acting dextroamphetamine) (GlaxoSmithKline) Dextroamphetamine generic (Barr) Dextrostat (dextroamphetamine) (Shire US) Adderall (mixed-salts amphetamine with extended-release) (Shire US) Adderall XR (mixed-salts amphetamine with extended-release) (Shire US) Desoxyn (methamphetamine) (Abbott) Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Attention-deficit/ hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1075. 7

Prevalence of Stimulant Abuse The use and misuse of stimulants often begins in childhood and adolescence. For example, a review of 161 children on methylphenidate (Ritalin) who noted that the stimulant was helpful revealed that 16% had been asked to give or sell their medication to others.27 Another survey of middle and high school students reports that 23.3% of students taking prescribed stimulants had been solicited to divert their medication to others at a rate that increased from middle school to high school.28 One Canadian study of students (grades 7, 9, 10, 12) noted 5.3% on stimulant medication; out of this group, 14.7% reported having given their medications to others, 7.3% sold their medication, 4.3% had their medications stolen, and 3.0% were forced to give up their prescription medications.29 These diversion patterns then continue on into the college milieu.30 There are some colleges and universities that report no or minimal stimulant abuse problems.20,31,32 However, most other studies disagree with this optimistic view. Non-medical use of prescription medications (including prescription stimulants and others) represents the second most common form of illicit drug use in the college population, second only to marijuana use/abuse.33 The University of Michigan’s Monitoring the Future Study has noted that college students report a 5.7% rate on non- prescription use of methylphenidate (MPH) in contrast to 2.5% in non- college peers.33 A 2001 study of 10,904 college students using self-reports identified a 6.9% life-time prevalence of non-medical prescription stimulant abuse, including a past year prevalence of 4.1% and a past month prevalence of 2.1%.31 A survey of 9,161 undergraduates reported an 8.1% lifetime prescription stimulant abuse, including 5.4% over the past year.34 A smaller Attention Deficit Hyperactivity Disorder: Psychopharmacology 89 study reported that 17% of 179 college males and 11% of 202 college females reported illicit prescription stimulant abuse, while 44% of these students knew students who abused stimulants.35 Another college survey reports that 35.5% of college undergraduates abused prescription amphetamines, males abusing more than females.36 One survey of 334 college students who were prescribed stimulants for ADHD reported that nearly 29% of these students had sold or given their medication to others.37 We only have a snapshot of this issue at this time; however, stimulant abuse is a real and serious problem on the college campus in the early 21st century.

Pemoline Pemoline (magnesium pemoline; Cylert) is a stimulant medication taken once daily with a dosage range of 0.5 to 3.0 mg/kg/day, with a maximum dose of 112.5 to 131.25 mg per day.1,5 A movement disorder is noted in some patients on this medication. Chemical hepatitis develops in approximately 3% of pemoline users after several months. In the United States, the Federal Drug Administration (Washington, DC) has given a “black box” warning for pemoline because of rare irreversible liver failure that may occur. Patients should be informed of this side effect and have their liver function tests closely monitored. Unfortunately, even close monitoring cannot prevent death from liver failure. Because of this problem, pemoline is now rarely used as an anti-ADHD drug in the United States and no longer actively produced.1

NONSTIMULANT MEDICATIONS

Alpha2 Agonists Clonidine

Clonidine (Catapres) is a presynaptic, central-acting alpha2-adrenergic agonist that is used by some clinicians to manage ADHD symptoms, though it may take weeks to months to achieve full benefit.1,4-7 Clonidine is used as an alternative or adjunctive medication to MPH; it is often given with MPH and may be helpful because its sedative properties off-set the insomnic effect of MPH. Clonidine is also used to manage Tourette’s syndrome, post-traumatic stress disorder, and severe aggressiveness with conduct disorder or oppositional defiant disorder. Table 8.10 lists potential adverse effects of clonidine. 90 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

The daily dose range of clonidine is 0.05 to 0.04 mg/day; depending on its use, clonidine is provided 2 to 4 times a day or only at bedtime. Gradual build-up and gradual withdrawal when stopping are recommended. Rapid withdrawal may lead to rebound hypertension. When prescribing this drug, it is important to take some baseline data (blood pressure, pulse, blood sugar, electrocardiogram [ECG]); follow this data on a regular basis, and include a repeat ECG every 6 months. Tolerance and liver function activation have also been reported. A few deaths have been reported in children and adolescents taking both MPH and clonidine. Clonidine is also available as a patch lasting 3 to 7 days; this patch may prevent sedation and/or elevated blood pressure that can be seen with the pill form. As with any patch, dermatitis may occur at the site of the patch application; changing the patch site and local application of hydrocortisone usually resolves the local dermatitis. Table 8.10: Side effects of clonidine Sedation (50%) Dry mouth Headache Attention impairment Decreased glucose tolerance Depression Dermatitis (from the patch) Dizziness Dysphoria Fatigue Inconsistent effects Irritability Itchy eyes Postural hypotension Potentiation of neuroleptic anticholinergic side effects Rebound phenomenon Tolerance Weight gain Withdrawal effects (rebound tachycardia and severe hypertension from sudden clonidine cessation) Worsening of preexisting cardiac arrhythmias Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Psychophar- macology of ADHD in adolescents. Adolesc Med 2002;13:615. 4

Guanfacine

Guanfacine (Tenex) is an alpha2A adrenergic agonist related to clonidine, and it is also not proven to benefit the attention span problems of ADHD.1,2 Attention Deficit Hyperactivity Disorder: Psychopharmacology 91

Its use may result in less blood pressure problems and sedation than seen with clonidine. Adverse reactions are similar to clonidine, but there may be more agitation and headaches. Its daily dosage range is 0.5 to 4.0 mg.

ANTIDEPRESSANTS Tricyclic Antidepressants Research studies note that tricyclic antidepressants (TCAs) may be of help to patients with ADHD and are usually listed as alternatives to stimulant medications if stimulants are not beneficial or are contraindicated; Table 8.11 lists other indications for the use of TCAs.1, 4 These medications include imipramine (Tofranil, others) 50-200 mg/day, desipramine (Norpramin, desipramine) 50-200 mg/day, amitriptyline (Elavil) 50–200 mg/day, and nortriptyline (Pamelor) 20-100 mg/day. Mechanisms of action include blockage of norepinephrine and serotonin reuptake and down regulation of beta-adrenergic receptors.1,4

Table 8.11: Indications for tricyclic antidepressants* ADHD Aggression disorders Anxiety disorders (as panic disorder or obsessive compulsive disorder) Depression Enuresis Insomnia Migraine headaches Tourette’s syndrome *Used with permission from: Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder” In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. NY and Lincoln, Nebraska:Universe 2006:134.

Table 8.12 lists adverse effects of TCAs, including those that are anticholinergic. Youth placed on TCAs should be monitored on a periodic basis, with evaluations noting vital signs, complete blood counts, serum levels of TCAs, and EKGs; management plans are noted in the literature.38,39 Plasma TCA levels correlate with toxicity, but not with level of benefit. TCAs tend to cause less rebound effect than stimulants, and tolerance may develop with TCAs. Sedation can be severe and is worse with imipramine than with desipramine or nortriptyline. TCA-induced tremors may be improved with careful reduction in TCA dose or adding 92 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics propranolol (10-40+ mg per day), though the latter may lead to depression in some patients.6,7 If agitation develops as a result of the TCA, lower its dose or add a benzodiazepines medication. Mania may develop if a TCA is added to someone with latent bipolar disorder; also, psychosis may develop if a TCA is added to someone with latent schizophrenia. Rare cases of death are noted in children or adolescents taking TCAs, especially desipramine.38,39 Nausea, emesis, fatigue, or worsening behavior may develop if the TCA is stopped too abruptly; this is called the discontinua- tion syndrome. Care must be taken when adding other medications to TCAs. For example, toxic TCA levels may occur when combining TCAs with selective serotonin reuptake inhibitors. The combination of MPH and a TCA may lead to rising TCA levels as well. Respiratory depression and death may develop after combining alcohol and TCAs.

Table 8.12: Tricyclic antidepressant side effects Blood dyscrasias Blurred vision (including cycloplegia and mydriasis) Cholestatic jaundice Confusion Constipation Delirium (in high doses) Dizziness Drowsiness and sedation Drug interactions (as with SSRIs [selective serotonin reuptake inhibitors]) Dry mouth (with decreased salivary flow and increased tooth decay) ECG changes (sinus tachycardia, AV blocks, increased QRS interval, increased QTc interval) Exercise-induced tachycardia Hypotension Increase in heart rate (10–15 bpm) and blood pressure (upto 8-10 mm Hg) Lowered seizure threshold Peripheral neuropathy Priapism Respiratory failure and death from an overdose Skin rash Sudden death Tachycardia Tremor Urinary retention Weight gain Source: Modified with permission from: Greydanus DE, Pratt HD, et al. Attention-deficit/ hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1079. 7 Attention Deficit Hyperactivity Disorder: Psychopharmacology 93

Bupropion Bupropion (Wellbutrin) is an antidepressant medication with noradrenergic/dopaminergic effects and is used to improve depression, reduce irritability, and ameliorate attention dysfunction.1,6 Bupropion is given in a daily dosage range of 50-300 mg (3.0 to 6.0 mg/kg/day) as Wellbutrin, 100-150 mg twice daily as Wellbutrin-SR, and once a day as Wellbutrin XL (150 mg or 300 mg). In an alternative formulation of bupropion (Zyban), it is used to manage tobacco addiction.40 Table 8.13 lists side effects of bupropion, including seizure activity, 0.1% under 300 mg a day and 0.4% over 300 mg a day. Bupropion is contraindicated if youth have epilepsy or eating disorders (such as bulimia nervosa). Risks for seizures are reduced if bupropion is not taken at least 8 hours apart, if the medication is slowly titrated upward in dose, if the SR formulation is used, and if high doses of the regular formulation are avoided. Drug-to-drug interactions are minimal with bupropion and it does not lead to cardiac conduction delays. Also, venlafaxine (Effexor) is an atypical antidepressant that is used by some clinicians for ADHD.

Table 8.13: Bupropion side effects*

Anorexia Agitation Nausea Restlessness Drowsiness Headache Tics (exacerbation) Seizures (0.1% under 300 mg/day and 0.4% over 300 mg/day) *Used with permission from: Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. NY and Lincoln, Nebraska:Universe 2006:136.

Atomoxetine Atomoxetine (Strattera) is a non-stimulant drug currently listed by the United States Federal Drug Administration as an alternative anti-ADHD drug and it is used in those not wishing to take a stimulant or where stimulant or other medications are not effective.41 Its actions include the blockade of the presynaptic norepinephrine transporter in the prefrontal cortex. It is started at 0.5 mg/kg/day and gradually raised to 1.0 to 1.4 mg/kg/day to a maximum of 100 mg per day, given once or twice a day. Table 8.14 lists potential adverse effects, including drug-to-drug 94 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics interactions involving selective serotonin reuptake inhibitors (SSRIs) and others metabolized by cytochrome P450 2D6. Atomoxetine has a low affinity for various receptors, such as alpha1-adrenergic, alpha2-adrenergic, serotonergic, cholinergic, and histaminic. There is no increase in drug addiction, drug diversion, cardiovascular complications, or tics. Table 8.14: Atomoxetine side effects* Anorexia Nausea Emesis Sedation Fatigue Dizziness Mood swings Dyspepsia Growth delay *Used with permission from: Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. NY and Lincoln, Nebraska:Universe 2006:137. Table 8.15: Nonresearch-supported ADHD treatment options* Antiyeast medications Chiropractic manipulation Dietary manipulation Electroencephalographic-biofeedback training Herbal treatments Megavitamin therapy Sensory integrative training “Herbal” products Acetyl carnitine DMAE (dimethylaminoethanol [Deaner]) Ginkgo Phosphatidylserine (CNS phospholipid) Essential fatty acids (gamma-linolenic acid, docosahexaenoic acid) *Used with permission from: Greydanus DE, Pratt HD: Attention deficit hyperactivity disorder In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. NY and Lincoln, Nebraska:Universe 2006:137.

New and Future ADHD Medications Table 8.15 lists treatment options used by some clinicians but none have been proven to benefit children, adolescents, or adults with ADHD.1 However, there are a variety of medications that are being released and being developed that have been shown to improve the attentional dysfunction of ADHD.1,42-44 As more research progress takes place, ADHD will be divided into subgroups depending on what part (s) of the central nervous system is responsible for a specific ADHD type. Medications will Attention Deficit Hyperactivity Disorder: Psychopharmacology 95 be designed to work at a specific area(s) of the CNS that is affected. Medications that are being studied include the ones listed in Table 8.16. Finally, it should always be clearly understood by clinicians, patients, and their families that medication is only part of the overall management strategy to improve the lives of our children and adolescents with ADHD.

Table 8.16: New and future ADHD medications1,42-44 Modafinil (Sparlon) (Also seen a Provigil to manage narcolepsy) NRP 104 (Dextroamphetamine with constant rate) Ropinirole (Dopamine agonist used to treat syndrome of restless legs) AMPA (ampakines) receptor modulator (to improve memory) Adderall XXR Focalin XR GABA B receptor antagonist

KEY LEARNING POINTS • The beneficial role of psychopharmacology is due to its effects on the central nervous system noradrenergic and dopaminergic pathways. • Though CNS stimulants (MPH) are of considerable value, the problems associated with their abuse have become a major bottleneck. • Nonstimulant medication (clonidine, guanfacine), used as alternative or adjacent to CNS stimulants, may produce cardiovascular side- effects. • Tricyclic antidepressants (TCAs) may be of help to patients with ADHD and are usually listed as alternatives to stimulant medications if stimulants are not beneficial or are contraindicated. • There are a variety of medications (dextroamphetamine with constant rate, dopamine agonist, GABA-B receptor antagonist), being released and developed, that have been shown to improve the attention dysfunction of ADHD. • As more research progress takes place, ADHD will be divided into subgroups depending on what part (s) of the central nervous system is responsible for a specific ADHD type. Medications will be designed to work at a specific area(s) of the CNS that is affected. Medications • It should always be clearly understood by clinicians, patients, and their families that medication is only a part of the overall management strategy to improve the lives of our children and adolescents with ADHD. 96 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

REFERENCES 1. Greydanus DE, Pratt HD. Attention-deficit hyperactivity disorder. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. NY and Lincoln, Nebraska:Universe 2006:107-142. 2. Feldman HM, Lee IM. Attention deficit hyperactivity disorder. In: FD Burg, JR Ingelfinger, RA Polin, AA Gershon (eds): Current Pediatric Therapy, 18th edn. Philadelphia: Elsevier 2006:1240-1245. 3. Barkley RA, Fischer M, Smallish L, et al. Young adult outcomes of hyperactive children: Adaptive functioning in major life activities. J Am Acad Child Adolesc Psychiatry 2006: 45:192-202. 4. Greydanus DE, Sloane MA. Psychopharmacology of ADHD in adolescents. Adolesc Med 2002;13:599-624. 5. Greydanus DE. Psychopharmacology of ADHD in adolescents: Quo vadis? Psychiatr Times 2003;20:5-9. 6. Greydanus DE. Pharmacologic treatment of attention-deficit/hyperactivity disorder. Indian J Pediatr 2005; 72:27-34. 7. Greydanus DE, Pratt HD, Sloane MA, et al. Attention-deficit/hyperactivity disorder in children and adolescents: Interventions for a complex costly clinical conundrum. Pediatr Clin North Am 2003;50:1049-1092. 8. Mental Health in the United States. Prevalence of diagnosis and medication for attention-deficit/hyperactivity disorder—United States, 2003. Morb Mort Weekly Rep 2005; 54:842-847. 9. Bradley C. The behavior of children receiving benzedrine. Am J Psychiatry 1937;94:577-85. 10. Jensen PS, Hinshaw SP, Swanson JM, Greenhill LL, Conners CK, et al. Findings from the NIMH Multimodal Treatment Study of ADHD (MTA): Implications and applications for primary care providers. J Dev Behav Pediatr 2001;22:60-73. 11. National Institute of Mental Health (NIMH). Attention deficit hyperactivity disorder. NIMH, NIH Publication No. 01-4589, 2001. Available at: http:// www.nimh.nih.gov/publicat/helpchild.cfm. 12. National Institute of Mental Health. NIMH Research on Treatment for Attention Deficit Hyperactivity Disorder (ADHD): The Multimodal Treatment Study— Questions and Answers. Washington, DC: NIMH, 2000. Available at: http:// www.nimh.nih.gov/events/mtaqa.cfm 13. Swanson JM and MTA Cooperative Group. National Institute of Mental Health Multimodal Treatment Study of ADHD Follow-up: Changes in effectiveness and growth after the end of treatment. Pediatrics 2004;113:762-769. 14. Barkley RA. Attention-deficit/hyperactivity disorder. Sci Am 1998;66-71. 15. Clarke SD. ADHD in adolescence. J Adolesc Health 2000;27:77-78. 16. Solanto MV, Arnsten AFT, Castellanos FX. The neuroscience of stimulant drug action in ADHD. In: Solanto MV, Arnsten AFT, Castellanos FX (eds): Stimulant drugs and ADHD. London: Oxford University Press 2001;355-379. 17. Pliszka SR, Greenhill LL, Crismon ML, et al. The Texas children’s medication algorithm project: Report of the texas consensus conference panel on medication treatment of childhood. Attention-deficit/hyperactivity disorder. Part I. J Am Acad Child Adolesc Psychiatry 2001;39:908-919. 18. Nissen SE. Adhd drugs and cardiovascular risk. N Engl J Med 2006; 354:1445- 1448. 19. Muthukumar T, Schwartz JE, Suthanthiran M. Stimulant medications and Attention-deficit/hyperactivity disorder(Letter). N Engl J Med 2006;354:2294- 2295. Attention Deficit Hyperactivity Disorder: Psychopharmacology 97

20. Staufer WB, Greydanus DE. Attention-devicit/hyperactivity disorder psychopharmacology for college students. Pediatr Clin North Am 2005;52:71-84. 21. Greydanus DE, Van Dyke DH.Neurologic disorders. In: Greydanus DE, Patel DR, Pratt HD (eds): Essential Adolescent Medicine. New York: McGraw-Hill 2006: 235-279. 22. Transdermal methylphenidate (Daytrana) fro ADHD. Med Lett 2006; 48:49-51. 23. Wolraich ML, Wibbelsman CJ, Brown TE. ADHD: A review. Pediatrics 2005;115:749-757. 24. Wender EH. Managing stimulant medication for attention-deficit/hyperactivity disorder: An update. Pediatr Rev 2002;23:234-236. 25. Schubiner H, Robin AL, Young J. Attention-deficit/hyperactivity disorder in adolescent males. Adolesc Med 2003;14:663-676. 26. Rappley MD. ADHD. N Engl J Med 2005;352:123-125. 27. Musser CJ, Ahmann PA, Theye FW, et al. Stimulant use and the potential for abuse in Wisconsin as reported by school administrators and longitudinally followed children. J Dev Behav Pediatr. 1998 ;19:187-192. 28. McCabe SE, Teter CJ, Boyd CJ. The use, misuse and diversion of prescription stimulants among middle and high school students. Subst Use Misuse 2004;39:1095-1116. 29. Poulin C. Medical and nonmedical stimulant use among adolescents: From sanctioned to unsanctioned use. CMAJ. 2001;165:1039-1044. 30. Carroll BC, McLaughlin TJ, Blake D. Patterns and knowledge of nonmedical use of stimulants among college students. Arch Pediatr Adolesc Med 2006; 160:481- 485. 31. McCabe SE, Knight JR, Teter CJ. Non-medical use of prescription stimulants among US college students: Prevalence and correlates from a national survey. Addiction 2005;100:96-106. 32. McCabe SE, Boyd CJ. Sources of prescription drugs for illicit use. Addict Behav 2005;30:1342-1350. 33. Johnston LD, O’Malley PM, Bachman JG. Monitoring the Future National Survey Results on Drug Use, 1975-2002. II. College Students and Adults Ages 19-40. NIH publication No. 03-5376. Washington, DC: US Department of Health and Human Services 2003. 34. Teter CJ, McCabe SE, Cranford JA, et al. Prevalence and motives for illicit use of prescription stimulants in an undergraduate student sample. J Am Coll Health. 2005 ;53:253-262. 35. Hall KM, Irwin MM, Bowman KA, Frankenberger W, et al. Illicit use of prescribed stimulant medication among college students. J Am Coll Health. 2005; 53:167- 174. 36. Graff Low K, Gendaszek AE. Illicit use of psychostimulants among college students:A preliminary study. Psychol Health Med 2002; 7:283-287. 37. Upadhyaya HP, Rose K, Wang W, et al. Attention-deficit/hyperactivity disorder, medication treatment, and substance use patterns among adolescents and young adults. J Child Adolesc Psychopharmacol. 2005;15:799-809. 38. Varley C. Sudden death of a child treated with imipramine. J Child Adolesc Psychopharmacol 2000;10:321-325. 39. Varley CK. Sudden death related to selected tricyclic antidepressants in children: Epidemiology, mechanisms and clinical implications. Pediatr Drugs 2001;3:613- 627. 40. Greydanus DE, Patel DR: The adolescent and substance abuse: Current concepts. Curr Probl Pediatr Adolesc Health Care 2005; 35 (3): 78-98. 98 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

41. Atomoxetine: Strattera revisited. Med Lett 2004;46:65. 42. Turner DC, Clark L, Downson J, et al. The use of modafinil for Adult ADHD. Biol Psychiatry 2004; 15:1031-1040. 43. Konofal E, Arnulf I, Lecendreux M, Mouren MC. Ropinirole in a child with attention-deficit hyperactivity disorder and restless legs syndrome. Pediatric Neurol 2005; 32(5): 350-351. 44. Greydanus DE, Pratt HD. Attention deficit hyperactivity disorder. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. New York: Lincoln/ Nebraska:Universe 2006: 107-142. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Childhood Disintegrative Disorder 9 and Rett’s Syndrome

Parthasarathy Biswas, Nitin Gupta

INTRODUCTION Autistic spectrum disorders are defined by the early onset of a constellation of difficulties in reciprocal social interaction, communication and restricted, repetitive behaviors or interest. They are particularly unique because of the marked consistency across individuals in some behaviors, and marked differences across others. Over the last three decades, clinical and research evidence has shifted the emphasis from a narrowly defined concept to a broader range of autistic disorders (that share the core deficits associated with the original concept but in which the degree of disability may vary considerably). Childhood disintegrative disorder (CDD) and Rett’s syndrome (RS) fall in this spectrum. Nearly a century ago, Theodore Heller, reported six children’s who had severe developmental regression at ages of 3-4 following a period of apparent normal development. Since these children had very poor developmental recovery after the onset of the illness, Heller1 termed this condition as dementia infantilis. Over the years, the concept of CDD has been variously named but just about over 100 cases have been reported in world literature. Information on this condition is much more limited as compared to autism.2 RS is one of the very few disorders which have generated considerable interest as well as controversy. Andrew Rett3 first described this syndrome as consisting of stereotyped hand movements, dementia, autistic behavior, ataxia, cortical atrophy and hyperammonemia occurring specifically in females. The syndrome was, therefore described as “cerebral atrophy with hyperammonemia”. Following the pioneering work of Hagberg and coworkers,4 RS has now been recognized as a separate clinical entity. Given 100 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics the rarity of these two disorders, coupled with lack of consensus amongst clinicians, many cases may have been overlooked or misdiagnosed.5 This chapter reviews the development of diagnostic concept, current definitions, clinical features, epidemiology, course and outcome and the differential diagnosis of these two unique but rare entities. Also discussed are the various management modalities for these two conditions besides the valuable contribution of Indian clinicians to the understanding of these rare disorders.

DEVELOPMENT OF DIAGNOSTIC CONCEPT The diagnostic debate initially centered on issues of continuity with schizophrenia and more recently with autism. Until the 1970s, researchers and clinicians grouped autistic disorders under a single category of childhood “psychotic” disorder. Pioneering studies by Kolvin6 helped separate this disorder into one with problems starting at birth or within one year of their birth and the other (later-onset) group apparently having a normal development for many years. The symptoms in the earlier-onset type were similar to those described by Kanner. The family history also suggested that this there is an increased incidence of schizophrenia in the later-onset type but not in the earlier-onset types.7 This led to the categorization of autism as a separate entity in the DSM- III8 for the first time under the diagnostic category of pervasive developmental disorders (PDD). Children who were described as having disintegrative psychosis in ICD-99 were similar to what was described in DSM-III-R10 for autistic disorder. Current nosological systems, i.e. ICD- 1011 and DSM-IV,12 have included CDD under the broad rubric of PDD, which suggests that CDD and typical autism (TA) have some core criteria in common. However, a close look at the diagnostic criteria reveals that these two distinct entities do not match on the criterion of period of normal development.13 Moreover, CDD is a rare disorder and is less researched in areas of biological parameters, treatment response, course and outcome as compared to TA.5,14 Both the ICD-1011 and DSM-IV12 have included RS under PDD and have adopted 5 months as the upper age level for normal psychomotor development and the lower age limit for purposeful hand skills. This coincides with the 5 months as the lower limit of appearance of deceleration of head circumference. The Rett’s Syndrome Diagnostic Work Group15 Childhood Disintegrative Disorder and Rett’s Syndrome 101 had mentioned that head circumference might be normal till 18 months, an important fact that is not mentioned either in the DSM-IV 12 or ICD-10.11 The major shortcoming of the classificatory systems, however, is the absence of any exclusion criteria and associated features. Though RS is described only in girls, however there are several case reports of Rett’s like syndrome in boys. Male sex is not an exclusion criterion as of now.

EPIDEMIOLOGY Epidemiological data on CDD is very sparse reflecting the relative rarity of the disorder and the lack of familiarity of clinicians with the concept leading onto cases being underdiagnosed. Though not on a truly epidemiological sample, yet the disorder was found to have a frequency of one-tenth of that of TA.16 The prevalence rate of CDD has been found to be about 1 in 10,000 children.17 The male preponderance seen in CDD is similar to those seen in TA. RS has been reported from all over the world, and been found to display familial relationship.18 However, unlike inborn errors of metabolism; RS does not show strong geographic, ethnic and familial accumulations. The prevalence of RS was found to be 1 in 15,000 live female births.19,20 In an American study RS was found to have a prevalence of 1 in 22,800 live female births.21 In a recent review,22 4 surveys (between 1966 and 2000) yielded estimates for CDD ranging from 1.1 to 6.4 per 100,000 subjects. A pooled estimate across these four surveys is 1.7 per 100,000. The most recent review showed that the prevalence of CDD is about 0.2 per 10,000.23 The conclusion is that CDD is very rare disorder and its prevalence is 60 times less than that for autistic disorder, assuming a prevalence of 10 per 10,000 for autism.24

CLINICAL FEATURES As per ICD-1011 criteria, CDD is characterized by the following: A. Development is apparently normal up to the age of at least 2 years. The presence of normal age-appropriate skills in communication, social relationship, play, adaptive behavior at age of 2 years or later is required for diagnosis. B. There is definitive loss of previously acquired skills at about the time of onset of the disorder. The diagnosis requires a clinically significant 102 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

loss of skills (not just failure to use them in certain situations) in at least two of the following areas: 1. Expressive or receptive language 2. Play 3. Social skills or adaptive behavior 4. Bowel or bladder control 5. Motor skills. C. Qualitatively normal social functioning is manifested in at least two of the following areas: 1. Qualitative abnormalities in reciprocal social interaction (of the type defined for autism) 2. Qualitative abnormalities in communication (of the type defined for autism) 3. Restricted, repetitive and stereotyped patterns of behavior, interests and activities including motor stereotypies and mannerisms 4. A general loss of interest in objects and in the surroundings. D. The disorder is not attributable to the varieties of pervasive developmental disorders; acquired aphasia with epilepsy; elective mutism; RS; or schizophrenia. The ICD-10 11 criteria for RS are characterized by the following: 1. There is an apparently normal prenatal and perinatal period and apparently normal psychomotor development through 5 months and normal head circumference at birth 2. There is deceleration of the head growth between 5 months and 4 years and loss of acquired purposeful hand skills between 5 and 30 months of age that is associated with concurrent communication dysfunction and impaired social interactions and the appearance of poorly coordinated/unstable gait and/or trunk movements 3. There is severe impairment of expressive and receptive language, together with severe psychomotor retardation 4. There are stereotyped midline hand movements (such as hand wriggling and hand-washing) with onset at or after the time when purposeful movements of the hands are lost. However, the ICD-1011 criteria do not take into account the supportive or the exclusionary criteria necessary for distinguishing RS from other closely related disorders. Recently, Clarke25 has formulated criteria for the clinical diagnosis of RS, which have been grouped as necessary and supportive criteria. Childhood Disintegrative Disorder and Rett’s Syndrome 103

The supporting criteria as per the Rett’s Syndrome Diagnostic Criteria Work Group15 are as follows: 1. Breathing dysfunction a. Periodic apnea during wakefulness b. Intermittent hyperventilation c. Breath-holding spells d. Forced expulsion of air or saliva. 2. EEG abnormalities a. Slow walking background and intermittent rhythmic slowing (3-5 Hz) b. Epileptiform discharges, with or without clinical seizures 3. Seizures 4. Spasticity, often associated with development of muscle wasting and dystonia 5. Peripheral vasomotor disturbances 6. Scoliosis 7. Growth retardation 8. Hypotrophic small feet. The exclusionary criteria as per the Rett’s Syndrome Diagnostic Criteria Work Group15 are as follows: 1. Evidence of intrauterine growth retardation 2. Organomegaly, or other signs of storage disease 3. Retinopathy or optic atrophy 4. Microcephaly at birth 5. Evidence of perinatally acquired brain damage 6. Existence of identifiable metabolic or other progressive neurologic disorder 7. Acquired neurological disorder resulting from severe infections or head trauma. To facilitate the characterization of this disorder a 4-stage system was proposed by Hagberg and Witt-Engestrom26 and adopted later by Rett’s Syndrome Diagnostic Criteria Work Group.15 The four stages are: 1. Early onset stagnation phase 2. Rapid destruction stage 3. Pseudostationary stage 4. Late motor degeneration stage 104 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

DIFFERENTIAL DIAGNOSIS RS: Before discussing about the prominent differential diagnosis, it would be important to mention about some clinical variants of RS. Two clinical variants of RS have been recognized in the literature (atypical form and forme fruste) though they do not fulfill all the diagnostic criteria.27 The atypical form shows severe seizure disorder, developmental delays prior to regression or lack stereotypes/maintained purposeful hand movements or both.28 Girls, who have forme fruste have only subtle initial symptoms and retain some language skills, do not have hand stereotypies, and have fewer developmental delays. However, by the time they grow up they clinically resemble like the older RS subjects.29 The most common differential diagnosis of RS is TA. However, while autism involves regression of only verbal skills, RS may involve not only verbal skills but also motor skills.30 Moreover, normal development can be seen till 6-18 months while typical autism has onset from the early infancy. The stereotypic behavior in autism, if present, is always more complex while RS always has stereotypic hand movements. RS also has acquired microcephaly, profound mental retardation, seizures (70% of the cases), breathing dysfunctions, ataxia, bruxism, loss of purposeful hand movements, choreoathetoid movements/dystonic movements, and poor eye contact. These features make RS very distinct from TA. At early stages RS may be indistinguishable from Infantile Neuronal Ceroid Lipofuscinosis (INCL), an autosomal recessive neurodegenerative disease; however, later myoclonus and retinal degeneration differentiates it from RS.31 RS has been linked to tuberous sclerosis, a neurocutaneous disorder. However examination of the skin and presence of typical abnormalities in serial CT scans distinguishes this from RS. Other syndromes with features similar to RS are Angelman syndrome (no normal period of development and subsequent regression), Landau-Kleffner syndrome (head circumference and motor skills are preserved) and acute/chronic encephalitis (typical CSF findings).

CDD: The differential diagnosis of CDD includes the entire PDD spectrum because it shares the essential features of autism. Concerns have been expressed about the diagnostic criteria for CDD and TA.45 However, a closer assessment of the case may reveal a normal development till 18-24 months. RS can be occasionally confused with CDD as some amount of Childhood Disintegrative Disorder and Rett’s Syndrome 105 regression is seen in both the disorders. In fact some authors have suggested that RS may well be a variant of CDD.17, 32 However, a closer look at these two conditions reveals that they have distinct presentations. Asperger’s syndrome has relatively preserved cognitive functions and severe regression does not occur in these cases. Similarly, very early onset schizophrenia (VEOS) also lacks the regression seen in CDD besides having characteristic delusions and hallucinations on serial mental status examinations. Medical conditions like Landau-Kleffner syndrome, gangliosidosis, Niemann-Pick Disease and metachromatic leukodystrophy may have an autistic-like picture. However, CDD and RS should be diagnosed tentatively at first presentation and only after regular follow- ups should a definitive diagnosis should be made.

ETIOLOGY Neurochemistry and Neuropathology There is a marked scarcity of information regarding neurochemistry of CDD. The current research indicates that elevation of brain derived neurotrophic factor (BDNF), and presence of autoantibodies (AAs) to endothelial cells (ECs) and myelin basic protein (MBP) suggests that the interaction between the immune mechanism and BDNF may be of etiological significance in CDD.33 Research has been going on regarding the relation between the role of biogenic amines and endorphins in the etiology of RS. The hypothesis that has the maximum evidence is: abnormality in the dopamine neurotransmitter system, evidenced by reductions in CSF levels of homovalinic acid- a dopamine metabolite.34 Other neurochemicals which have been implicated are choline acetyltransferase (ChAT), β endorphins35 and CSF glycolipids like GD1a and GD1b; the latter has been the focus of recent research as they have been found to be selectively decreased in cases with RS.36 Some studies on the neuropathology of RS suggest that there is evidence of diffuse brain atrophy and decreased brain weight, 37, 38 which seemed to be related to the duration of illness; however no signs of active degenerative disease were noticed. Some of the neuropathological findings include mild gliosis in the basal ganglia,39 lack of pigmentation in the substantia nigra and locus ceruleus40 and abnormal neuritis in the frontal cortex and caudate nucleus41 all of which point towards an arrest 106 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics or retardation of maturation of neurons. These findings are further supported by the fact that the nerve growth factor (NGF) in CSF of children with RS was less than in normal controls.42 It has been postulated that the lowered levels of NGF may be involved in the pathogenesis of the disorder or it may reflect the underlying damage.27 Biopsy of the brain showed inclusion bodies in the neuronal mitochondria.43

Neurological Aspects EEG abnormalities have been seen in 25% of cases of CDD. 13, 14, 44-46 Malhotra and Singh13 had reported that the developmental deterioration in CDD was associated with seizures. There is marked scarcity of observations on neuropsychology, neurophysiology and neuroanatomy of CDD. Very high percentages (84%) of girls with RS have seizures, 47 which are either GTCS or CPS.47, 48 There is slowing and disorganization of background activity in the awake state. Multifocal epileptiform discharges maximal over the central and temporal regions, and intermittent, high amplitude discharges followed by relative attenuation of background activity are seen during sleep.49

Genetics The information on genetics of these disorders is also very limited. Several attempts to find a genetic basis have been inconclusive. It is believed that RS may be due to a non-random pattern X-chromosome inactivation.50 Since RS is considered to be X-linked dominant, therefore it is lethal in hemizygous males. More than 95% cases of this syndrome are, however, sporadic. Yet another hypothesis is the two-step mutation51 where it has been proposed that the girls with RS have an inherited mutated gene in the X chromosome and a somatic mutation at the same locus in the other X chromosome. Other hypothesis includes mitochondrial inheritance,52 interference of an autosomal modifying gene in addition to the X- chromosome “Rett” gene,53 Ornitine carbamoyltransferase (OCT) gene locus as a marker gene54 and Fragile X site.55 Genetic linkage and association studies have implicated the involvement of nearly every chromosome in the human genome in autism spectrum disorder. Recently, in 1998, by performing chromosome linkage analysis on the few available familial cases, the region of interest was localized to Xq286.56 In 1999, it was Childhood Disintegrative Disorder and Rett’s Syndrome 107 discovered that RS is associated with a mutation in the MECP2 gene on the X chromosome.57 However, the most consistently replicated linkage findings in CDD have been on chromosome 7q, 2q, and 15q. The positive associations from candidate gene studies are largely unreplicated, with the possible exceptions of the GABRB3 and serotonin transporter genes.58

Medical Conditions Associated with CDD and RS These include tuberose sclerosis, neurolipidosis, metachromatic leukodystrophy, Addison-Schilder’s disease and SSPE. A recent study detected a prolonged QT interval in 9 out of 34 cases of RS on ECG.59 These evidences suggest that CDD14 and RS may be more than likely to be neurobiological in origin. However, neuropathological changes and their relation to neurochemistry remain unknown.

ASSESSMENT Evaluation of CDD and RS should be multi-disciplinary.

Investigations Exploration into the birth history, early developmental history, medical history and family history are of paramount importance. Particular emphasis should be given to evaluation for neurodegenerative disorders and errors of amino acid metabolism. EEG and CT scans should be done. ECG must be done in every child with RS since prolonged QT syndrome is a serious condition with a risk of sudden death.59

Developmentally Based Assessments These include tests for communication and cognitive abilities and should be applied depending on the level of functioning. Developmental tests used to assess such children include Scale for Infant Psychological Development.60 Communication scales used in such cases are Receptive-expressive Emer- gent Language (REEL) Scale,61 Sequenced Inventory of Communicative Development,62 and Reynell Development Language Scale.63

Behavioral Assessments The Vineland Adaptive Behavior Scale64 and Bayley Scales of Adaptive Abilities65 has been widely used to document the adaptive behavior. This 108 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics scale can act as a diagnostic tool as well. Playroom observations can also help in both diagnosis as well as behavioral assessment of the case.

Diagnostic Assessments One of the important diagnostic tools is the PDD Behavior Inventory (PDDBI).66 It is a rating scale filled out by caregivers or teachers that was designed to assess children having a PDD (Autism, Asperger disorder, PDD-NOS, or CDD). Both adaptive and maladaptive behaviors are assessed in the scale, making it useful both as a diagnostic and prognostic tool.

INTERVENTIONS Enhancing Language and Communication There are very few existing interventional programs and available literature on the communication and language deficit in RS and CDD. Children with RS can be taught non-verbal gestures like eye pointing, communication boards (pictures), facial expressions and gestures.67

Behavioral Interventions There is some evidence that behavioral interventions may be helpful in management of cognitive arrest/stagnation and movement disorder.68 Cognitive skills training done over 3 years has been found to be effective in RS.69 Other behavioral interventions aimed at improving gait and movement abnormalities involve use of therapy ball, balance-stimulating floor activities, segmental rolling and rotation and weight shifting activities.70

Family Interventions Family interventions in form of psychoeducation regarding illness, support groups, group meetings and availability of adequate local resources may be helpful.5

Rehabilitation Rehabilitation strategies should include placement in special schools/ educational settings. Main targets should be development of certain basic Childhood Disintegrative Disorder and Rett’s Syndrome 109 communication skills and ambulation in form of independent standing skills. However, the rehabilitation of these children has been difficult with only minimal success.70

Psychopharmacology RS: Once a diagnosis of RS is made in a child, it should be presumed to be an irreversible disorder. Most of the pharmacological treatments have been used on empirical basis with minimal success. Some of the medications that have been tried are L-dopa,71 magnesium citrate,72 L-carnitine73 and naltrexone.74 In cases of seizures, standard doses of carbamazepine have been found to be effective. Adrenocorticotrophic hormone and prednisolone has been found to be effective in infantile spasms.31 The growth retardation seen in RS is more likely to be due to nutritional factors rather than anything else.27 Dietary measures (ingestion of high-liquid- content fruit, fiber, mineral oil, etc.) may be helpful but at times laxatives, enemas and suppositories are often required. Orthopedic interventions like splinting have been found to be effective in interrupting hand-to- mouth and hand wriggling movements.75, 76 However, there are several studies where these interventions have not produced the desired results.

CDD: There is no specific treatment for children with CDD and the drugs that have been used in autism have been used for such cases as well. Antipsychotics like olanzapine and other serotonin-dopamine antagonists have been used in CDD with limited success.77, 78

COURSE AND PROGNOSIS There are very few long-term studies on course and outcome of these two disorders. In 2/3rd of the cases of CDD, however, there is deterioration but after that it stabilizes. However, in rest of the cases, after the marked developmental regression there is only partial recovery.16 Few cases may however, show a developmental deterioration, which continues even after several years.79 Life expectancy appears to be normal. However, the prognosis is certainly worse than autism.80

INDIAN DATA Very few cases of RS have been reported in Indian literature. 46, 81, 82 Similarly there are very few studies reporting cases of CDD in India.13, 83, 84 The clinic 110 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics based prevalence of CDD ranges from 0.22-0.45%.13, 45 The male female ratio is about 5:1 which is similar to western population. Attempt has been made on trying to validate CDD as a clinically distinct disorder from TA.45

CONCLUSIONS Both CDD and RS are rare disorders, which though subsumed to be ‘functional illnesses’, are more ‘organic’ in etiology/origin. They, in fact, should probably be of great interest to pediatricians. It may be helpful to develop special ‘developmental’ clinics run by pediatricians in close liaison with psychiatrists (and other mental health professionals). This should help not only in a more comprehensive approach to diagnosing and clinically managing such disorders, but also in conducting research to develop a further in-depth understanding of such developmental disorders.

KEY LEARNING POINTS • CDD and RS are very rare disorders. • Due to scarcity of literature, there is lack of awareness and expertise amongst physicians and mental health professionals. • Though subsumed under the broad category of PDD, these disorders are unique entities in their own right and different from typical autism on several accounts. • In absence of any specific laboratory marker, accurate diagnosis depends upon careful analysis of clinical presentation and signs/ symptoms. • The underlying neurobiological basis is still not fully understood but it appears to be due to arrested neurodevelopment rather than being neurodegenerative. • Future research is required to understand several aspects like etiopathogenesis and treatment, with the ultimate goal of prevention of these disorders (possibly by identification of genetic/biochemical markers). • Specialized’ developmental clinics’ are needed with close liaison between pediatricians and mental health professionals. Childhood Disintegrative Disorder and Rett’s Syndrome 111

REFERENCES 1. Heller T. Dementai infantilis. Zeitschrift fur Kinderforschung 1908; 2: 141-165. 2. Volkmar FR. Childhood disintegrative disorder: Issues for DSM-IV. J Autism Dev Disord 1992; 22: 625-642. 3. Rett A. Ein zerebral-atrophisches syndrom bei hyperammonaemia. Wein Med Wochenscher 1966; 116: 723-738. 4. Hagberg B, Aicardi J, Dias K, Ramos O. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett syndrome: Report of 35 cases. Ann Neurol 1983; 14: 471-479. 5. Volkmar FR, Klin A, Mrans W, Cohen DJ. Childhood disintegrative disorder. In: Cohen DJ, Volkmar FR (eds): Handbook of Autism and Pervasive Developmental Disorders, 2nd edn. New York: Wiley 1997: 47-59. 6. Kolvin I. Studies in childhood psychosis: 1. Diagnostic criteria and classification. Br J Psychiatry 1971; 118: 381-384. 7. Volkmar FR, Cohen DJ, Hoshino Y, Rende RD, Paul R. Phenomenology and classification of the childhood psychoses. Psychol Med 1988; 18 : 191-201. 8. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders,3rd edn. Washington DC: APA, 1980. 9. World Health Organization (WHO). International Classification of Mental and Behavioural Disorders, 9th edn. Geneva: WHO 1978. 10. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 3rd edn Washington DC: APA 1987. 11. World Health Organization (WHO). International Classification of Mental and Behavioural Disorder,10th edn. Geneva: WHO 1992. 12. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th edn. Washington DC: APA 1994 13. Malhotra S, Singh S. Disintegrative psychosis of childhood: An appraisal and case study. Acta Pedopsychiatr 1993; 56: 37-40. 14. Malhotra S, Gupta N. Childhood disintegrative disorder. J Autism Dev Disord 1999; 29: 491-498. 15. Rett’s Syndrome Diagnostic Work Group: Diagnostic criteria for Rett’s syndrome. Ann Neurol 1988; 23: 125-128. 16. Volkmar FR, Cohen DJ. Disintegrative disorder or ‘late-onset autism’? J Child Psychol Psychiatry 1989; 30: 717-724. 17. Burd L, Fisher W, Kerbeshian J. Pervasive developmental disorder: Are Rett’s syndrome and Heller dementia infantilis subtypes? Dev Med Child Neurol 1989; 31:609-616. 18. Zoghbi HY. Genetic aspects of Rett Syndrome. J Child Neurol 1988; 3 (Suppl.): S76-S78. 19. Hagberg B. Rett syndrome: Swedish approach to analysis of prevalence and cause. Brain Dev 1985; 7: 277-280. 20. Kerr AM, Stephenson JBP. A study of natural history of Rett’s Syndrome in 23 girls. Am J Med Genet 1986; 24 (Suppl. 1), 369-376. 21. Kozinetz CA, Skender ML, MacNaughton N, et al. Epidemiology of Rett’s syndrome: A population-based registry. Pediatrics 1993; 91: 445-450. 22. Fombonne E. Prevalence of childhood disintegrative disorder. Autism 2002; 6:149- 157. 23. Fombonne E. Epidemiology of autistic disorder and other pervasive developmental disorders. J Clin Psychiatry 2005; 10 (66 Suppl.): 3-8. 112 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

24. Merrick J, Kandel I, Morad M. Trends in autism. Int J Adolesc Med Health 2004; 16: 75-78. 25. Clarke A. Rett’s syndrome. J Med Genet 1996; 33: 693-699. 26. Hagberg B, Witt-Engestrom I. Rett’s syndrome: A suggested staging system for describing impairment profile with increasing age towards adolescence. Am J Med Genet 1986; 24 (Suppl. 1): 47-59. 27. Van Acker R. Rett’s syndrome: A pervasive developmental disorder. In Cohen DJ, Volkmar FR (eds) Handbook of Autism and Pervasive Developmental Disorders. 2nd edn. New York: Wiley 1997: 60-93. 28. Goutieres F, Aicardi J. Atypical forms of Rett’s syndrome. Am J Med Genet 1986; 24: 183-194. 29. Akesson HO, Hagberg B, Wahlstrom J. Rett’s syndrome: Classical and atypical: Genealogical support for a common origin. J Med Genet 1996; 33: 764-766. 30. Percy AK, Zoghbi HY, Lewis KR, Jankovic J. Rett’s syndrome: Qualitative and quantitative differentiation from autism. J Child Neurol 1988; 3 (Suppl.): S65-S67. 31. Sekul EA, Percy AK. Rett’s syndrome: Clinical features, genetic considerations, and the search for a biological marker. Curr Neurol 1992; 12: 173-198. 32. Millichap JG. Rett’s syndrome: A variant of Heller’s dementia? Lancet 1987; 1: 440-445. 33. Collony AM, Chez M, Streif EM, et al. Brain-derived neurotrophic factor and autoantibodies to neural antigens in sera of children with autistic spectrum disorders, Landau-Kleffner syndrome, and epilepsy. Biol Psychiatry 2006; 59: 354-363. 34. Zoghbi HY, Milstien S, Butler IJ, et al. Cerebrospinal fluid biogenic amines and biopterin in Rett’s syndrome. Ann Neurol 1989; 25: 56-60. 35. Gillberg C, Terenius L, Hagberg B, Witt-Ingerstrom I, Erickson I. CSF b- endorphins in childhood neuropsychiatric disorders. Brain Dev 1990; 12: 88-92. 36. Lekman A, Hagberg B, Svennerholm BA. Membrane cerebral lipids in Rett’s syndrome. Pediatr Neurol 1991; 7: 186-190. 37. Harding BN, Tudmay AJ, Wilson J. Neuropathological studies in a child showing some features of the Rett’s syndrome. Brain Dev 1985;7:342-344. 38. Jellinger K, Seitberger F. Neuropathology of Rett’s syndrome. Am J Med Genet 1986; 24 (Suppl. 1):259-288. 39. Lekman A, Witt-Ingerstrom I, Gottfries J, Hagberg BA, Percy AK, Svennerholm L. Rett’s syndrome: Biogenic amines and metabolites in postmortem brain. Pediatr Neurol 1989; 5 : 357-362. 40. Brucke T, Sofic E, Killman W, Rett A, Reiderer P. Reduced concentrations and increased metabolism of biogenic amines in a single case of Rett’s syndrome: A post-mortem brain study. J Neural Trans 1988; 68: 315-324. 41. Jellinger K, Armstrong D, Zoghbi HY, Percy AK. Neuropathology of Rett’s syndrome. Acta Neuropathol 1988; 76: 142-158. 42. Lappalainen R, Lindholm D, Riikonen R. Low levels of nerve growth factor in cerebrospinal fluid of children with Rett’s syndrome. J Child Neurol 1996; 11: 296-300. 43. Cornford ME, Philippart M, Jacobs B, Scheibel AB, Vinters HV. Neuropathology of Rett’s syndrome: Case Report with neuronal and mitochondrial abnormalities in the brain. J Child Neurol 1994;9: 424-431. 44. Volkmar FR, Rutter M. Childhood disintegrative disorder: results of DSM-IV autism field trial. J Am Acad Child Adolesc Psychiatry 1995;34: 1092-1095. 45. Malhotra S, Gupta N. Childhood disintegrative disorder-Re-examination of current concept. Eur Child Adolesc Psychiatry 2002;11: 108-114. Childhood Disintegrative Disorder and Rett’s Syndrome 113

46. Malhotra S, Kumar D, Gupta N. Rett’s syndrome—A Neurodevelopmental Disorder: Report of two cases. Neurol India 2002; 50: 330-333. 47. Naidu S, Murphy M, Moser HW, Rett A. Rett’s syndrome: Natural history in 70 cases. Am J Med Genet 1986; 24 (Suppl. 1): 61-72. 48. Iyama CM. Rett’s syndrome. Adv Pediatrics 1993; 40: 217-245. 49. Trauner DA, Haas RH. Electroencephalographic abnormalities in Rett’s syndrome. Pediatr Neurol 1987; 3: 331-334. 50. Comings DE. The genetics of Rett’s syndrome: The consequences of a disorder where every case is a new mutation. Am J Med Genet 1986; 24 (Suppl. 1): 383-388. 51. Wahlstrom J, Anvert M. Chromosome findings in thr Rett’s syndrome and a test of a two-step mutation theory. Am J Med Genet 1986; 24 (Suppl. 1): 361-368. 52. Eeg-Olofsson O, Al-Zuhair AGH, Teebi AS, Al-Essa MMN. Rett’s syndrome: Genetic clues based on mitochondrial changes in muscle. Am J Med Genet 1989; 32: 142-144. 53. Carlin ME, Arena JFP, Ing PS. Rett’s syndrome seen in association with a complex chromosomal translocation: Possible mapping to the short arm of the chromosome X. Am J Human Genet 1989; 45: A73. 54. Thomas S, Hjelm, Oberholzer V, Brett EM, Wilson J. Rett’s syndrome and ornithine carbamoyltransferase deficiency. Lancet 1987; 2: 1330-1331. 55. Wahlstrom J, Witt-Engerstrom I, Mellquist L, Anvert M, Oden A. The Rett’s syndrome related to fragile X (P22) in caffeine-induced lymphocyte culture. Brain Dev 1990; 12: 128-130. 56. Siriami N, Naidu S, Periera JL, Pillotto RF, Hoffman EP—Rett’s syndrome: confirmation of X-linked dominant inheritance and localization of the gene to Xq28. Am J Med Genet 1998; 63: 1552-1558. 57. Amir RE, vanden Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett’s syndrome is caused by mutations in X-linked MEPC2, encoding methyl-CpG- binding protein 2. Nat Genet 1999; 23: 185-188. 58. Santangelo SL, Tsatsanis K. What is known about autism: Genes, brain, and behavior. Am J Pharmacogenomics. 2005; 5: 71-92. 59. Ellaway CJ, Sholler G, Leonard H, Christodoulou J. Prolonged QT interval in Rett’s syndrome. Arch Dis Child 1999; 80: 470-472. 60. Uzgiris IC, Hunt JM. Assessment in infancy: Ordinal scales of psychological development. Urbana: University of Illinois Press 1975. 61. Bzoch K, League R. Receptive-Expressive Emergent Language Scale. Gainesville, FL: Computer Management Corporation: Language Education Division 1971. 62. Hendrick D, Prather F, Tobin A. Sequence Inventory of Communicative Development. Seattle: University of Washington Press 1975. 63. Reynell J, Gruber C. Reynell development language scales (US edn). Los Angeles: Western Psychological Services 1990. 64. Sparrow SS, Balla D, Cicchetti DV. Vineland Adaptive Behavior Scales (Expanded Form). Circle Pines: American Guidance Service 1984. 65. Bayley N. Bayley Scales of Infant Development. New York: Psychological Corporation, 1969. 66. Cohen IL, Schmidt-Lackner S, Romanczyk R, Sudhalter V. The PDD Behavior Inventory: A rating scale for assessing response to intervention in children with pervasive developmental disorder. J Autism Dev Disord 2003; 33: 31-45. 67. Van Acker R, Grant SB. An effective computer-based requesting system for persons with Rett’s syndrome. J Child Com Dis 1995; 16: 31-38. 68. Stephenson JB, Kerr SB. Rett’s syndrome: Disintegration not dementia. Lancet 1987; 1: 741-744. 114 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

69. Woodyatt GC, Ozanne AE. A longitudinal study of cognitive skills and communication behaviors in children with Rett’s syndrome. J Intellect Dis Res 1993; 37: 419-435. 70. Hanks SB. Motor disabilities in the Rett’s syndrome and physical therapy strategies. Brain Dev 1990; 12: 157-161. 71. Percy AK, Schultz R, Glaze DG, et al. Trial of opiate antagonist, naltrexone, in children with Rett’s syndrome. Ann Neurol 1991; 30: 486-159. 72. Egger J, Hofacker N, Schiel W, Holthausen H. Magnesium for hyperventilation in Rett’s syndrome. Lancet 1992; 340: 621-622. 73. Plioplys AV, Kasnicka I. L-carnitine as a treatment for Rett’s syndrome. South Med J 1993; 86: 1411-1413. 74. Matsuishi T, Urabe F, Percy AK, et al. Abnormal carbohydrate metabolism in cerebrospinal fluid in Rett’s syndrome. J Child Neurol 1994; 9: 26-30. 75. Hanks SB. The role of therapy in Rett’s syndrome. Am J Med Genet 1986; 24 (Suppl. 1): 247-252. 76. Aron M. The use and effectiveness of elbow splints in the Rett’s syndrome. Brain Dev 1990; 12: 162-163. 77. McDougle CJ, Holmes JP, Bronson MR, Anderson GM, Volkmar FR, Price LH, Cohen DJ. Risperidone treatment of children and adolescents with pervasive developmental disorders: A prospective open-label study. J Am Acad Child Adolesc Psychiatry 1998 ; 37: 15-16. 78. Kaidar M, Zalsman G. Olanzapine for childhood disintegrative disorder. Isr J Psychiatry Relat Sc 2004; 41: 71-72. 79. Burd L, Ivey M, Barth A, Kerbeshian J. Two males with childhood disintegrative disorder: A prospective 14-year-outcome study. Dev Med Child Neurol 1998; 400: 702-707. 80. Mouridsen SE. Childhood disintegrative disorder. Brain Dev 2003; 25: 225-228. 81. Kalra V, Sud DT. Rett’s syndrome. Indian Pediatr 1994; 31: 711-715. 82. Ghofrani M, Mahmoodian T. Rett’s syndrome. Indian J Pediatr 2000; 67: 539-540. 83. Jaydeokar S, Bal G, Shah N. Childhood disintegrative disorder. Indian J Psychiatry 1997; 39: 85-87. 84. Agarwal V, Sitholey P, Mohan I. Childhood disintegrative disorder - An atypical presentation: A case report. J Autism Dev Disord 2005; 8:1-2. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Autism and Related Disorders: 10 Regression of Milestones

Shabina Ahmed

INTRODUCTION An adult is a culmination of series of orderly and dynamic changes both in physical and mental functioning and all this takes place is a very standardized and predictable manner. Any deviation, delay or aberrant growth curve in achievement of milestones signifies a constitutional problem or a response to stress. In the process of development there are two ways the child lags behind from the norm; one is called delay—where the child has not been able to achieve his milestones in the stipulated time and another one is called regression—where the child did achieve normal milestone for a period of time and then started deceleration. In regression deceleration may be evident before the age of two years or may be delayed; sometimes, a maturing infant might move forward in a particular behavior pattern but then may regress and show an inclination to return to a previous more immature behavior, as in after learning how to walk the child might elect to crawl again. This is a short term regression.

PATTERN OF INFANT DEVELOPMENT Developmental milestone assessment covers areas of functioning that include motor and adaptive behavior, activity level, verbal communication, personal and social behavior, and emotional and autonomic regulation.

MOTOR AND ADAPTIVE BEHAVIOR These are intimately combined in early life, because under the pressure of growth, a normal infant feels compelled to put each newly attained motor ability to repetitive use, and to exercise it with experimental variations. Then by subtle growth stages the motor activities start having voluntary purposeful movements. 116 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

LANGUAGE DEVELOPMENT Language development has a motor basis. It is initially mediated by postures, facial expressions, manual gestures and vocalizations. At four weeks he produces throaty sounds, at eight weeks he vocalizes single vowel sounds and then stage by stage consonant sounds and monosyllables appear. At first year he imitates sounds, by 15 months to 2 years he develops jargon of three word sentences including use of pronouns. Language and communication skill acquisition are complete by 5 years.

PERSONAL AND SOCIAL BEHAVIOR Right from the time the child is born he feels the impact of personal care. This he expresses by reduction of general activity by four weeks of age, at eight weeks he starts smiling, at twelve weeks he both vocalizes and smiles in social response. By 18 months a child generally develops joint attention. In the preschool years from age two to six years, children progress from being relatively unsocial to distinctly socialized individuals wherein the child learns to adapt himself to others and cooperate in play activities in which several children are involved.

EMOTIONAL AND AUTONOMIC REGULATIONS The pattern of the child’s emotional life changes with his perceptual insights and his personal social adjustment. At 28 weeks, an infant discriminates visiting strangers but then amiably adjusts to them. By 9 months he shows timidity in new situations and needs more time to adjust. Comparable changes in emotional maturity occur throughout the life cycle. Every stage of development follows a sequence, and the transition from one stage to another involves process of integration. Environmental factors may speed up or slow down development but the sequence of development remains the same. This factor exposes the fact that there are critical periods in a child’s life which, with clinician’s keen observation, can help to detect delays and understand the forces that work towards child development. These forces that are constantly at work are: a. Maturation of the central and autonomic nervous systems, b. Realization of competence which nourishes the child with a feedback mechanism, relying on the completion of a task that is done by the self alone, and Autism and Related Disorders: Regression of Milestones 117 c. Reinforcement from the environment which feeds the affective and cognitive needs.1,2 The child’s developmental level of functioning is determined by combining observations to standard developmental measures.

NEUROPLASTICITY Scientists once thought that the brain stopped developing after the first few years of life but recent research on animals and humans have shown that the brain continues to reorganize itself by forming new neural connections throughout life. This phenomenon, called neuroplasticity, allows the neurons in the brain to compensate for injury and recover brain functions by recruiting non-committed areas of the brain and remodeling of connections. The cortex is one of the last structures to mature extending several years. Therefore, the cortex lies substantially on environment factors to shape its organization. This is the basis for the use of development assessment and early detection of deficits for formulation of early interventional programs. Autism spectrum disorder is one of the most common causes of regression of milestones. Autism may sometimes present as regression of milestones and sometimes as a case of psychosocial and cognitive delay. 62 percent of children with ASD were reported by their mothers to have a period of normal development, followed by a major regression at age 12 to 30 months (18 months on an average standard deviation of 4 months). 38 percent of the children seemed to have had developmental delays from birth. Children with regressive ASD reached their developmental milestones in sitting, crawling, walking and talking at almost exactly the same age as the normal child. The interest in autism has grown recently because of increased number of children presenting with abnormal language and communication deficits with associated behavioral problems. Autism epidemics have recently been reported in the United States and in the United Kingdom. Recent studies have analyzed the prevalence of autism from the mid- 1980s through 2002 in the United States and the United Kingdom (Table 10.1). The prevalence of autism is estimated to have risen from one in 2500 children in the mid 1980s to as common as 1 in 150 by 2002. All these studies find the prevalence of autism in males to be four times that of females, the male prevalence of this disorder exceeding one in 100. These studies show that the rise in the prevalence in autism is genuine 118 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 10.1: Developmental disorder increase

Disorder 1987 1998 % change Autism 3,864 11,995 + 210 Cerebral palsy 19,972 28,529 + 43 Epilepsy 22,683 29,645 + 31 Mental retardation 72,987 108,563 + 49

(Source: State Department of Developmental Services, University of California, 2000). and not the result of population migrations, differences in criteria or other potential confounders.1-4 Autism, which is a complex disorder, is heterogenous both in its phenotypic expression and etiology. The search for neurobiological mechanisms that underlie behavioral problems have been hampered by their heterogeneity.

DEFINITION OF AUTISM Autism is a developmental neuropsychiatric disorder. According to fourth edition of Diagnostic Statistical Manual of Mental Disorder (DSM IV) defining features are impaired sociability, impaired language and communication and interest and activities. Mental deficiency is frequent but not universal. The cognitive profile is characteristic with a superior but narrow talent. Perseveration, concreteness, affective blunting and lack of insight into other person’s thinking may be conspicuous. In DSM-IV the pervasive developmental category is comprised of autistic disorder (the prototypical pervasive developmental disorder), Rett’s disorder, childhood disintegrative disorder, Asperger’s syndrome and pervasive developmental disorder not otherwise specified (PDD-NOS) which includes atypical autism.

BASIC PROBLEMS AND CONSEQUENCES OF AUTISM5 1. Limited capacity for emotional involvement with others 2. Altered capacity for sensory input modulation 3. Limited capacity for imitation and sharing attention 4. Limited capacity for understanding mental states 5. Limited capacity for abstraction and symbolism. Autism and Related Disorders: Regression of Milestones 119

Therefore, the problems these children face are: 1. Limited empathy for people 2. Altered perception of the world 3. Limited pragmatic communication skill 4. Limited theory of mind (for others and self) 5. Limited fantasy life and executive functions.

NEUROBIOLOGICAL INSIGHTS INTO AUTISM It was only in 1980 that autism received official recognition distinct from schizophrenia as a development problem.7 This has been on the basis of uncovering neurobiological defects. It is now considered to be a neurological disorder resulting from an error in brain development, and psychogenic theories have been completely invalidated. However, the precise location and nature of this deficit are still being actively debated and investigated. One theory emphasizes a dysfunction of the limbic system that result is impairment in acquisition of information. Second theory proposes a primary role for dysfunction of the cortical association networks responsible for the processing of information.5,6

Evidence for an Organic Cause Steffenburg6 found that almost 90 percent of a sample of autistic children had evidence of a brain abnormality of which substantial proportion was associated with megaencephaly.7,8 Five approaches have been used to study the neurobiology of autism. These methodologies include genetic, radiological, neurophysiological, neurochemical and neuropathological studies.

Genetic Factor Based on evidence from family studies, genetic factor plays a primary role. The American College of Medical Genetics has emphasized a high diagnostic utility of cytogenetic studies and of neuroimaging in clinical settings.9 The disorder is highly likely to be genetically heterogenous and X-linkage may be responsible for only a portion of autism cases, perhaps more commonly in high IQ males. In females it may be a result of an autosomal abnormality. Etiology in males may involve a mixture of both autosomal and X-linked defects.10 It is believed that chromosome 15 is implicated in this whole process. 120 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Recently, by identification of putative genes using genome-wide linkage analyses, and comorbidities with known genetic mutations, the RELN gene has been identified. This gene codes for an extracellular protein, guiding neuronal migration, and has been implicated in autism.11

Neuroimaging Studies Computerized tomography, magnetic resonance imaging (MRI) and positron emission tomography (PET) and functional MRI hold promise for elucidating the neurobiology of autistic disorders, but they present difficult practical and scientific challenges. However, few abnormalities have been found in common. Cortical abnormalities are frequently seen where whole brain is heavier than expected before the age of 12 years. But then it is lighter than expected in adults. Structural abnormalities: Mini Column abnormalities. These are basic functional units of the brain that organize neurons in cortical space and are involved in sensory information processing. Dr Manuel Casanova of Medical College of Georgia Studies on Mini Column cells has found that the brains of people with autism have more mini columns than the average brain. This variation in number, which is genetically determined, may affect signaling or how information is processed.

Subcortical abnormalities: Limbic system of the forebrain includes the limbic association cortex, hippocampal formation, and the amygdaloid complex. The limbic association cortex includes the cingulate gyrus, para- hippocampal gyrus. This area receives information from the higher order sensory areas and conveys this information to the hippocampal formation and amygdala. Patterns of abnormal behavior in autism have been compared with the severe disturbances observed in nonhuman primates that have undergone neonatal removal of the limbic system. These animals failed to develop normal relationship, displayed blank facial expression and poor body language, showed memory deficit and exhibited locomotor stereotypes such as twirling circles and doing somersaults. Neuroimaging studies in brains of autistic children have noted defects in these areas; smaller and immature neurons with poor dendrite Autism and Related Disorders: Regression of Milestones 121 arborization, voluminous amygdala suggesting incomplete neuronal pruning and sometimes smaller amygdala with advancing age suggesting cell death. Another study has shown that the neural architecture has greater number of cells per unit volume and a greater volume of neuropil in autism than control cases. Neuropil is the complex net of nerve fibres that forms the bulk of the CNS in which nerve cell bodies lie embedded. Elevated cell packing densities and neuropil volumes could indicate an abnormally high number of cortical connections in autism. Further studies have shown that a specific type of brain cells—glial cells—have shown signs of having suffered oxidative injury or stress. Significant reduction in number of Purkinje neurons has been noted in the cerebellum and brainstem. Cerebellum is the main area involved in visuospatial processing, emotional processing and motor processing. These abnormalities suggest that the onset of abnormal brain development may have started during the second trimester of gestation. Autistic children have defect in development of joint attention. It is hypothesized that the ventromedial prefrontal cortex plays a role in this development and possibly some other aspects of the autistic syndrome.10 Another major problem of autism is understanding the theory of mind of self and others. This has been studied by event related potential methodology to index activity of neural systems that are engaged during theory of mind and reasoning in adults. Left frontal areas showed enhanced positivity with diminution over left parietal areas. These findings provide an important perspective in both children’s theory of mind development and the neurobiology of disorder in which theory of mind is impaired.

Neurochemical PET and MRI studies have shown increased activity in normal subjects when viewing happy and fearful expressions in contrast to viewing neutral faces. But this change in activity is lacking in autistic patients. Neurotransmitter studies using PET suggest abnormalities of serotogenic and dopaminergic functions in the cortex and thalamus. There is increased of levels of beta-endorphins. This is an endogenous opiate-like substance in the body. 122 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Oxytocin and Vasopressin may prove to be important in the pathophysiology of clinical disorders, such as autism, characterized by an inability to form normal social attachment.

Magneto Encephalography This suggests a high incidence of epileptiform activity in children with autistic regression.

Magnetic Resonance Spectroscopy (MRS) MRS has begun to document metabolic deficits in the frontal cortex and cerebellum.

DIFFERENTIAL DIAGNOSIS Autism like symptoms are present in a lot of other medical conditions presenting as regression of milestones or developmental delay.

Rett’s Syndrome (RS) This is a neurological disorder seen almost exclusively in females. A child with RS usually shows an early period of apparently normal or near normal development until 6-18 months; then comes a period of stagnation during which child loses communication skills and purposeful use of hands. Then the child develops stereotyped hand movements, gait disturbances and deceleration of head growth. Febrile illness sometimes precedes the onset of the neurological picture. Hence this supports the hypothesis that environment factors may trigger onset of Rett’s Syndrome in genetically predisposed subjects. This Rett gene (or MeCP2), controls the cells which are involved in regulating the manufacture of a protein known as methyl-CpG-binding protein 2. This gene is located on the long arm of the chromosome in a region assigned with an identification of Xq 28.12,13 RS is most often misdiagnosed as autism, cerebral palsy or nonspecific developmental delay. Prevalence rate varies from 1:10,000 to 1:23,000 live female births. It is considered as a developmental disorder rather than a progressive disorder. All girls and women with RS do not display all of these symptoms, and individual symptoms may vary in severity. Autism and Related Disorders: Regression of Milestones 123

Infantile Neuronal Ceroid Lipofuscinosis (INCL) There is regression of milestones, after a period of normal development, at the age of 2 ¾ to 3 years, presenting with characteristic visual failure, rapidly decreasing motor ability, head control deterioration, hyperexcitability and trunk-limb extension tonus. However, before 2 years of age, it is difficult to differentiate from Rett’s Syndrome. Early clues for diagnosis are appearance of transient drop spells, loss of head control, irregular myoclonias, flattening of EEG and early cortical atrophy with abnormal ERG. For accurate diagnosis of INCL, a biopsy with characteristic EM findings of ‘snowball’ aggregates is a necessity (Hagberg, Witt-Engerstrom,1990).14

Fragile X Syndrome This is the most common form of inherited mental retardation and second most common single etiology of cognitive impairment. Prevalence is 1:1000 in males and 1:2000 in females. The syndrome is particularly interesting because of the substantial degree of genetic and phenotypic heterogeneity. The physical phenotype is subtle; it includes facial dysmorphism (long facial contour with midline hypoplasia and large anteverted ears); hyperextensible joints and macrorchidism in 80 percent of males. The signs and symptoms present as: Problems in intelligence and learning Problems in physical features Social and emotional problems Speech and language problems Sensory problems. The genetics of Fragile X Syndrome reflect major advances in human genetics. The fragile site is located at XQ 27.3. The most accurate test for detection is DNA molecular test—Southern Blot studies—that shows how quickly DNA passes through gel or liquid when electricity is passed.15,16 Other available tests are Chromosome test and Protein test. The former looks for broken areas of chromosome; the latter measures the amount of FMRC (Fragile X Mental Retardation Protein) and is a helpful test for mass screening. 124 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Childhood Disintegrative Disorder This is characterized by marked regression in various skills after two or three years of apparent normal development. It is a rare disorder with a prevalence rate of 0.11 in 10,000. Incidence is higher in boys. It is thought to be a neurological disease process, but no specific process has been demonstrated so far. A number of CNS disorders like neurolipidosis, Schilder’s disease (adrenoleucodystrophy) and metachromatic leukodystrophy have been associated. Onset of illness may be acute or gradual. The course of the disorder is not progressive. There is no specific treatment and management is similar to autism.

Asperger’s Syndrome There is characteristic impairment in social interactions but there is no delay in language and cognitive function or age appropriate self-help skills. Many children with Asperger’s syndrome show motor clumsiness in gross and fine motor movements.17

Tuberous Sclerosis (TSc) This is a disease that causes benign tumors to grow in the brain and other vital organs of the body. It commonly affects the central nervous system and results in combination of symptoms, like developmental delays, behavioral problems, autistic symptoms alongwith seizures, skin abnormalities and kidney disease. The disorder affects as may as 25,000-40,000 in the United States and 1 to 2 million worldwide. TSc may be present at birth but signs of the disorder can be subtle and full symptoms may take some time to develop. TSc caused by defects or mutation on two-genes TSc 1 and TSc 2. TSc 1 gene is on chromosome 9 and produces a protein called hamartin. TSc 2 gene is on chromosome 16 which produces a protein called tuberin and this is responsible for the tumor like growth. Prognosis is dependent on severity of symptoms.

Hypomelenosis of Ito In this condition small 0.5-1 cm hypopigmented, or white macules, coalesce to form reticulated patches along the lines of Blaschko. There may be Autism and Related Disorders: Regression of Milestones 125 associated dysmorphism, cleft palate, hemihypertrophy and psychomotor delay.

Neurocutaneous Syndromes Neurofibromatosis type 1 may be associated with developmental delay. Chedak Higashi syndrome; presents as recurrent infections, fingerprint like depigmentation, blood dyscrasias, lymphoreticular malignancy, developmental retardation, and these children may develop peripheral neuropathy by 2 years.

Metabolic Disorders Phenylketonuria (PKU): This condition arises due to phenylalanine hydroxylase deficiency and tetrahydrobiopterin cofactor deficiency. The child is initially normal except for musty odor in urine (phenylacetic acid); delay starts by 3 months and regression by 2 years if remains untreated.14 Aminoaciduria: In homocysteinuria there is cystathionine-synthase deficiency. These children are normal at birth, then delay or regression sets in if untreated and may manifest with ataxia, dystonia and aphasia and lens dislocation. Lactic acidosis: Lactic acid elevations in the central nervous system can be found in extramitochondrial neurologic disease and may prevent with symptoms of autism.

William Syndrome (WS) A rare genetic defect that results in severely impaired spatial cognition. Expressive language skills are less delayed in contrast to the important delay in language comprehension and fine motor skills. One commonly observed neuroanatomical abnormality in adults with WS is an enlarged cerebellum relative to small cerebrum.

Angelman’s Syndrome It is an association of severe mental retardation with absence of language, ataxia, convulsions, hyperactivity and joyful behavior with frequent bouts of laughing. Age of onset of symptoms is between 20 months to 8 years. It is confirmed by a cytogenetically detected deletion of chromosome bands 15q11-q13 (SNRPN). 126 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Infections Herpes encephalitis, rubella embryopathy, CMV infections may have associated regression of milestones with autistic features. HIV encephalopathy is also noted to cause regression of milestones. This supports the role of environment factors such as infections in the etiology of autism.18

Epileptic Encephalopathy West syndrome: These children present with infantile spasms, developmental plateau/regression and hypsarrhythmia. Landau-Kleffner (LKS) is a rare acquired epileptic aphasia, 85 percent occurring in otherwise healthy early school aged children. They have impaired verbal short term memory. This is related to a persistent decrease of activity in the posterior superior temporal gyri that were involved in the epileptic focus during the active phase. These children may have attention disorder and hyperkinesia. LKS is a rare disorder and is extremely important to detect it because early diagnosis and prompt medicinal treatment may improve the prognosis, whereas neglect and late diagnosis result in language disability.19

WORK UP 1. Detailed physical examination Focus on head circumference, dysmorphic features, organomegaly, skin examination, developmental and neurological examination. Auditory and ophthalmic examination 2. Neuroimaging: MRI, fMRI, MRS, PET 3. Electrophysiology: EEG and EMG when indicated 4. Metabolic Screening: Serum amino acids, lactate, pyruvate, carbon dioxide, ketones, glutamate and ammonia 5. Screening for toxic heavy metals like zinc, magnesium and lead. 6. Chromosomal analysis.

PSYCHOSOCIAL ASSESSMENT Assessment of Development Two stage screening procedure is advised. 1. The first stage utilizes questionnaire to be filled by parents, i.e. Denver Prescreening Developmental Questionnaire. Autism and Related Disorders: Regression of Milestones 127

2. The second stage screening is Denver Developmental Screening Test (DDST), which can detect developmental delay during infancy, and the preschool years. This test addresses gross motor, fine motor, adaptive and personal social skills. Other scales that are used are Bayley Scales of Infant Development (BSID). This test can be applied from birth to 30 months of age and consists of three major scales designed to evaluate cognitive skills, motor skills and social emotional maturity.

Ordinal Scale of Infant Psychosocial Development It is a reference test based on Piaget’s Theory of Cognitive Development. The scale is used for age up to 24 months. This test addresses issues of sensory (child becoming aware of its environment), motor (his subsequent actions) and object permanence scale (pursuing an object), which is not visible, and Cause Effect Relationship Scale (learning that activities produce specific results).20

Baroda Scale Development scale adapted to Indian children developed by the Department of Child Development, University of Baroda. The scale is applicable to babies up to age of 30 months.

Test of Assessment of Intelligence Stanford Binet Intelligence Scales. It can be used for individuals above 2 years of age. The test consists of naming pictures, giving meanings of vocabulary words, building tower, counting and calculating and identifying simila- rities. It is mainly a verbal scale and cannot be used for children with speech disorder and language deficits.

Weschler’s Primary and Preschool Scale of Intelligence (WPPSI) It is designed to assess intelligence after 4 years of age. It consists of eleven subsets grouped into verbal scales (Verbal Comprehension) and a performance scale (perceptual organization) from which the IQ can be generated from the test scores. 128 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Weschler’s Intelligence Scale for Children Revised (WISC-R) WISC-R—commonly used intelligence test between 6 years and 16 years 11 months. It consists of 5 sets of Verbal scale; information, vocabulary, arithmetic, comprehension, supplementary test (digit span). There is also a performance scale of 5 subsets.

Seguin Form Board It is designed for children from 3 years above. The child needs to put different shapes into a cut out board and the time of competition is noted and this assesses hand dexterity and intelligence.

Gassel’s Screening Test It is a type of intelligence test where the drawings are correlated with mental age. Different shapes like circles, cross, triangle, rectangle and diamond are presented to the children to copy and each shape determines age of child. It is given to children from 2 years to 6 years, and the IQ obtained thereof.

Adaptive Skills Measurement of adaptive skills aids the interpretation of intelligence tests. One such test is Vineland Maturity Scale, which addresses issues of communication, locomotion, dressing, self-help skills and socialization. It is applicable from infancy to 16 years. The other is the Developmental Screening Test.

FUTURE PROSPECT The unfurling of neurobiological basis of autism and related disorders has led to better understanding of the disease process. The neurochemical studies have influenced neuropharmacological observations. However, till date, the management of regression of milestones and autism in particular, needs one-to-one structured teaching, improvement of developmental skills, social skills, and behavioral management as early as possible. In those conditions where there is a definite known offending agent, removal of the agent helps in circumventing the delay. Autism and Related Disorders: Regression of Milestones 129

Social skill training is assuming a prominent role in behavior modification programs. The exact genetic mechanisms and genes involved in autism is under research. In the future genetic analysis and genetic linkage studies will need to consider using a broader definition of the autism phenotype including not just only autism but also individuals with severe cognitive and social disorders.

KEY LEARNING POINTS • Developmental milestones assessment covers areas of motor and adaptive behavior, verbal communication, personal and social behavior. • Any deviation in achievement of milestones signifies constitutional problem. • Child may lag behind for two reasons: developmental delay or due to regression of achieved milestones. • Two percent of cases of autism are due to regression. • Regression of milestones may be due to various causes, metabolic, genetic abnormality, neurocutaneous syndromes and infections.

REFERENCES 1. Ritvo ER, Freeman BJ, Pingree C, et al. A University of Utalt epidemiologic survey of autism: Prevalence. Am J Psychiatry 1989;146:194-199. 2. Allsopp MY, Rice C, Karapurkar T, et al. Prevalence of autism in a US metropolitan area. JAMA 2003;289:49-55. 3. Bertrand J, Mars A, Boyle C, et al. Prevalence of autism in a United States population: The Brick Township, New Jersey investigation. Pediatrics 2001;108:1155-1161. 4. Fiona JS, Baron-Cohen S, Bolton P, et al. Brief report: Prevalence of autism spectrum conditions in children aged 5-11 years in Cambridgeshire, UK. Autism 2002;6:231-237. 5. Rapin I, Katzman R. Neurology of autism. Ann Neurol 1998;43:7-14. 6. Minshew NJ, Paylon JB. New perspectives in autism, Part II: The differential diagnosis and neurobiology of autism. Curr Probl Pediatr 1988;18:613-694. 7. Bailey A, Luthert P, Bolton P, et al. Autism is associated with megaloencephaly (letter). Lancet 1993;341:1225-1226. 8. Bailey A, Couteur L, Gottesman O, et al. Autism as a strongly genetic disorder: evidence from a British twin study. Psychol Med 1995;25:63-77. 9. Battalgia A, Bianchini E, Carey JC. Diagnostic yield of the comprehensive assessment of developmental delay/mental retardation in an institute of child neuropsychiatry. Am J Med Genet 1999; 82:60-66. 10. Szatmari P, Jones MB. IQ and the genetics of autism. J Child Psychol Psychiatry 1991; 32:879-908. 130 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

11. Acosta MT, Pearl PL. The neurobiology of autism: new pieces of the puzzle. Curr Neurol Neurosci Rep 2003; 3:149-156. 12. Dunn HG. Importance of Rett’s syndrome in child neurology. Brain Developm 2001; 23:38-43. 13. von Tetzchner S, Jacobsen KH, Smith I, et al. Vision, cognition and developmental characteristics of girls and women with Rett’s syndrome. Developm Med Child Neurol 1996; 38:212-225. 14. Hagberg B, Witt-Engerstrom I. Early stages of the Rett’s syndrome and infantile neuronal ceroid lipofuscinosis: A differential diagnosis. Brain Developm 1990;12:20-22. 15. Mazzocco MM, Kates WR, Baumgardner TL, et al. Autistic behaviors among girls with fragile X syndrome. J Autism Dev Disord 1997;27:415-435. 16. Lotspeich IJ, Ciaranello RD. The neurobiology and genetics of infantile autism. Rev Neurobiol 1993;35:87-129. 17. Rinehart NJ, Bradshaw JI, Brereton AV, et al. A clinical and neurobehavioural review of high-functioning autism and Asperger’s disorder. Aust-NZ J Psychiatry 2002;36:762-770. 18. Ghaziuddin M, Al-Khouri I, Ghaziuddin N. Autistic symptoms following herpes encephalitis. Eur Child Adolesc Psychiatry 2002;11;112-116. 19. Raybarman C. Landau Kleffner syndrome: A case report. Neurol Ind 2002;50: 212-213. 20. Bavdekar SB, Kasla RR, Mehta CC, et al. Assessment of Psychosocial Development. In Gupte S (ed): Recent Advances in Pediatrics (Special Vol. 1 Nutrition, Growth and Development). Jaypee Brothers: New Delhi 1997;63-81. RAP Special Volume 19: Developmental and Behavioral Pediatrics 11 Obsessive-Compulsive Disorder

Helen D Pratt, Dilip R Patel, Donald E Greydanus

INTRODUCTION Obsessive-compulsive disorder (OCD), which is classified in DSM-IV as an anxiety disorder, is characterized by recurrent, time-consuming obsessive or compulsive behaviors that cause distress and/or impairment.1 Obsessions may include repetitive intrusive images, thoughts, or impulses. These are unwanted ideas or impulses that repeatedly emerge in the mind of the adolescent with OCD. Persistent fears that harm may come to self or a loved one, an unreasonable concern with becoming contaminated, or an excessive need to do things correctly or perfectly, are common. Again and again, the individual experiences a disturbing thought, e.g. “My hands may be contaminated—I must wash them”; “I may have left the gas on”; or “I am going to injure my little sister/brother.” These thoughts are intrusive, unpleasant, and produce a high degree of anxiety. Sometimes the obsessions are of a violent or a sexual nature, or concern illness.1 Compulsions are often (hand-washing or cleaning rituals) an attempt to displace the obsessive thoughts. In response to their obsessions, most people with OCD resort to repetitive behaviors called “compulsions”. The most common of these are washing and checking. Other compulsive behaviors include counting (often while performing another compulsive action such as hand washing), repeating, hoarding, and endlessly rearranging objects in an effort to keep them in precise alignment with each other. Mental problems, such as mentally repeating phrases, list making, or checking are also common. These behaviors generally are intended to ward off harm to the person with OCD or others. Some people with OCD have regimented rituals while others have rituals that are 132 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics complex and changing. Performing rituals may give the person with OCD some relief from anxiety, but it is only temporary. 1

PSYCHOLOGICAL IMPACT Many youth work hard to control their obsessive-compulsive symptoms when they are at school, work or with friends. However, the result is exhaustion and exaggerated symptoms once they reach the safety of their homes. These youth may clearly understand that their obsessions and compulsions are unrealistic; at other times they may feel conflicted about the reality or validity of their fears or compulsions. These youth may struggle to banish their unwanted, obsessive thoughts and to prevent themselves from engaging in compulsive behaviors. But over the months or years, their resistance weakens, and they their symptoms may become so severe and time-consuming that the rituals take over their lives, making it impossible for them to continue activities outside the home. Hiding their disorder can become a full-time activity for adolescents who are afraid to tell their parents, friends, or teachers or seek help. This secrecy can result in their obsessions or compulsion reaching a serious state of distress.

INCIDENCE AND PREVALENCE Approximately, 3.3 million (2.3%) American adults ages 18 to 54, or about 2.3 percent of people in this age group in a given year, have OCD. The first symptoms of OCD often begin during childhood or adolescence (one- third of cases). OCD is equally common in males and females. OCD cost the U.S. $8.4 billion in 1990 in social and economic losses, nearly 6% of the total mental health bill of $148 billion.1 Approximately, 21 percent of US youth, ages 9 to 17 years, had a diagnosable mental or addictive disorder that caused at least some impairment. When diagnostic criteria were limited to significant functional impairment, the estimated incidence was 11 percent, for a total of 4 million children who suffered from a psychiatric disorder with limitation of their ability to function. The 1-year-estimated prevalence of OCD in children ages 9 to 17 is 13 percent (0.2 to 0.8 % in children, and up to 2% of adolescents).1 Obsessive-Compulsive Disorder 133

COMORBIDITY OCD is sometimes accompanied by depression, eating disorders, substance abuse, attention deficit hyperactivity disorder, or other anxiety disorders. When a person also has other disorders, OCD is often more difficult to diagnose and treat. Symptoms of OCD can also coexist and may even be part of a spectrum of other brain disorders, such as Tourette’s syndrome. Investigators are currently studying the hypothesis that a genetic relationship exists between OCD and the tic disorders. Other illnesses that may be linked to OCD are trichotillomania (the repeated urge to pull out scalp hair, eyelashes, eyebrows or other body hair), body dysmorphic disorder (excessive preoccupation with imaginary or exaggerated defects in appearance), and hypochondriasis (the fear of having—despite medical evaluation and reassurance—a serious disease). Genetic studies of OCD and other related conditions may enable scientists to pinpoint the molecular basis of these disorders.1-3

BEHAVIOR THERAPY Treatments for OCD include medications and behavioral therapy, a specific type of psychotherapy. The combination of these treatments is often most effective.1-4 PET scans show that in individuals with OCD, both behavioral therapy and medication produce changes in the striatum. This is graphic evidence that both psychotherapy and medication affect the brain.2 The literature on the treatment of pediatric OCD is limited and lags behind studies of pharmacological treatment. However, current research supports that cognitive behavior therapy (CBT) carries considerable tentative promise.5 A review of the research on CBT showed that most studies are based on multiple baseline research designs or case reports. Several open trials are currently in progress at NIMH. The paucity of literature belies firm conclusions about efficacy of CBT in the treatment of OCD, and more is research needed.3 Less empirical literature is available for the treatment of OCD than for generalized anxiety disorders (GAD) and other anxiety disorders. However, the literature CBT suggests it is psychotherapeutic treatment of choice for this disorder. The use of CBT for treating OCD generally involves graded exposure and response prevention with some cognitive techniques and family involvement. Treatment modalities include individual group 134 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics and family sessions; booster or follow-up and family therapy increase treatment effectiveness. More study is needed to recreate a more extensive body of empirical literature for this for of treatment. Traditional psychotherapy, aimed at helping the patient develop insight into his or her problem, is generally not helpful for OCD. However, a specific behavioral therapy approach called “exposure and response prevention” is effective for many people with OCD. In this approach, the patient deliberately and voluntarily confronts the feared object or idea, either directly or by imagination. At the same time, the patient is strongly encouraged to refrain from ritualizing, with support and structure provided by the therapist, and possibly by others whom the patient recruits for assistance. For example, a compulsive hand-washer may be encouraged to touch an object believed to be contaminated, and then urged to avoid washing for several hours until the provoked anxiety has greatly decreased. Treatment then proceeds on a step-by-step basis, guided by the patient’s ability to tolerate the anxiety and control the rituals. As treatment progresses, most patients gradually experience less anxiety from the obsessive thoughts and are able to resist the compulsive urges. Studies of behavior therapy for OCD have found it to be a successful treatment for the majority of patients who complete it. For the treatment to be successful, it is important that the therapist be fully trained to provide this specific form of therapy. It is also helpful for the patient to be highly motivated and have a positive, determined attitude. Although not conclusive, new research has found that cognitive-behavioral therapy may also prove effective for OCD. This variant of behavior therapy emphasizes changing the OCD sufferer’s beliefs and thinking patterns. Additional studies are required before the promise of cognitive-behavioral therapy can be adequately evaluated. The positive effects of behavior therapy endure once treatment has ended.

Pharmacotherapy5-8 Behavior therapy remains the cornerstone of treatment for obsessive- compulsive disorder. In some instances addition of selective serotonin reuptake inhibitor (SSRI) agents (Table 11.1) has been shown to be effective in controlling symptoms.7 Only fluoxetine and fluvoxamine have been studied for their use in children and adolescents with obsessive- compulsive disorder. Typically, a singe SSRI agent is used for a period of Obsessive-Compulsive Disorder 135

Table 11.1: Serotonin re-uptake inhibitors Agent Typical daily dosage range Citalopram 20-40 mg Duloxetine 20-60 mg Escitalopram 10-20 mg Fluoxetine 5-60 mg Fluvoxamine 50-200 mg Sertraline 25-150 mg at lease 3 months in adequate dosage. If the response to the initial SSRI agent is not satisfactory, another agent can be tried. If there is no response to the second SSRI agent the patient may need augmentation of the pharmacologic treatment with other class of psychotropic agent, e.g. an antipsychotic or an antidepressant. A consultation with child psychiatrist should be obtained for further psychotropic management. Patients who respond to SSRI typically need ongoing use of the agent. Predictors of response are not known. The side effects of SSRIs are listed in Table 11.2.

Table 11.2: Side effects of SSRIs Activation of manic symptoms Akathisia Decreased appetite Decreased libido Diarrhea Dizziness Headaches Irritability Serotonin syndrome

CONCLUSION Appropriate diagnosis and treatment of other disorders are important to successful treatment of OCD. It is easy to overlook the seriousness of childhood mental disorders. Adolescents may present with symptoms that are different from or less clear-cut than the symptoms exhibited by adults with the same disorders. Younger children, especially, and sometimes older children as well, may not talk about what is bothering them or may have difficulty clearly describing their symptoms. Additionally, OCD affects not only the sufferer but the whole family. The family often has a difficult time accepting the fact that their adolescent cannot stop the 136 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics distressing behavior. Family members may show their anger and resentment, resulting in an increase in the OCD behavior. Or, to keep the peace, they may assist in the rituals or give constant reassurance. Education about OCD is important for the family. Families can learn specific ways to encourage their adolescents to adhere fully to behavior therapy and/or pharmacotherapy programs. Self-help books are often a good source of information. Left untreated, obsessions and the need to carry out rituals can take over an adolescent’s life. OCD is often a chronic, relapsing illness.

KEY LEARNING POINTS Obsessive-compulsive disorder is: • Not caused by rigid discipline and rules about cleanliness • Not caused by family problems • Has genetic and neurological basis • Can be effectively treated in most cases

REFERENCES 1. National Institute of Mental Health (NIMH). Facts about Obsessive-Compulsive Disorder Author. Bethesda, MD 1999 Publication OM-99 4154 (Revised) http:// www.nimh.nih.gov/publicat/ocdfacts.cfm Updated 2/17/06. 2. National Institute of Mental Health (NIMH): Step on a Crack... Obsessive Compulsive Disorder. Bethesda, MD, 2001. http://www.nimh.nih.gov/publicat/ ocdsoms.cfm Updated 2/17/06. 3. Wilson GT, Becker CB, Heffernan K. Eating Disorders In Mash EJ, Barkley RA (eds): Child Psychopathology, 2nd edn. New York: Guilford 2003. 4. Chorpita BF, Souatham-Gerow MA. Fears and Anxieties. In: Mash EJ, Barkley RA (eds): Treatment of Childhood Disorders, 3rd edn. New York: Gulford 2006:271- 335. 5. De Haan E. Effective treatment of OCD? J Am Acad Child Adolesc Psychiatry 2006; 45: 383. 6. Kendall PC, Hedtke KA, Aschenbrand SG. Anxiety Disorders. In Wolfe DA, Mash EJ. Behavioral and Emotional Disorders in Adolescents: Nature, Assessment, and Treatment. New York: Gulford 2006: 269-299. 7. Kamon J, Tolan PH, Gorman-Smith D. Interventions for adolescent psychopathology: Linking treatment and prevention. In: Wolfe, DA, Mash E J(eds): Behavioral and Emotional Disorders in Adolescents: Nature, Assessment, and Treatment. New York: Guilford 2006:56-88. 8. Varley CK, Varley JA, Smith CJ. Anxiety disorders in children and adolescents. In Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, Lincoln, NE: Universe, 2006: 619-648. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Lead Toxicity and 12 Neurobehavior

Tina Nichols, Arthur Feinberg

INTRODUCTION The goals of this article are to give a brief overview of the history of lead poisoning, discuss exposures, pathophysiology, clinical manifestations, toxicology with particular emphasis on neurobehavioral effects, testing and case management.

HISTORY OF CHILDHOOD LEAD POISONING Lead has a long history in the ancient world. It was favored in the Rome Empire due to its sweet taste and many uses. They used lead to flavor foods and wine, as well as to line piping. Hence, the derivation of the term plumbum. Many of the Roman elite demonstrated mental incompetence, infertility, and stillbirths due to chronic exposure to lead. Many modern scholars believe that it resulted in the fall of the Roman Empire.1 The first reports of lead toxicity in children occurred in Brisbane, Australia in 1892. Until 1904 many doubted that lead caused the epidemic, but subsequently Brisbane banned lead paint from households. Until 1943 lead toxicity was an acute event that resulted in either death or full recovery. At that time a follow up study showed 19 out of 20 children had significant behavior and learning disorders. It was not until 1970 when studies demonstrated that children without acute toxicity demonstrated deficits in attention, language, and IQ scores.2

EXPOSURES Environmental sources of lead are abundant in many societies. Lead is not biodegradable and, therefore, persists in an environment where 138 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics humans ingest or inhale it.3 Food, water, air, and soil are all potential hazards. Lead solder, used in the process of canning foods in many countries, is a contaminant. Banning the process in the US reduced intake in a 2-year-old from 30 μg/day to 2 μg/day. Water can be a source of lead exposure via lead pipes, lead solder, and brass pipes that surprisingly contain up to 8% lead.4 Water is more likely to become contaminated when it is stationary in leaded pipes, warm, or acidic (pH < 6.5).5 Leaded fuels are a major source of exposure. The combustion of leaded gasoline causes emission of lead into the atmosphere where it is absorbed via the respiratory tract. Atmospheric lead can also deposit in the soil adding another source of exposure. The introduction of the catalytic converter in the US has reduced lead emissions by approximately 98%. Unfortunately, many countries still use leaded gasoline placing their population at risk for lead toxicity. Lead paint is the major source in young children.4 As the paint begins to chip, or is disrupted in high contact areas such as window sills, young children easily find and ingest it. They are at highest risk due to their normal hand-to-mouth activity. By the mid-1970s lead was removed from paint in the US, but many homes built before this time, contain lead-based paint.6 Lead paint chips may be particularly attractive to young children due to its sweet taste.2 Lead crosses the placenta and is present in breast milk. Mothers with lead deposited in their bones will have higher serum lead levels during pregnancy and lactation due to increased bone turnover. In Mexico, maternal calcium supplementation was shown to decrease blood lead concentration, presumably due to decreased skeletal resorption.6 Another source of lead exposure can be herbal vitamins. There is no regulation for these types of substances and their content can be difficult to determine. Moore7 et al described a case of lead toxicity in a developmentally delayed child who took a “Tibetan herbal vitamin.” These tablets contained varying amounts of lead and the child’s blood level was 86 μg/dl necessitating chelation therapy. This paper emphasized the importance of interviewing families abut the use of any herbal or cultural medications when lead toxicity is in the differential diagnosis. Lead also appears in certain eye cosmetics known as kohl, tiro or surma. During production of these products lead substitutes for antimony to decrease the cost. Sindoor is a cosmetic in India and contains about 40% Lead Toxicity and Neurobehavior 139 lead.8 In a study conducted by Sprinkle9 et al eye cosmetics usage in Pakistan strongly correlated with increased blood lead levels.

PATHOPHYSIOLOGY Absorption Two types of lead exist, organic and inorganic. Leaded gasoline contains organic (tetraethyl) lead, which is primarily absorbed through the skin. Inorganic lead is absorbed via the respiratory and gastrointestinal tract. In children, 50% of the lead quantity ingested is absorbed versus 10-15% in adults. These absorption rates are increased in children who have dietary deficiencies of calcium, phosphorus, iron, or zinc.3

Distribution Once lead is absorbed, it enters the circulation. Erythrocytes contain 99% of lead in the bloodstream, and plasma contains the remaining 1%. It is this 1% that distributes to soft tissues (brain, renal cortex, lungs) and bone. This process takes approximately 4 to 6 weeks. In children; the skeleton contains 73% of total body burden of lead. The estimated half life of lead in bone is 20-30 years, and may be greater in children. Conditions that increase bone turnover can cause mobilization of lead into the bloodstream and lead to toxicity.3

Excretion The body slowly excretes lead over many years. The main route of lead excretion is via the urinary tract, and this is the mechanism of action for a number of chelating agents. Biliary excretion may also play a small role in lead excretion.3

CLINICAL MANIFESTATIONS There is a large range of signs and symptoms associated with lead toxicity, some of which may be nonspecific, or the child may be completely asymptomatic. This makes the diagnosis of lead toxicity very challenging. Entertain a diagnosis of lead toxicity in any child presenting with hyperactivity, developmental delays in learning and speech, hearing, and memory dysfunction. Physical symptoms with mild toxicity include mild fatigue, abdominal pain, myalgia and paresthesias. Moderate toxicity may 140 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics present as tremor, weight loss, headache, vomiting, inability to concentrate, and arthralgia. Severe toxicity may include paralysis, encephalopathy including seizures, coma, and death.

TOXICOLOGY Neurological Effects Lead has multisystem effects, those upon CNS the most significant. Lead is a divalent cation that binds strongly to proteins via the sulfhydryl group, leading to distortion of key proteins and enzymes.2 The earliest report of nervous system effects of lead exposure was in 1892 in Brisbane, Australia by J. Lockhart Gibson.2 Encephalopathy occurred at lead levels of 70 μg/dl or greater. Dr Herbert Needleman first noted the effects of lead on neurobehavioral development and cognition to be long lasting despite removal from exposure. Recently, researchers are focusing on the exact molecular targets of lead to attempt to discover ways to reverse its effects.

Learning and Memory In various animal models, lead affects learning and memory by altering the synaptic plasticity of the hippocampus. Synaptic plasticity is increased efficiency after stimulation over brief periods. This occurs via a glutamatergic receptor, N-methyl-D-aspartate receptor (NMDAR). Hippocampal NMDAR is essential to long-term potentiation (LTP), the means by which the brain is able to develop learning and memory at the cellular level. The NMDAR subunit expression is inhibited by lead exposure, resulting in its dysfunction, specifically spatial discrimination and learning.4 Children are particularly vulnerable to the effects of lead because their brains are still developing and their blood-brain barrier is incomplete.6 This is the “developmental window” of Kuhlmann et al, who found that rats who were exposed to lead during gestation and lactation, showed decreased performance in water maze testing as adult rats (100 days old) despite the fact that their blood lead levels were equal to that of older control animals. Individual children are vulnerable to lead at different times, thus having their own “windows of vulnerability.” This poses a great challenge to researchers who are striving to determine the specific targets and effects of lead toxicity.10 An encouraging study demonstrated that animals exposed to lead, showed improved ability to Lead Toxicity and Neurobehavior 141 learn when placed in “enrichment cages” versus animals that were lead exposed and placed in standard cages. The enrichment cages were a “combination of complex inanimate objects and social stimulation”. This suggests that environmental interventions may play a role in reducing the effects of lead exposure on children. 4

Intellectual Functioning In 1991, the Centers for Disease Control and Prevention lowered the acceptable blood lead level to <10 μg/dl. This was a vast improvement over the last half-century where the acceptable level was 60 μg/dl.11 Lead effects occur at blood lead levels < 10 μg/dl,3,5 especially diminishing intellectual functioning (IQ score), the measurement of the cognitive ability of an individual compared to the current population. Some researchers with data comparing IQ and blood lead levels have been able to predict a “slope” of IQ points lost per 1 μg/dl increase in blood lead levels. These data suggest that the slope is variable depending on blood lead level and the slop increases at lower blood levels. In one study the slope was 0.232 IQ points lost per 1 μg/dl increase in blood lead level. Another study revealed the slope was 0.232 IQ points lost for every 1 μg/dl increase in blood lead level between 15 and 10 μg/dl, but increased to 0.58 for levels below 10 μg/dl.12 In 1979, researchers showed deficits in psychometric intelligence scores, classroom performance, language and speech processing, and attention in children with elevated dentin lead levels. A follow up study was performed 11 years later and showed that the same children whose dentin lead levels were > 20 ppm had a higher rate of dropping out of high school and reading disability when compared to children from the same study with dentin lead levels < 10 ppm.13 Lanphear et al11 measured four different cognitive functions when determining the effects of blood lead levels. They reported a decrease in mean arithmetic scores by 0.7 for every 1 μg/dl increase in blood lead concentration. Nonverbal reasoning showed a 0.1 point decrease and short- term memory a 0.5 point decrease. They also noted these changes in children whose blood levels were < 5 μg/dl. 142 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Early Cognitive Development Bellinger et al14 demonstrated a decrease in infant developmental scores in those who had elevated lead levels in umbilical cord blood samples at birth. The infants fell into three groups, the low group with umbilical cord lead level < 3 μg/dl, medium group 6-7 μg/dl, and high group >10 μg/dl. The infants underwent developmental testing at 6 months, 12 months, 18 months, and 24 months. The children with the medium and high levels demonstrated decreased scores in developmental assessment. Interestingly, the postnatal blood lead levels of the infants did not correlate with the umbilical cord blood values.14

Behavior Adverse effects on behavior include inattention, hyperactivity, disorganization, aggression, and delinquency. These behaviors were documented 15 years after prenatal and postnatal exposure, showing that lead toxicity has lasting and possibly permanent effects.6 Nevin discovered the increasing and decreasing rates of behaviors such as violent crime and unwed pregnancy were remarkably consistent with long-term trends in lead exposure from gasoline and paint in the general population.12 One study using mice demonstrated that lead caused changes in the cholinergic system and resulted in hyperactivity in the mice. The prefrontal cortex may be one of the primary sites of damage induced by lead. This would help to explain certain behaviors that can be seen in children with lead toxicity such as a decreased ability to inhibit behavioral responses, and distractibility.15,16 As mentioned above, the hippocampus is also a main site of the brain affected during lead toxicity. There may be an imbalance that occurs in the hippocampus between acetylcholine and norepinephrine that may contribute to hyperactivity that is seen in children with elevated blood lead levels. The symptoms caused by lead toxicity in many children suggest attention deficit hyperactivity disorder.18

Neurodevelopment Unfortunately, lead also damages the blood-brain barrier. It accumulates in the endothelial cells of the vasculature of the brain, although when in isolated culture, endothelial cells were somewhat resistant to lead at low concentrations. Astrocytes, in isolated culture are very sensitive to lead,15,16 Lead Toxicity and Neurobehavior 143 and subject to injury. Astrocytes contain foot processes that encircle the microvessels of the brain forming a sealed barrier. As they are damaged, endothelial cells of the vessel walls become vulnerable to the effects of lead, and lead accumulates within the endothelial cells damaging the blood-brain barrier. Lead also disturbs cellular processes that utilize calcium including messenger systems. One of the key messengers is protein kinase C (PKC). Lead has been found to activate PKC to cause an increase in intracellular calcium. This seems to have some effects on voltage gated calcium channels, and may cause a disruption in the balance of neurotransmitters released, and the signaling that occurs along the individual nerves. 15 Neurosensory processing is affected by lead. Studies demonstrate changes in auditory processing and sensitivity in children with increased blood lead levels. A health survey showed increased thresholds for hearing in children with blood lead levels between 6-18 μg/dl. They have also discovered children with elevated lead levels perform more poorly in tests that require timed reactions.15 Lead interferes with myelin production.2 This may be the mechanism for development of the long-known lead neuropathy of lead toxicity. Studies demonstrate a decrease in nerve fibers including axons and myelin sheaths as well as segmental demyelination. Rats treated with lead demonstrated demyelination as well.17

Hematological Effects Lead negatively affects heme synthesis. The primary enzymes involved are delta aminolevulinic acid dehydratase (ALA) and ferrochelatase. Ferrochelatase incorporates iron into the porphyrin ring.2 When ferrochelatase is blocked it causes an increase in erythrocyte protoporphyrin (EP). Inhibition of this process eventually leads to decreased circulating hemoglobin.18 In the past, levels of EP screened for lead poisoning. This is no longer a useful tool because the EP level is not sensitive enough to identify a level of 10 g/dl, which is the current level necessitating intervention. The classic finding on peripheral smear is basophilic stippling of erythrocytes, hypochromic microcytic anemia, and rarely hemolysis. This can occur with blood lead levels of 20 μg/dl in adults and lower levels in children.2 144 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Renal Effects Blood lead levels affect renal function. Lead can cause interstitial fibrosis, decreased glomerular filtration rate, and renal insufficiency.2

Dental Effects Lead accumulates in developing teeth in both the preeruptive and posteruptive stages. Animal models and epidemiological studies demonstrate that the risk of dental caries increases as the developing tooth encounters lead. In animal studies, lead exposure leads to decreased salivary flow resulting in high rate of caries.19

PRIMARY PREVENTION Primary prevention is the most important step in protecting children from the effects of lead toxicity. Educating parents about the risks and long- term effects of lead exposure is every pediatrician’s responsibility. The major source of lead exposure in children is dust created by lead based paint. Thus the primary method of preventing lead exposure is to eliminate lead paint surfaces from the home. Unfortunately this is expensive and dangerous due to the production of additional dust during renovations. Remove children from these environments during abatement procedures including professional cleaning, replacing old windows with vinyl windows, and making floor coverings easily cleanable by replacing flooring with vinyl or linoleum.20 Encouraging governments and landlords to eliminate lead from housing is also an important step in primary prevention. The economic cost associated with decreased functioning and productivity of society’s children is far greater than the cost associated with making the environment safe from the hazards of lead. Some of the greatest health advancements include elimination of lead from the environment. The first step in 1971 was the banning of lead in household paint, but, unfortunately many homes built before this time contain lead- base paint. The second step was in 1977 when the Environmental Protection Agency began removing lead from gasoline.2

Screening The most effective means of testing blood lead levels is by venous sampling. Some communities use a finger stick, but if there is skin Lead Toxicity and Neurobehavior 145 contamination by lead, this may falsely elevate the lead level, necessitating a venous sample for confirmation.6 However, finger-stick remains the primary screening procedure because of its simplicity and convenience. In 1991, CDC recommendations included a questionnaire and screening of children aged, 9 to 72 months who failed it. They made an exception for children in communities that could prove that their children were not at high risk. Very few communities had this data, thus resulting in de facto universal screening. These recommendations changed in 1997 to a more targeted screening. This included screening of children 1 and 2 years of age, or 36-72 months if not previously tested, if they could be included in any of the following three categories: 1) child resides in a zip code that has 27% or more of houses built before 1950; 2) child receives benefits from public assistance programs such as Medicaid and Women, Infant, and Children; and 3) child’s parent/guardian answers “yes” or “don’t know” to any of these three risk assessment screening questions: 1. Does your child live in or regularly visit a house or child care facility built before 1950? 2. Does your child live in or regularly visit a house or child care facility built before 1978 that is being or has recently been renovated or remodeled (within the last 6 months) 3. Does your child have a sibling or playmate that has or did have lead poisoning?” Studies have evaluated these questionnaires to determine sensitivity, specificity, positive and negative predictive values with varying results. At best, questionnaires similar to the abovesaid had a 98% negative predictive value for detecting children with blood lead levels > 10 μg/dl when living in zip codes considered lower risk. Current studies are being conducted to determine additional questions to include in such questionnaires in order to enhance them, hoping to develop means to identify patients with lead levels below 10 μg/dl.18 The recommendations were again modified in 2005 to include the mandatory screening of all Medicaid- eligible children at 1 and 2 years of age. This recommendation arose due to the high prevalence of elevated lead levels in this patient population as well as to the low number of Medicaid-eligible children being screened.6 The age at which screening occurs is important. Early childhood is a time of increased hand-to-mouth behavior that places children between 6 months and 2 years of age at increased risk. If an elevated lead level occurs 146 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics at the 1 year screen, this provides an opportunity to correct the hazardous exposures, and decrease the amount of lead exposure. The lead level at 2 years of age is the most predictive of future effects of lead toxicity. Lead levels will usually decrease after the age of 2, regardless of intervention.6

MANAGEMENT According to current guidelines, children do not have elevated blood lead levels under 10 μg/dl. Recent evidence indicates that blood lead levels below 10 μg/dl are associated with decreased IQ points, which underscores the importance of protecting children from lead exposure regardless of the amount of exposure.6 Treatment for lead toxicity is based upon the actual blood lead level. The first step when a child has a blood lead level of 10-14 μg/dl is to repeat the level in 1-3 months with a venous sample, and to provide education re-environmental risk, diet, cleaning and hand-mouth contact to the family. Levels between 15-19 μg/dl need the above education plus a health department referral, and if persistently high (at least 3 months) manage them as if in the range of 20-44 μg/dl. This range necessitates laboratory studies including hemoglobin and iron levels as well as neurodevelopmental monitoring, and abdominal radiography to evaluate for lead ingested. Initiate chelation therapy when levels become > 45 μg/dl. Oral chelation with Dimercaptosuccinic acid (DMSA, Succimer, and Chemet) is the preferred treatment at levels between 45 and 70 μg/dl, 350 mg/m 2 /dose q8h for 5 days, then q12h for 14 days. Hospitalization typically occurs for lead levels > 70 μg/dl.6 with parenteral chelation therapy using two drugs,

Ca Na2 EDTA (versentate) plus either BAL (Dimeraprol, British Anti- Lewisite) or Succimer. Administer EDTA as 1000-1500 mg/m2/day either IV or IM for 5 days, every 6 or every 12 hours. In the event of encephalopathy, EDTA and BAL, 300-500 mg/m2 /day IM only for 3-5 days is the preferred combination. Note that chelation therapy will diminish blood lead levels, but they often rebound due to storage in bone. Repeated chelation treatments may be necessary and children with markedly elevated blood lead levels may take months to years to normalize. Benefits of chelating children at lead levels < 45 μg/dl are unclear. A study in 1994 administered cognitive test scores and gave EDTA (ethylenediaminetetraacetic acid) and iron to children between the ages of 13-87 months with blood lead levels between 25-55 μg/dl. They followed Lead Toxicity and Neurobehavior 147 up in 6 months with cognitive test scores which improved in the children with the highest lead levels regardless of whether they received iron or chelation therapy with EDTA. The Treatment of Lead-Exposed Children (TLC) trial, a large randomized study, showed no improvement in cognitive or neuropsychologic testing in children who received Succimer, despite showing a decrease in lead concentration.6

FUTURE ADVANCEMENTS There are numerous avenues regarding lead and its toxic effects on the human body that still require research. Since our ability to reverse the effects of lead toxicity is dismal, prevention is where we have the greatest impact. As we continue to eliminate lead exposure in our environment, we will be salvaging the IQ of future generations. As we learn more about the mechanism of action of lead on various body systems, primarily the CNS, we can attempt to develop ways to reduce the negative effects of lead. Providing an “enriched environment“, with appropriate educational stimulation may have a role in improving neurological function after exposure to lead, another exciting area for further research.

KEY LEARNING POINTS • Lead poisoning has occurred through history, but not deemed a cause until the turn of the 20th century. • Investigators did not acknowledge chronic lead toxicity a cause of symptoms until 1970. • Lead is omnipresent in the environment and children are most vulnerable to exposure. • Acute lead poisoning has significant toxic encephalopathic effects especially on the CNS causing severe encephalopathy at very high blood levels (> 70 μg/dl). • Chronic lead poisoning has profound cognitive, behavioral and neurodevelopmental effects in children. Even levels < 10 μg/dl can cause significant decreases in IQ. • Studies demonstrate pharmacologic treatment for lead toxicity is of variable benefit. • Removal of environmental lead is still the most effective avenue to approach the problem of lead toxicity. 148 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Future research should address better understanding of the pathophysiology of lead poisoning and development of more effective modes of treatment. Enrichment of the learning environments of children with lead toxicity is worth exploring.

REFERENCES 1. Lewis J. Lead poisoning: A historical perspective. EPA J 1985;10:100-106. 2. Needleman H. Lead poisoning. Ann Rev Med 2004; 55:209-222. 3. Papanikolaou NC, Hatzidaki EG, Belivanis S, Tzanakakis GN, Tsatsakis AM Lead toxicity update. A brief review. Med Sci Monitor 2005;11: RA329-336. 4. Toscano CD., Guilarte TR. Lead neurotoxicity, from exposure to molecular effects. Brain Res Rev 2005; 49:529-554. 5. CDCP. Managing Elevated Blood Lead Levels Among Young Children. Atlanta: Centers for Disease Control and Prevention 2002. 6. AAP Policy Statement. Lead Exposure in Children: Prevention, Detection, and Management. Pediatrics 2005; 116: 1036-1046. 7. Moore C, Adler R. Herbal Vitamins: Lead Toxicity and Developmental Delay Pediatrics 2000; 106: 600-602. 8. Leaded Gasoline in Context Global Opportunities for Reducing the Use of Leaded Gasoline. US Department of Health and Human Services. Case Studies in Environmental Medicine. Lead Toxicity, course SS3059: 1-55. 9. Sprinkle RV. Leaded eye cosmetics: A cultural cause of elevated lead levels in children. J Fam Pract 1995 ; 40: 358-362. 10. Bellinger DC. Lead. Pediatrics 2004; 113: 1016-1022. 11. Lanphear, BP, Dietrich K, Auinger P, Cox C. Cognitive deficits associated with blood lead concentrations <10μg/dL in US children and adolescents. Public Health Reports 2000;115:100-110. 12. Nevin R. How lead lxposure relates to temporal changes in IQ, violent crime and unwed pregnancy. Environmen Res 1998:83:1-22 13. Needleman HL, Schell A., Bellinger D., Leviton A, Allred, EN. The long-term effects of exposure to low doses of lead in childhood. An 11-year follow-up report. NEJM 2000; 322: 83-88. 14. Bellinger D, Leviton A, Waternaux C, Needleman, H Rabinowitz M. Longitudinal analyses of prenatal and postnatal lead exposure and early cognitive development. NEJM 1987: 316: 1037-1043. 15. Finkelstein Y, Markowitz ME Rosen JF. Low level lead-induced neurotoxicity in children: An update on central nervous system effects. Brain Res Rev 1998; 27:168- 176. 16. Sanborn MD, Abelsohn A, Campbell M, Weir E. Identifying and managing adverse environmental health effects: 3. Lead exposure. CMAJ 2002; 166 1287- 1292. 17. Krigman MR. Neuropathy of heavy metal intoxication. Env Health Persp 1978;26:117-120. 18. Laraque D, Trasande L. Lead poisoning: Successes and 21st century challenges. Pediatr in Rev 2005; 26: 429-436. 19. Billings RJ, Berkowitz RJ, and Watson G. “Teeth”. Pediatrics 2004; 113: 1120- 1127. 20. Commentary. The Road to primary prevention of lead toxicity in children, Pediatrics 2001: 107: 581-583. RAP Special Volume 19: Developmental and Behavioral Pediatrics 13 Epilepsy in the Adolescent

Donald E Greydanus, David H Van Dyke

INTRODUCTION The annual incidence of epilepsy in the United States is nearly 25 per 100,000 population for 10-14 years old and over 18 for 15-19 years old. 1,2 Prevalence for India is noted at 2.5 to 5.3/1000 population.3 Approximately 1% of the general population has epilepsy or a seizure disorder and about 25% of those with epilepsy started with seizures under age 18 years of age. Most cases are idiopathic though a wide variety of causes have been identified (Table 13.1).1-9 If an adolescent presents with new-onset seizures, such causes should be considered as drug abuse, head trauma, central nervous system infection, pseudoseizures, cerebrovascular, cancer or cancer treatment effects, and idiopathic epilepsy.1, 2 Certain seizure types typically begin in adolescence; these include juvenile absence epilepsy and juvenile myoclonic epilepsy. Table 13.1: Causes of seizures or seizure-like activity1-3,10 • Idiopathic epilepsy • Intracranial granuloma • Drug abuse • Syncope • Systemic and central nervous system (CNS) infections • CNS congenital defects and neoplasms • Neurocutaneous syndromes • Metabolic disorders (hypoglycemia, hypocalcemia, others) • Cerebrovascular accident • Hypertensive encephalopathy • Collagen vascular disorders • Renal failure • Liver disease • Pseudoseizures • Many others 150 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

A careful evaluation is necessary for adolescents presenting with seizure or seizure-like activity. The history should look at what type of seizure took place, what precipitating factors are present, and what family history is present. Potential underlying factors include puberty, menstruation, fever, drug abuse, trauma, stress, sleep deprivation, sleep itself, hyperventilation, photic stimulation (such as flickering lights), and others. The physical examination should focus on the blood pressure (standing and lying), auscultation for bruits, skin lesions, body/extremity asymmetry, and a very thorough neurological evaluation. The laboratory evaluation is determined by the history and physical examination findings. Potential laboratory tests include a complete blood count (hemoglobin, hematocrit, white blood count, various cell indices), urinalysis, electrolytes, glucose, kidney function tests, drug screens, lumbar puncture in selected cases, MRI (or CT), and others. A 16-channel electroencephalogram (EEG) is important while the adolescent is awake and, if possible, asleep or sleep deprived; photic and hyperventilatory stimuli are important parts of the EEG as well.8 Some patients with epilepsy have normal EEGs, while characteristic EEGs are noted in those with absence, Lennox-Gastaut, and infantile spasms.

SEIZURES: GENERAL ISSUES The World Health Organization and International League Against Epilepsy uses four basic categories of seizures: Primary generalized epilepsies, partial epilepsies, secondary generalized epilepsies, and unclassified epilepsies.11,12 The most common types of seizures seen in adolescents include rolandic seizures, absence, pure tonic-clonic, absence with tonic-clonic, clonic-tonic, juvenile myoclonic, and complex-partial (temporal-lobe epilepsy). Most have one type, though mixed seizures may be found as well. The reference section provides articles and texts that provide detailed information on the specifics of these seizure types.1-10 Most youth with epilepsy are well-controlled with anticonvulsant medications. Consultation with neurology consultants is necessary for the best seizure control for the individual patient.1,2,13 The first line of medication for partial seizures are carbamazepine, oxcarbazepine and phenytoin. The second set of medications includes phenobarbital, valproate, gabapentin, levetiracetam, topiramate and lamotrigine. The third line of medications includes felbamate, primidone, and Epilepsy in the Adolescent 151 benzodiazepines. The first line of medications for generalized seizures include valproate and phenytoin, while the second line-includes the benzodiazepines and levetiracetam. The third line of drugs for generalized seizures include phenobarbital, lamotrigine, and acetozolamide. These anticonvulsant medications have numerous side effects and careful attention to these adverse effects are necessary to maximize medication compliance by the adolescent.

Rolandic Epilepsy This is a form of simple partial epilepsy that is noted in children and adolescents; it usually starts between ages 5 and 9, typically resolving during the adolescent years. It is also called Benign Epilepsy with Rolandic Spikes (BERS) or Benign Childhood Epilepsy with Centrotemporal Spikes. Rolandic epilepsy is an autosomal dominant disorder that starts with a tonic-clinic seizure often at night; there can be speech dysfunction with involuntary muscle contraction of one side of the body—face, arm, leg, or pharynx. The EEG is characteristic with rolandic (central) spikes. Medications are often not necessary for this self-limiting disorder; if anticonvulsants are used, seizure control is usually excellent.

Juvenile Absence Epilepsy This is a form of absence seizure disorder that may be first noted around the time of puberty. A generalized tonic-clonic form of epilepsy can occur before or after the onset of the juvenile absence seizure disorder. The EEG shows a greater than 3 per-second pattern. Treatment is with valproic acid or ethosuximide with clonazepam as an alternative.

Juvenile Myoclonic Seizure (Juvenile Myoclonic Seizure of Janz) This is classified as a primary generalized seizure that accounts for 12% of pediatric epilepsy and is localized to the short arm of chromosome 6. This seizure pattern starts with myoclonic jerks of neck/shoulder flexor muscles in association with clonic-tonic seizures typically shortly after awakening in the morning. Generalized tonic-clonic seizures may develop within 2 years and the entire seizure pattern becomes more problematic with stress, substance abuse, and lack of sleep. The EEG patterns are well- known (4-6 Hz or multispike and wave pattern). Medications often useful for seizure control include valproate with clonazepam, levetiracetam, and lamotrigine as alternatives. 152 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

SEIZURES: SPECIAL ISSUES IN ADOLESCENTS WITH EPILEPSY Adverse Effects of Antiseizure Drugs Side effects of these medications can lead to limited compliance by the adolescent, resulting in poor seizure control. Thus, it is imperative that clinicians select the medication (s) that provide the least amount of side effects possible. For example, phenytoin may lead to gingival hyperplasia, coarse facies, behavioral problems, and cognitive dysfunction. Valproate may lead to weight gain, impotence, menstrual dysfunction, polycystic ovary syndrome, hyperandrogenism, hair loss, gastrointestinal problems, and rash. Phenobarbital may lead to behavioral and cognitive dysfunction; there may be some association with depression and suicide behavior as well. Topiramate may produce cognitive change and word finding difficulty. Lamotrigine may give a serious rash if introduced rapidly.

Psychological Aspects Youth with chronic illness, including epilepsy, may be at increased risk for low self-esteem, parent-child conflicts, and family dysfunction.14-17 These youth should be monitored carefully for their progression through adolescence. These adolescents often have limited knowledge of their illness, and, thus, thorough education about their condition needs to be provided on a routine basis. They may not know that exposure to flickering lights, as in video games, may worsen their seizures; special filtered glasses may help if photic stimulation is a problems. Many adolescents are behind in their sleep needs, and this sleep dept can lead to worsening seizure patterns. Drug and alcohol abuse, a common problem in some youth, will also complicate the epilepsy management.18,19 Some medications lead to reduced unbound testosterone with reduced sex drive. The presence of psychiatric disorders in youth with a seizure disorder may be noted. However, the exact cause(s) is not clear: whether due to medication effects (as ethosuximide), chronic illness issues in general, status epilepticus effects, rapid medication withdrawal, or other factors. The process may also be complicated by the development of pseudoseizures, in which there is seizure-like activity that does not represent overt epilepsy; in these situations, the EEGs are normal.

Contraception

Pregnancy in the adolescent female with epilepsy can provide additional complications, such as worsening the seizure pattern, increased dose of Epilepsy in the Adolescent 153 antiepileptic drugs, and reduced compliance with the required medications. Effective contraception is important for these youth who wish to avoid unwanted pregnancy. Some epileptic medications can induce the liver microsomal enzyme system, leading to increased oral contraceptive metabolism and less efficacy; these medications are listed in Table 13.2.20-22 Some drugs do not interfere with oral contraceptives, including valproic acid, gabapentin, felbamate, and lamotrignine.

Table 13.2: Antiseizure medications interfering with oral contraceptive efficacy • Phenobarbital • Phenytoin • Carbamazepine • Primidone • Topiramate • Ethosuximide • Tiagabine

Pregnancy Pregnant adolescents who also have epilepsy should be on appropriate amounts of folate to lower their risk for neural tube defects; some experts suggest a dose of 1 mg per day, up to 4 mg per day if there is a family history for tube defects or sickle cell disease. The potential teratogenic potential of antiseizure medications can also be of concern to the patient and her obstetrician. The fetus is actually at more risk from seizure activity of the mother during pregnancy than from the antiseizure drugs themselves. Serum levels of these drugs may drop during pregnancy and thus, increased drug dosages are often necessary until after the pregnancy. Genetic counseling is also necessary for the pregnant youth.

Teratogenicity It is during the first 2 months of pregnancy that teratogenicity may develop. There is a 2 to 3-fold higher rate of birth defects in the offspring in which the father or mother is on antiseizure medications. Tridione should not be prescribed to pregnant females because it induces a very high rate of stillbirths and birth defects. Also associated with birth defects are such medications as valproate (spina bifida) and phenytoin. If valproate is prescribed during pregnancy, monitoring of alpha-fetoprotein levels is mandatory. Also associated with neural tube defects is carbamazepine in 154 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics pregnancy. The safety of lamotrigine and gabapentin in pregnancy is unclear at this time.

Vocational Guidance Adolescents with chronic illness, such as epilepsy, need competent vocational guidance to offset the potential for limited academic success that may result. Some youth have problems adjusting to a chronic illness, such as dealing with potentially negative family reactions to a sick child or teen, poor seizure control, adverse effects of antiseizure medications (such as attention span reduction), and concomitant learning problems.14,15 Some professions are limited to or closed to those with epilepsy, and these youth should understand this concept. For example, even those with full control may experience difficulty entering the military, police academy, or fire departments. Sports: Sports participation can be a very healthy and positive aspect of the adolescent’s life, and those with good seizure control can be active in sports, including contact sports.23,24 Research suggests that physical activity itself does not cause or worsen epileptic episodes.14 Cooperation between the adolescent, parents, and clinician is important to determine the extent and nature of the sports play in each situation. Potential seizure activity can be hazardous in some situations, however, and thus restrictions may be imposed; for example, rope climbing, gymnastics with parallel bars, high diving, competitive underwater swimming, and other high-risk activities will lead to such restrictions. Contact sports, such as hockey, have increased risks for head trauma and some clinicians will impose limitations on the play of those with epilepsy.

Driving As the adolescents seeks to leave the dependency of childhood and enter the privileges of adulthood, certain activities become important to the teen to convince him or her that they are becoming autonomous adults. One of these very important activities for many youth is driving a car, motorcycle, or other motorized vehicle. Each country and even regions within a country have imposed laws on how long a youth with epilepsy must have seizure-free periods before driving is allowed; these periods may range from 3 months to 2 or more years in the United States.14 If the Epilepsy in the Adolescent 155 clinician is considering the withdrawal of antiseizure medications in a youth with good control, it should not be around the time she/he is applying for a drivers’ license. The restriction of a license may lead to negative behavior on the part of the youth—such as lowered self-esteem, reduced socialization, job employment restrictions, reduced compliance with driving rules, and others. Youth with epilepsy and any form of chronic illness, should be allowed access to as normal a life as an adolescent as possible. Clinicians privileged to care for youth should help in this important goal as much as possible.

KEY LEARNING POINTS • The most common types of seizures seen in adolescents include rolandic seizures, absence, pure tonic-clinic, absence with tonic- clonic, clonic-tonic, juvenile myoclonic, and complex-partial (temporal-lobe epilepsy). • Most have one type, though mixed seizures may be found as well. • The first line of medication for partial seizures are carbamazepine, oxycarbamazepine and phenytoin; the second set of medications includes phenobarbital, valproate, gabapentin, levetiracetam, topiramate and lamotrigine. The third line of medications includes felbamate, primidone, and benzodiazepines. • The first line of medications for generalized seizures include valproate and phenytoin, while the second group includes the benzodiazepines and levetiracetam. The third line of drugs for generalized seizures include phenobarbital, lamotrigine, and acetozolamide. • Clinicians must select the medication(s) that provide the least amount of side effects. • Drug and alcohol abuse, a common problem in some youth, also complicates the epilepsy management. • Pregnant adolescents who also have epilepsy should be on appropriate amounts of folate to lower their risk for neural tube defects. • If valproate is prescribed during pregnancy, monitoring of alpha- fetoprotein levels is mandatory. • Competent vocational guidance is important to offset the potential for limited academic success that may result. • Physical activity itself does not cause or worsen epileptic episodes. • Youth with epilepsy and any form of chronic illness, should be allowed access to as normal a life as possible. 156 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

REFERENCES 1. Greydanus DE, Pratt HD, Van Dyke HD. Neurologic and neurodevelopmental dilemmas in the adolescent. Adolesc Med 2002;13:413-686. 2. Greydanus DE. Neurologic disorders. In: Hofmann AD, Greydanus DE (eds): Adolescent Medicine. Stamford Appleton and Lange 1997: 242-288. 3. Kanhere S, Bhave SY. Epilepsy in adolescence. In: Bhave SY, Nair MKC, Parthasarathy A, Menon PSN, Greydanus DE (eds): Bhave’s Textbook of Adolescent Medicine. New Delhi: Jaypee Brothers 2006: 402-415. 4. Johnston MV. Seizures in Childhood. The nervous system. In: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics,17th edn. Philadelphia: Saunders 2004:1993-2009. 5. Bradley WG, Daroff RB, Fenichel GM, Marsden CD. Neurology in Clinical Practice: Principles of Diagnosis and Management, 3rd edn. Available at: www.nicp.com 6. Chang BS, Lowenstein DH. Epilepsy. N Engl J Med 2003;349:1257-1266. 7. Liebenson MH,Rosman NP. Seizures in adolescents. Adolesc Med 1991; 2: 629- 648. 8. Paolicchi JM. Epilepsy in adolescents: Diagnosis and treatment. Adolesc Med 2002; 13:443-459. 9. Swaiman KF Ashwal S. Pediatric neurology: Principles and practice. In: The Epilepsy Foundation, St Louis: Mosby 1999. Available at: www.epilepsyfoundation.org 10. Greydanus DE, Van Dyke D. Neurologic disorders. In: Greydanus DE, Patel DR, Pratt HD (eds): Essential Adolescent Medicine. New York: McGraw-Hill 2005: 231-275. 11. Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30:389-399. 12. Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and epilepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001; 42:796-803. 13. LaRoche SM, Helmers SL. The new antiepileptic drugs. JAMA 2004; 291:605- 620. 14. Greydanus DE, Van Dyke D. Epilepsy in the adolescent: The Sacred Disease and the Clinician’s Sacred Duty. Int Pediatr 2005; 20:6-8. 15. Loring DW, Meador KJ. Cognitive and behavioral effects of epilepsy treatment. Epilepsia 2001;42(Suppl 8):24–32. 16. Nordli DR Jr. Special needs of the adolescent with epilepsy. Epilepsia 2001; 42(Suppl 8):10–17. 17. Goldson E. Behavioral issues in the care of children with special health care needs. In: Greydanus DE, Patel DR, Pratt HD(eds): Behavioral Pediatrics, 2nd edn. NewYork: Universe 2006: 82-205. 18. Greydanus DE, Patel DR. The adolescent and substance abuse: Current concepts. Disease a Month 2005;51: 392-431. 19. Greydanus DE, Reed WJ. Substance use and abuse in adolescents. In: Greydanus DE, Patel DR, Pratt HD (eds): Behavioral Pediatrics, 2nd edn. New York:Universe 2006:812-855. 20. Rimsza ME. Contraception. In: Greydanus DE, Patel DR, Pratt HD (eds): Essential Adolescent Medicine. New York: McGraw-Hill 2006:543-558. 21. Stewart DC. Contraception. In: Hofmann AD, Greydanus DE (eds): Adolescent Medicine, 3rd edn. Stamford: Connecticut 1997:566-588. Epilepsy in the Adolescent 157

22. Greydanus DE, Rimza ME, Matytsina L. Contraception for college students. Pediatric Clin North Am 2005;52:135-161. 23. Patel DR, Greydanus DE, Luckstead EF. Sports preparticipation evaluation. In: Greydanus DE, Patel DR, Pratt HD (eds) : Essential Adolescent Medicine. NewYork: McGraw-Hill 2006:669-675. 24. Patel DR, Pratt HD, Greydanus DE. Psychological aspects of sports participation. In: Greydanus DE, Patel DR, Pratt HD(eds): Behavioral Pediatrics, 2nd edn. NewYork: Universe 2006:265-292. RAP Special Volume 19: Developmental and Behavioral Pediatrics

14 Headaches in the Adolescent

Donald E Greydanus, Artemis K Tsitsika David H Van Dyke, Daniel Olson

INTRODUCTION Headache is a very common condition described for over 5000 years.1 According to Greek mythology, Zeus (the king of Olympian gods) suffered from severe headache attacks, until Athena, his daughter (goddess of wisdom), finally popped out of his head and permanently cured her father! Hippocrates of Cos (460-377 BC), the Father of Modern Western Medicine, described what were later called migraine headaches.1 Headache is the most common pain manifestation in adolescence, and it is noted in three-quarters of females and half of males who are given a 30 days survey.1-4 Approximately 10% of females and 5% of males report more than one headache per month.4

ETIOLOGY Headaches may be classified according to their underlying causes (Table 14.1). The two main types of headaches are due to intracranial vasculature dysfunction (migraine headaches) and spasms of the external muscles of the head, face and neck (tension headaches). The prevalence for migraine is 15%, and for non-migraine headaches 17%.5 According to the International Headache Society (IHS) classification system, headaches are mainly divided into primary types (migraine, tension and cluster headaches) and secondary types(due to an underlying organic cause, such as trauma, infection, or neoplasm).4,6 Persistence of adolescent headaches into adulthood may occur in 60% of cases.7 Intracranial pathology must be considered and excluded in adolescents with chronic headaches (> 15 days/month); fortunately, investigation is rarely positive for a neoplasm. Headaches in the Adolescent 159

Table 14.1: Differential diagnosis of headaches* • CNS disorders Infections: Meningitis, encephalitis, brain abscess Subarachnoid or subdural hemorrhage: Trauma, spontaneous rupture of aneurysm or arteriovenous fistula, thromboembolism Cerebral edema: Pseudotumor cerebri, benign intracranial hypertension Hypertension: Complication of radiation treatment for a brain tumor Brain tumor Hydrocephalus Subdural hematoma • Vascular disorders Migraine headaches As a component of tension headaches Primary hypertension or hypertension secondary to renal disease, pheochro mocytoma, carcinoid tumors; more typically seen in hypertensive crisis • Disorders of the eyes, ears, nose, and throat Eye disorders: eye strain, optic neuritis, glaucoma Pain which is actually referred otalgia (secondary to disorders of the teeth, nasopharynx, temporomandibular joint) Sinusitis Otitis (acute or chronic) Hay fever • Systemic and metabolic disorders Endocrine dysfunction (hypoglycemia, diabetes mellitus, hyperthyroidism) Collagen vascular disorders Acute and chronic infections other than CNS, particularly with fever and toxicity Dialysis patients • Musculoskeletal disorders Tension headaches Cervical spine disorders Systemic lupus erythematosus Cervicogenic headache (cervical spine dysfunction) • Food and drug-related disorders Caffeine withdrawal (coffee, tea, cola beverages, cocoa) Ergotamine withdrawal (in relation to migraine treatment) Use/abuse of alcohol, cocaine, marijuana, and other drugs Nicotine use or withdrawal (cigarettes) Monosodium glutamate ingestion in susceptible persons Nitrate and nitrite food preservatives Foods that exacerbate migraine (see text) Chronic use of large amounts of analgesics (as acetaminophen) Carbon monoxide poisoning Side-effects of various medications, including antihypertensives, antidepressants, psychotropics • Psychiatric disorders Psychosis Depression Conversion disorder Contd... 160 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Contd... • Miscellaneous Exercise Trauma (as postconcussion) Excessive video game use Lack of sleep Temporal arteritis Trigeminal neuralgia Sick building syndrome Effect of toxins, such as lead Electrolyte imbalance Complication of lumbar puncture Malingering Migraine with CSF pleocytosis *Reprinted with permission from: Greydanus DE, Van Dyke D: “Neurologic Disorders” In: Greydanus DE, Patel DR, Pratt HD (eds): Essential Adolescent Medicine, New York: McGraw-Hill 2006;251.4

Evaluation A careful history is necessary when evaluating a youth with headaches, including specific features of the symptom such as intensity, quality, localization, frequency, duration, trigger factors, and aura or associated manifestations (i.e. emesis, fever, weight loss, visual dysfunction, recent neurological losses, or recent personality changes). Open-ended questions help the patient reveal valuable information and give a more accurate description of the pain. Family history and impairment of daily function are also points of interest. Ten-point scales can be used for intensity and a prospective headache diary can be very useful to both the patient and clinician.8 There are some worrisome features that imply possible severe underlying pathology needing appropriate investigation and management. These include recent onset and gradually worsening headache, acute presentation, morning attacks with vomiting, dizziness with memory or sensory loss, and abnormal neurological or other physical findings.8 A thorough physical examination is necessary, with special attention to the blood pressure, eyes (including fundi and visual acuity), ears, nose, throat, sinuses, temporomandibular joints, auscultation of skull and orbits, cardiovascular and neurological system. Table 14.2 lists principles of the clinical evaluation and laboratory studies to consider in the work-up of adolescents with severe or chronic headaches. Neuroimaging can be Headaches in the Adolescent 161

Table 14.2: Clinical and laboratory evaluation of headaches 1. History Location, description, duration, frequency, time of day or night, relation to time of year, effect on daily activity, sleep patterns, diet history, menstrual history, precipitating events or warning symptoms, disrupts sleep or interrupts activity, how relieved, associated symptoms and signs, other health and mental health issues, including use of drugs, alcohol, cigarettes, oral contraceptives, family history, psychosocial history, response to medication trials. 2. Physical examination Particular attention to blood pressure, eyes (including fundi and visual acuity), ears (including Weber and Rinne tests and auditory acuity), nose, sinuses, TMJ, auscultation of skull and orbits, throat, cardiovascular system, neurologic system 3. Laboratory tests* a. Routine: Complete blood count and urinalysis b. Erythrocyte sedimentation rate c. Blood values: Glucose (fasting or tolerance test); if urinalysis is abnormal: urea nitrogen and creatinine d. Roentgenograms: Cervical spine, sinuses, ear and mastoid, temporomandibular joint e. Other imaging procedures: Head CT or MRI; except for evaluation of the cervical spine, the CT may be the better initial choice f. Lumbar puncture† g. EEG h. Visual fields, intraocular pressure i. Specialized neurologic and neuroendocrine procedures j. Therapeutic trial with selected medications in migraine suspects k. Psychologic or psychiatric evaluation l. Others: Based on the specific situation *To be preformed according to clinical judgment as suggested by prior findings and clinical course. A careful history (including family history) and examination remain the mainstay of headache evaluation. The yield of imaging studies is low in patients who have no history or physical findings suggestive of a space-occupying lesion inside the skull (i.e. brain tumor, arteriovenous malformation, aneurysm, meningoencephalitis, or pseudotumor cerebri). †Lumbar puncture is only useful for suspected infection or pseudotumor cerebri. *Reprinted with permission from: Greydanus DE, Van Dyke D. Neurologic disorders. In: Greydanus DE, Patel DR, Pratt HD (eds):Essential Adolescent Medicine. New York: McGraw-Hill 2006 : 235-279.4 considered for those with neurologic complications, such as papilledema, vision compromise, and epilepsy. Neuroimaging should also be done for those with headaches that occur in the morning (during waking from sleep) or with mental status changes. Stable headache patterns for > 6 months usually do not require imaging studies.9 162 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Psychiatric comorbidity (i.e, anxiety, depression, somatization) is an important risk factor for the development of headache in adolescents.2 It is estimated that one in three examined headache patients need psychological support and therapy. All headache sufferers must be screened for mood or anxiety disorders, either by direct questions or psychological testing.1,2,5

MIGRAINE HEADACHES Headaches are noted in as many as 12 to 15% of adolescents and adults, with a male to female ratio 1:3.5.1-4 The term “hemicrania” was first used by Claudius Galen (130-201 AD), the Greek physician living in Rome. This Greek word, “hemicrania,” means “half of the head” and became the English word for migraines.1 Migraine headaches have a genetic basis, and the family history is positive in as many as 90% of these cases. About 33% of migraineurs become symptomatic before puberty. The incidence of migraine elevates in adolescence, peaking in the late adolescent years. The prevalence has remarkably increased during the last decade, especially in girls over 12 years of age.10 Youngsters suffering from migraine may be at increased risk for depression, epilepsy, strokes, panic attacks, and other anxiety disorders. A variety of migraine headaches are described, including migraine with aura (classic migraine), migraine without aura (common migraine), and migraine variants.1-4 The International Headache Society criteria for migraine (Table 14.3) are still the “gold standard” for diagnosing migraineurs, although some criteria are not so common in youngsters.6 Current theories on the cause of migraine headaches implicate neuropeptide release from the trigeminovascular system; this electrochemical event releases such chemicals as substance P, neurokinin A, and others.11,12 5-HT (5-hydroxy-tryptamine), or serotonin, may be released from platelets as the migraine event starts; 5-HT works on different receptors causing vasodilatation or vasoconstriction in various blood vessels. At least four types of 5-HT receptors are noted and classified as 5-HT.1-4 Dysregulation of serotonergic and noradrenergic systems is common in migraine headaches, depression and anxiety disorders; this may lead to the increased risk for some individuals to develop both psychological and head pain symptoms.13 A variety of factors may precipitate migraine headache in various individuals, such as stress, bright lights, exercise, menstruation, fatigue, ovulation, humidity or barometric changes, head trauma, change Headaches in the Adolescent 163 in sleep (too little or too much), strong odors, allergens, and increase in blood pressure. A number of drugs and some types of food can also induce migraine headaches in some cases, including oral contraceptives, caffeine, tyramine, red wine, chocolate, nitrate preservatives, some cheese categories, monosodium glutamate, reserpine, vasodilator drugs, and others.4

Table 14.3: International Headache Society (HIS) criteria for migraine6 1. Episodic attacks 2. 2-48 hours duration (4-72 hours in those 15 years of age and older) 3. Moderate or severe intensity 4. Unilateral localization of pain 5. Pulsating quality 6. Worsening with activity 7. Nausea +/- vomiting 8. Light and sound hypersensitivity 9. Visual disturbance, unilateral neurological manifestations, speech impairment

Migraine with Aura (Classic Migraine) This migraine type begins with a well-defined aura that starts suddenly and lasts 10-30 minutes. The aura may be visual (seeing spots, lights, or other phenomena), motor, sensory, or vocal. In uncommon cases, there may be fainting, confusion, and even coma. The aura is followed by a throbbing headache that is unilateral, can last 4 to 72 hours (2 to 48 hours in those under age 15 years), and may be complicated by the addition of nausea, vomiting, and anorexia. It can be worsened with physical activity and may be seen with photophobia (hypersensitivity to light) and phonophobia (sound hypersensitivity). Confusion with various types of seizure disorders is common among clinicians. Studies in adult females note increased risk of cardiovascular disease in those having migraine with aura.14

Migraine without Aura (Common Migraine) This is similar to classic migraines, except that there is no aura or the aura is not well-defined. It is one-sided in two-thirds of cases, and bilateral in about one-third. Major precipitants include stress, sleep pattern change, and caffeine withdrawal. Its frequency varies considerably, from one episode in several months to daily headaches. 164 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Atypical and Rare Forms of Migraine and Migraine-like Headaches Patients with ophthalmoplegic migraine develop a migraine headache along with oculomotor paralysis that can last three to five days; this paralysis usually clears fully, but persistent nerve damage may also be seen. Amaurosis fugax refers to temporary spasm of the retinal artery in one eye leading to transient vision loss in that eye in migraine patients; spontaneous clearing occurs unless there are other complications. Those with hemiplegic migraine develop hemiplegia, hemisensory deficits, or aphasia. Spontaneous resolution is usually the rule, though cerebral infarction has been noted. Familial cases have been linked to chromosome 19 (subtype1) or chromosome 1 (subtype 2). Subtype 1 has more severe clinical manifestations. Basilar artery migraine (Bickerstaff’s syndrome) is noted in females in relation to menstruation. There is an aura for 20 to 30 minutes that reflects cranial nerve pathology—emesis, ataxia, weakness, tinnitus, vision loss, loss of consciousness and others. The aura is followed by a severe, throbbing, occipital headache. Some patients with migraine headaches also have recurrent episodes of abdominal pain, and this is called migraine equivalent. In addition, some adolescents who have epilepsy with occipital paroxysms can have migraine-like headaches; there can be daytime, visual hallucinations and a post-ictal headache pattern. Finally, cluster headache, the most painful and distressing of primary headaches, is a rare disorder with a prevalence of 0.09-0.4% in adult males and 0.1% in young people < 18 years of age.15 The IHS committee has classified cluster headache as distinct from migraine; it has a shorter duration of 30 minutes to 3 hours.6 It is described as a strictly unilateral, hot, burning pain, with associated ipsilateral autonomic features (i.e. conjunctival injection, tearing, forehead and facial sweating, nasal discharge, ptosis of the eye as partial Horner’s syndrome).16 Systemic manifestations, such as bradycardia and hypertention, are also noted in cluster headaches. Patients are restless and may feel relief by exposure to cold air. The headaches tend to occur at night, last up to 1½ hours, and recur in clusters of 8 to 12 weeks.16

Migraine Management A careful evaluation is necessary in order to identify the type of migraine headache in a particular adolescent, as well as the factors that seem to Headaches in the Adolescent 165 precipitate headaches or any complicating factors.3, 4 Underlying infections should be identified and treated, such as ear, dental, or sinus infections. Underlying or co-morbid epilepsy should also be identified and managed with appropriate antiseizure medication. It is often helpful to deal with underlying stress, establish proper sleep-rest habits, and recommend moderate caffeine intake, if excess consumption is noted. A careful search for the best medication to control the headaches is important; narcotic medications are to be avoided. Counseling may help by dealing with underling emotional problems.2,17 It is always important to encourage adolescents to fully understand their situation, teach them ways to cope with environmental factors, avoid underlying precipitating factors, and sufficiently take care of themselves.2,5 A minimal headache intervention program is presented in Table 14.4. A variety of medications can be used to treat the acute migraine headache. Some youth do well with analgesics, such as nonsteroidal anti- inflammatory agents–NSAIDs (ibuprofen or naproxen sodium) or ergotamine tartrate (many brands). A variety of analgesics are available, providing a combination of medications: Caffeine, acetaminophen, butalbital, and others. Antinauseant medications may be helpful as well, including promethazine, trimethobenzamide, and hydroxyzine. Prednisone has been used for refractory migraines. Prednisone can be given in 30 to 60 mg doses per day over 3 to 5 days, followed by a tapering over 1 week. The most effective medications used for the acute management of migraines are the triptan medications. These are serotonin (5-HT) agonists used to constrict intracranial blood vessels and reduce sensory nerve inflammation (Table 14.5).1,3,4,11 Clinicians can see which medications are available and which ones help a specific patient. For example, sumatriptan can be given at a dose of 25 mg to 50 mg orally and repeated in 2 hours if necessary; the maximum dose for adults is 200 mg a day. It can be given as an intranasal medication at 20 mg and repeated in 2 hours; a subcutaneous dose is also available as 6 mg and repeated in 1 hour if necessary. Zolmitriptan is given as 2.5 mg or 5 mg, repeated in 2 hours if necessary, with a maximum daily dose of 10 mg for adults. It is also available as an intranasal medication at 5 mg and repeated in 2 hours if necessary. Current research is looking at developing medications that act as antagonists for the neuropeptide calcitonin gene-related peptide 166 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

(CGRP), since the release of CGRP from trigeminal nerves seems to play an important role in the development of migraine headaches.1,3,4,11,18-21 Indications for daily administration of medication in order to prevent migraine attacks are : Three or more episodes per month causing severe dysfunction, frequent headaches leading to therapeutic drug overuse, treatment failure, attacks with extended aura or severe clinical manifestations. A variety of medications have been used to prevent migraine headaches, as listed in Table 14.6.

Table 14.4: Minimal therapeutic approach for headache patients 1. Reassurance that the situation is not life-threatening 2. Minimization of stress factors 3. Establishment of proper sleeping-rest habits 4. Control of trigger factors 5. Adjustment of dietary habits (i.e. reduction or avoidance of caffeine, red wine, chocolate, nitrate foods-hot dogs, salami, Chinese food containing monosodium glutamate, nuts, other precipitating foods) 6. Recommendation of relaxation strategies 7. Psychological counseling if necessary

Table 14. 5: Triptan medications for migraine headaches • Sumatriptan (Imitrex) • Zolmitriptan (Zomig or Zomig ZMT) • Almotriptan (Axert) • Eletriptan (Relpax) • Frovatriptan (Frova) • Rizatriptan (Maxalt, Maxalt-MLT) • Naratriptan (Amerge)

Table 14. 6: Migraine prophylactic medication • Non-steroidal anti-inflammatory drugs (NSAIDs) • Propranolol hydrochloride (Inderal) • Calcium-entry blockers (verapamil, nifedipine, nimodipine, others) • Anticonvulsant medication (topiramate, valproic acid, gabapentin) • Antidepressant medication (amitriptyline, imipramine, fluoxetine) • Acetazolamide (Diamox) (For some females with menstrual period-related migraines) • Cyproheptadine hydrochloride (Periactin)

TENSION HEADACHES It is the most common type of headache (80% of the general population has had an experience with this type of headache), and yet it is the one we Headaches in the Adolescent 167 know less about. Women suffer 3 times more often than men. Tension headache or tension-type headache refers to pain generated by spasm of various head and neck muscles, with or without accompanying vascular headaches.1,3,4,22 Stress may be the underlying cause, though the exact etiology is not often clear. It is more common than migraine headaches and the majority of the population has experienced this type of headache at some point in their lives. The episode tends to be more common in the afternoon or evening and during the school day than the weekend. The pain can last from minutes to hours or days. It is often described as a dull ache over the head that is constant with a feeling of tightness over the forehead, head, and neck. The family history is often positive and it may worsen with overt stress in the adolescent’s life. There is no aura, but the physical examination may reveal tenderness or spasm in various head or neck muscles. Some youth may have both tension headaches and migraine headaches; they also may have headaches with features of both types. Management of tension headaches involves methods to reduce stress in the youth’s life (i.e. counseling, psychotherapy, biofeedback, yoga, etc.) along with the judicious use of analgesics (i.e. acetaminophen, aspirin, NSAIDs, others). The use of acupuncture is under research, though some studies support its use.23 Reassurance about the basic benign nature of tension headaches is also helpful. The use of antidepressant medication may be helpful, whether an underlying depressive disorder is identified or not. Narcotic medications should be avoided, since tension headaches may turn into a chronic condition and addiction to narcotics may then develop. Some youth present with rebound headaches that are related to frequent use of analgesics. Management involves getting the patient off medications. This recommendation is not easily accepted by the headache patient who is in a daily cycle of taking “pain” medications. Both migraine and tension headaches are very common causes of pain in adolescence, affecting daily routines of home, school, and social life. Direct (health care expenses) and indirect (reduced productivity, absenteeism) social costs emerge when the situation persists in adulthood. Early detection and appropriate intervention are the keys to minimization of the above consequences for adolescents and their future quality of life. 168 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

KEY LEARNING POINTS • The two main types of headaches are due to intracranial vasculature dysfunction (migraine headaches) and spasms of the external muscles of the head, face and neck (tension headaches). • According to the International Headache Society (IHS) classification system, headaches are mainly divided into primary types (migraine, tension and cluster headaches) and secondary types (due to an underlying organic cause, such as trauma, infection, or neoplasm). • Intracranial pathology must be considered and excluded in adolescents with chronic headaches (> 15 days/month); fortunately, investigation is rarely positive for a neoplasm. • The most effective medications used for the acute management of migraines are the triptan medications. • Management of tension headaches involves methods to reduce stress in the youth’s life (i.e., counseling, psychotherapy, biofeedback, yoga, etc.) along with the judicious use of analgesics (i.e., acetaminophen, aspirin, NSAIDs, others).

REFERENCES 1. Brandes JL, Saper JR, Diamond M, et al. Topiramate for migraine prevention: A randomized controlled trial. JAMA 2004;291: 965-973. 2. Bruener CC. Alternative and complementary therapies. Adolesc Med Clin 2006; 17: 455-467. 3. Cutrer FM, Silberstein SD, Mathew NT, et al. Antiepileptic drugs in migraine, cluster headache, and mood disorders. Headache 2001; 41(Suppl 1):S1-S10. 4. Durham PL. CGRP-receptor antagonists-A fresh approach to migraine therapy? N Engl J Med 2004;350:1073-1075. 5. Greydanus DE, Pratt HD, Van Dyke HD. Neurologic and neurodevelopmental dilemmas in the adolescent. Adolesc Med 2002;13:231-275. 6. Greydanus DE, Van Dyke D. Neurologic disorders. In: Greydanus DE, Patel DR, Pratt HD (eds):Essential Adolescent Medicine. New York: McGraw-Hill 2006; 235-279. 7. Greydanus DE. Neurologic disorders. In: Hofmann AD, Greydanus DE (eds): Adolescent Medicine. Stamford Appleton and Lange 1997; 242-288. 8. Guidetti V, Galli F. Evolution of headache in childhood and adolescence: An 8-year-follow-up. Cephalalgia 1998;18: 449-454. 9. Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgia, and facial pain. Cephalalgia 1988; 8 (Suppl 7): 1-96. 10. Hyekyun R. Prevalence and predictors of headaches in US adolescents. Headache 2000; 40: 528-538. 11. Just U, Oelkers R, Bender S, et al. Emotional and behavioral problems in children and adolescents with primary headache. Cephalalgia 2003; 23: 206-213. Headaches in the Adolescent 169

12. Kurth T, Gaziano JM, Cook NR, et al. Migraine and risk of cardiovascular disease in women. JAMA 2006; 296: 283-291. 13. Lampl C. Childhood-onset cluster headache. Pediatr Neurol 2002; 27: 138-140. 14. Lewis DW. Migraine headaches in the adolescent. Adolesc Med 2002; 13:413-432. 15. Lipton RB, Pan J, et al. Is migraine a progressive brain disease? JAMA 2004; 291:493-494. 16. Maizels M. The patient with daily headaches. Am Fam Physician, 2004; 70, 2299- 2306. 17. Mathew NT. Antiepileptic drugs in migraine prevention. Headache 2001;41(Suppl 1):S18-S24. 18. Mitsikostas DD, Thomas AM. Comorbidity of headache and depressive disorders. Cephalalgia 1999; 19: 211-217. 19. Pakalnis A. Nonmigraine headaches in adolescents. Adolesc Med 2002 ;13:433- 442. 20. Reeve A, Strasburger VC. Basic principles of psychosomatic medicine in children and adolescents. In: Greydanus DE, Patel DR, Pratt HD(eds): Behavioral Pediatrics, 2nd edn. New York: Universe 2006: 739-768. 21. Silberstein SD. Practice parameter: Evidence-based guidelines for migraine headache (an evidence based review): Report of the Quality Standards Subcom- mittee of the American Academy of Neurology. Neurology 2000; 55:754-62. 22. Strine TW, Okoro CA, McGuire LC, et al. The associations among childhood headaches, emotional and behavioral difficulties, and health care use. Pediatrics 2006; 117: 1728-1735. 23. Thomas S, Kanupuru D. The clinical features of cluster headache: Differential diagnosis and treatment recommendations. Res Staff Phys 2006; 52: 14-20. RAP Special Volume 19: Developmental and Behavioral Pediatrics

The Adolescent with Spina Bifida: Psychological Aspects of 15 Development

Tina Head

INTRODUCTION Refined surgical techniques, prophylactic procedures, and diligent monitoring for urinary tract deterioration have coalesced into treatment strategies with increased longevity and quality of life for a burgeoning population of adolescents with spina bifida. Medical advances were made possible following the development of the one-way valves for ventriculoperitoneal or ventriculatrial shunts in the late 1950s.1 As a result of the complexity of primary and associated disabilities, multidiciplinary teams comprised of specialists such as neurosurgeon, orthopedic surgeon, orthopedist, urologist, pediatrician, neurologist and ophthalmologist are considered the standard of care for this population.2 Psychologists and social workers also are considered vital members of multidisciplinary teams due to psychosocial and mental health concerns and the need for families to access programatic supports.1 While most states in the US have children’s clinics—Alaska, Montana, Nevada, and Wyoming are the exceptions—only 62 clinics in 25 states coordinate the specialized care necessary for adults with spina bifida.3

BACKGROUND Genetic and environmental factors are believed to combine to create causal factors in spina bifida, with the prevalence rate varying widely from among ethnicities and countries.4 Rates are higher among Caucasian and Hispanic populations and rarely occur in those of African or Asian descent.5 In Ireland, 4.5 out of 1,000 have the spinal cord malformation, while less than 0.1 out of 1,000 in Columbia are affected.5 The incidence rate in the The Adolescent with Spina Bifida 171

US is in between, with 0.7 out of 1,000 live births affected. However, the rate declined 31% following the mandate of supplementation of wheat- based and other products with folic acid in 1998.6 Also, geneticists from Australia have identified a gene in mice that causes the spinal cord malformation, and current studies are examining DNA from children with spina bifida.7 Medical surgical advances and prophylactic treatment have resulted in 80 to 90% of the children born today surviving into adulthood.8-10 Those born with these spinal cord lesions have some degree of paralysis, neurogenic bladder, and Chairi II malformations. A majority of these children also have hydrocephalus, corpus callosum abnormalities, spine and hip malformations, and club foot. A myriad possible secondary conditions may develop, i.e. seizures, cognitive deficits, attention problems, nonverbal learning disorders, strabismus, latex allergy, obesity, decubitus ulcers, obesity, scoliosis, or kyphosis, any combination of which make treating adolescents with spina bifida a challenge in meeting their needs throughout their lifespan (see Table 15.1). A majority with sacral-level lesions generally have normal ambulation, with about one-third showing reduced muscle strength.11 Reports of low-lumbar community ambulators vary from 38 to 95%, with about 30% of those with high lumbar-level lesions able to walk at school and shop with the use of orthotics.12, 13 Aggressiveness in bracing to promote walking for those with thoracic- level lesions vary widely, with reports of up to 33% being community ambulators.13 By adolescence, many with lumbar and higher lesions will be using a wheelchair at least part time. Increasing reliance on wheelchair use by this group is the result of weight gain, center of gravity and strength change, and energy expenditure (wheelchairs are appreciably faster and use relatively little energy).14 Spina bifida is Latin for split (bifid) spine and is used as a lay term for myelomeningocele/meningomyelocele, the most common form of spinal malformation.15 Myelo refers to the spinal cord, meningo stands for the meningeal layer that covers the spinal cord and brain, and cele means cyst.10 Spina bifida is an umbrella term that encompasses several related conditions that vary in severity, including occulta, meningocele, and myelomeningocele (commonly meningomyelocele in Europe). Spina bifida occulta or the “hidden” form occurs in 10 to 15% of the population, and 172 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics involves a failure of the vertebrae to close.10 On rare occasion, a person with spina bifida occulta may experience degenerative symptoms later in life. With a meningocele, the least common defect, the meninges herniate through the vertebrae and is usually skin covered. If nerves are not affected, there is usually no neurological damage; however, the person may have sensory and minor motor deficits.16 Myelomeningocele is a type of cyst or cele that occurs during the embryonic stage, approximately 28 days after conception, when the neural plate begins to fold up into a tube (the future neural tube) and fails to close somewhere along the spine.15 Nerves form abnormally in this cele, or cyst, causing full or partial interruption of neural impulses, resulting in paralysis. Although the term spina bifida emphasizes the opening of a vertebral segment, it encompasses most forms of spinal cord malformations. For purposes of this text, spina bifida is used interchangeably for myelomeningocele/meningomyelocele, lipomyelomenin- gocele (myelomeningocele with fatty tissue), and lipomeningocele (meningocele with fatty tissue). Spina bifida is primarily a neurological disorder, with 80% having shunted hydrocephalus and other affects on the brain.14 Virtually all children with spina bifida have the Chiari II malformation, which is where the hindbrain is pulled down further into the cervical vertebrae, causing compression of the hindbrain. Usually, this is not severe enough to cause symptoms, but when compression is severe, the Chiari II malformation causes breathing and swallowing problems, and is the leading cause of death in newborns with spina bifida.14 This compression also is usually responsible for blocking the flow of CNS fluid to the spine and body where it can be absorbed by the body. Other associated conditions that may develop include psychosocial problems, depression, and anxiety. Although children with spina bifida are at higher risk of adjustment problems17 and low self-esteem, some recent studies show teens with spina bifida report self-esteem and self- worth that is comparable to their siblings or peers.18-20 Early and current studies show these patients have plans for a vocation or career, and aspirations for marriage and family that is typical for adolescents.

PHYSICIAN ATTITUDE Parents of children with spina bifida and other disabilities often report their physicians predict “an excessively negative impact on the family”;21 The Adolescent with Spina Bifida 173 however, there is a significant difference between pessimistic physician predictions and actual parent responses to raising a child with spina bifida.21 Data supports the more optimistic parental perception of daily life for these families, therefore physician apprehension appears to be largely unfounded.21 In fact, teens report more positive relationships and less conflict with parents than do nondisabled adolescents.22, 23

LIFE SKILLS AND FAMILY FUNCTION Life skills that are necessary for a healthy adolescent development may be of greater importance for those with spina bifida.24 The Carnegie Council on Adolescent Development identified several domains of functioning that all teens must achieve for healthy development—these domains are vital for healthy psychosocial adjustment for those with spina bifida and other physical disabilities:25 • Find a valued place in a constructive group • Feel a sense of worth as a person • Achieve a reliable basis for making informed choices • Know how to use the support systems available to them • Believe in a promising future with real opportunities • Find ways of being useful to others • Master social skills, including the ability to manage conflict peacefully • Cultivate the inquiring and problem-solving habits of mind for lifelong learning.

Teens with spina bifida have been found to be independent in daily living skills, but typically are not participating in the full range of activities necessary for a successful transition to adulthood (e.g., decision making, friendship activities, household responsibilities, managing money). Parents have the largest sphere of influence for these domains, particularly for relinquishing decision-making control, self-care, money management, and ensuring their teen makes meaningful contributions to household tasks. In multiple studies, decision-making maturity for teens with spina bifida was found to be underdeveloped.24 Forty-five percent of this sample reported they never did tasks which are age-appropriate for much younger children. These very low scores on the household responsibilities index reflected a lack of participation in routine chores such as setting the table or washing dishes.23 The main source of an adolescent’s self-esteem comes from successfully completing daily chores and feeling valued/needed in 174 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics his or her family. Also, a majority of teens with spina bifida report they are not employed nor do they receive allowance, and 41% reported they have no way of obtaining spending money.24 Families also can help with increasing activities with friends outside of school. This sample reported they rarely went shopping, school activities, movies, or parties, all of which are activities that can be facilitated by families providing transportation and encouragement.

PARENTAL ISSUES Parental supervision is necessary at the beginning; however, too much vigilance and these preteens and teens feel intrusion and deprived of the ability to make their own decisions.26 When the process goes awry, parental demands for compliance increase concurrent with child resistance until the helping process deteriorates. The teenager may refuse treatment or may feign incompetence in an effort to get his parents to reduce their demands. This behavior also may act to engender a sense of autonomy and control. “Intrusiveness” in this context is the defining feature of psychological control, the hallmark of “miscarried helping”.26 This type of familial interaction occurs when parents’ efforts to achieve a positive medical outcome for their child leads to increasingly coercive interactions that are detrimental to the child’s ability to function independently. Likewise, excessive assistance, constant vigilance, and frequent prompts for self-care by parents who are overly deferential and want their child avoid activities are likely to negatively impact their teen’s well being. Adolescents with overprotective parents have lower self-esteem scores, are more self-conscious, less popular, and report being less happy compared to youth with spina bifida who did not perceive their parents as overprotective.22 Conversely, parents who facilitate psychological autonomy while continuing to provide discipline, monitoring, warmth, and affection appear to aid the best adolescent adjustment outcomes.27, 28 As teens with physical disabilities approach adolescence, part of the natural independence process includes progressively assuming responsibility for medical regimens, much as typically developing children are granted more autonomy.28 Optimal parenting of a teen with spina bifida occurs when parents monitor compliance with medical regimens and insist their child contribute to their household by completing a reasonable number of chores of which they The Adolescent with Spina Bifida 175 are physically capable. The crucial element is for the parents and child to do problem-solving around modification of household tasks, (e.g. using a wheeled cart to clear dinner dishes and move those dishes to the kitchen to be loaded into the dishwasher). If families are unable to generate ideas for chore modification, occupational therapists can visit the home and generate solutions and recommend adaptive products for the family.

SELF-CONCEPT In addition to social support by parents, it is well established that self- ratings of physical appearance, and, to a lesser degree, peer acceptance, are strong predictors of self-worth in able-bodied adolescents.18-20 Researchers and health-care professionals who work with adolescents with spina bifida and other physical disabilities often predict this group will have lower self-image and less confidence in themselves than their nondisabled peers. When severity of disability is measured in self-concept studies, most professionals also predict increased severity of the disability will further decrease spina bifida adolescents’ sense of self-worth. Indeed, until the late 1980s authors found diminished self-esteem was common among this group.30, 31 More recently, however, emerging evidence shows adolescents and young adults with spina bifida have a sense of self-worth that is comparable to non-disabled peers.19 Research findings for the effect of severity on self-concept for these adolescents is inconsistent and may depend upon the definition of severity for different studies. In general, higher-level lesions indicate more severe impact of the disability, with increasing need for ambulatory assistive devices. A 1983 study found those who relied more on assistive devices had lower self-concept scores than those who need less;20 and like self-concept studies that did not examine severity, recent studies found no effect of severity of disability on self- worth for adolescents with spina bifida.18,28 With a majority of children having spina bifida attending their neighborhood school, these students identify with able-bodied peers, but feel less academically and athletically competent and less accepted.19 A study published in 1994 found no differences in global measures of self- concept or self-worth measures for youth with spina bifida; however, closer evaluation of the data showed older female teens with spina bifida valued themselves less than did other adolescents with spina bifida and nondisabled teens, and younger females with spina bifida placed the most 176 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics importance on physical appearance.19 This study also found physical appearance and social acceptance were more strongly associated with self- worth for girls with spina bifida than for boys with the disability. A 2004 study found no gender differences, but older teens with spina bifida and spinal cord injuries had lower self-worth scores than did younger participants.18 In a study that measured how teens with spina bifida rated themselves on measures of body image, authors’ predictions of low scores for this group were unsupported.32 Body image scores for this sample were just slightly below average and did not differ significantly from scores of typical teenagers. Studies that examined urinary incontinence have not found differences in self-concept between continent preteens and teens with spina bifida and control group participants.33,34 However, incontinent adolescents— especially females—were found to be especially at risk for low self-worth and social acceptance than continent youth with spina bifida or controls. Incontinent females with spina bifida rated themselves as being less socially accepted and having lower self-worth than continent females; and incontinent spina bifida males rated themselves lower on physical appearance, social acceptance, scholastic competence, and behavioral conduct compared to continent males.34 Surprisingly, one study found preteens and teens in the group who had more frequent bowel accidents scored better on measures of self-concept than those who had fewer accidents.20 The authors speculated that predictability or habituation to accidents decreases worry about bowel accidents and, therefore, did not affect self-concept scores.

SOCIAL SKILLS AND SOCIAL ISOLATION Lack of social competence increases the risk of peer rejection and decreases social opportunities35,39 Some studies link social skills deficits with social isolation and depression in this population.14, 31 Studies that examined social competency in youth with spina bifida are not in agreement: Several found this population has social skills deficits,38,44,45,58 while others did not.36,37 A study that used rated observations of role-play tests did not find differences between children with spina bifida and controls.37 In another study that did not find differences in parent and teacher ratings of social skills between youth with spina bifida and their siblings, the authors speculated that parents and teachers are more tolerant of social skills deficits in The Adolescent with Spina Bifida 177 children with physical disabilities than they are of children with ADHD or learning disabilities.36 While parents and teachers may overlook social shortcomings, children’s peers are unlikely to be as accepting.37 While sociometric studies have not been done for teens with spina bifida, children with disabilities have been found to have lower sociometric status, meaning they are less often chosen as friends by nondisabled students.38 In general, disabled children are less accepted and more rejected than same-age peers. School achievement and physical attractiveness are positively associated with sociometric status;39 however, these are mediated by social competence. Thus, teens with spina bifida would likely benefit from social skills training that helps them interact effectively with both initiation and positive responsiveness to initiations of others. But, it is not enough to have acceptable social skills. Identification with peer groups that develop during adolescence are a vital part of normative development, and is especially important for teens with a disability. The experience of feeling part of a peer group is more likely to positively impact self-esteem than having individual friendships.19 Social isolation was the largest concern reported by Blum and his colleagues, who noted most adolescents with spina bifida have at least one close friend, but only 16% reported going to their friend’s home.22 Only 15% of these teens were dating, while they reported that half of their friends went on dates. Peer home accessibility for teens who use wheelchairs is a largely insurmountable problem. Portable ramps may work for solving accessibility problems of low-height thresholds, but since incline for ramps is 1 inch per foot, temporary ramps for typical thresholds of 12 to 33 inches are not practical. While younger, lighter children may be safely lifted into friend’s homes; by adolescence carrying teens is neither realistic nor safe. An acceptable alternative is for youth with spina bifida to meet their friends out in the community. Teens typically gather at local malls, arcades, movie theaters, sporting events, and restaurants, all of which are accessible. Pediatric social workers are great resources for directing families to programs that can help with transportation issues. It is incumbent upon clinicians and other team members to work with these teens and their families to remove physical and behavioral barriers to social opportunities. 178 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

DEPRESSION AND SUICIDAL IDEATION When clinical depression is suspected, physicians must first eliminate medical conditions which can induce or mimic depressive symptoms, including: shunt malfunction, severe infections, and medication side- effects. With amelioration of a causal underlying medical condition, depressive symptoms disappear.40 Without an underlying medical cause, obtaining a patient history is vital, and screening for depression is essentially the same as for nondisabled teens. In the exam room, doctors can observe whether the patient makes eye contact or has flat affect. What are the teen’s current health problems, medications, family history, school functioning, and peer/social support? Does the patient have other family members with depression or mental illness? Are there problems at home? Does he have activities, he currently enjoys or used to enjoy? Does he experience depressed mood (teens may refer to depressed mood as feeling “down,” “blue,” “sad,” or “heavy.” He also may label himself as “emo,” a current popular term for cluster of behaviors that includes depressed mood, crying, writing dark stories or poems about death and dying, and cutting or other self-injurious behavior. It is important to note that adolescents with depression may experience irritable rather than depressed mood.41 Is she experiencing problems falling asleep, staying asleep, or with early morning awakenings? Does he have friends at school and outside of school? Have there been changes in school functioning (i.e. missed classes, not doing homework, inability to concentrate). Have there been changes in appetite? Has she thought about hurting herself, someone else, or committing suicide? A positive response to suicidal or para-suicidal ideation warrants a full suicide interview. Behavioral activation, psychotherapy, and/or pharmacological treatment may be warranted. Adolescents with spina bifida may participate in wheelchair sports such as basketball, track, or hippotherapy (therapeutic horseback riding programs), which have been shown to improve mood and physical well-being.42 No studies have looked specifically at treatment outcomes for this population for either antidepressants or psychotherapy. Teens with poor social skills, low self- esteem, and/or learned helplessness especially may benefit from psychotherapy.40 Behavioral activation, which includes increasing pleasurable activities and engaging in exercise, may be an effective The Adolescent with Spina Bifida 179 treatment strategy, particularly when the focus of the treatment is increasing social contact. As with self-concept research, results are mixed as to whether the spina bifida population was at higher risk for depression. Some earlier studies30,31,43 and some more recent44,45 found higher rates of depression among adolescents and young adults with spina bifida; however, some studies found no difference in depressive symptoms when this population was compared to age-matched controls.44 Global self-worth was found to be a mediating factor for effects of perceived physical appearance on symptoms of depression for both youth with spina bifida and matched able-bodied controls.45 The mediating effect of self-worth on depression had the largest effect on the spina bifida group, indicating the way they judge their appearance influences how much they value themselves. Since young female adolescents tend to place more importance on physical appearance and evaluate themselves more critically, they may be at higher risk for reduced self-worth and depressive symptoms. Other studies have found perceived deficit from disability had more impact on depressive symptoms than actual severity of disability.46 Few studies have measured suicidal ideation. Appleton and his colleagues found children and adolescents with spina bifida were at increased risk of having thoughts of suicide, and that perceived physical appearance was significantly associated with suicidal ideation.45 In 1977 Dorner reported that 25% of older teen females with spina bifida she interviewed had thought about suicide within the past year.46

ANXIETY Similarly, few studies have examined the incidence of anxiety in adolescents with spina bifida. However, two studies that looked at excessive worry did not agree. In one study, youth with spina bifida reported experiencing less worry than their siblings.20 Within this small sample, those who used long leg orthotics and/or a wheelchair had less anxiety than others who used a wheelchair exclusively, or short leg braces only, or walked unaided. The second study published in 1998, found 17 percent of the youth with spina bifida had an anxiety disorder, whereas 11% of nondisabled controls, and only 8% of the epilepsy group had an anxiety disorder.47 180 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In general, adolescents who feel they are powerless do not cope under adverse conditions and are unlikely to seek help when they feel distressed.48 Cognitive therapy can focus on shifting locus of control (feeling influence on external events comes from themselves rather than events they do not control). Confronting dysfunctional thoughts, decreasing avoidance behaviors via imaginal exposure, and increasing problem-solving skills are typical cognitive-behavioral therapy techniques that are effective in treating anxiety.48

COGNITIVE FUNCTION The majority of patients with spina bifida have intellectual functioning in the average range;49,50 however, research for this population has shown that several factors negatively impact cognitive function, including hydrocephalus, repeated shunt infections, thoracic-level lesions, and extended absences from school.51,54 Whether or not students with spina bifida have hydrocephalus, they usually have perceptual-motor difficulties50, 52 and many with normal intelligence have specific learning difficulties.51 As a group, youth with spina bifida and hydrocephalus have lower mean IQ scores, more academic difficulties, attention and executive function deficits, and poorer memory.55 Students with spina bifida tend to have better verbal intelligence scores than nonverbal or performance measures, and verbal index scores are better predictors of academic achievement than full-scale intelligence scores.16,52 A study published in 2004 compared measures of attention, executive function, memory, and emotional intelligence in young adults with spina bifida and hydrocephalus in four groups: spina bifida with shunted hydrocephalus, spina bifida alone, hydrocephalus alone, and a healthy control group.53 The authors found both hydrocephalus groups were impaired compared to the controls and those with spina bifida with no hydrocephalus on most tests. Participants with hydrocephalus demonstrated the most difficulty on tests requiring integration of cognitive processes. Attentional deficits, rigidity of thought, and decreased performance suggest a constellation of impairment of executive function with intact crystallized intelligence (school-acquired knowledge and acculturation). The spina bifida only group did poorer than the control group on only one task which tested participants’ semantic fluency. This study supported the findings of Barf and associates who also found all of The Adolescent with Spina Bifida 181 the scores for the spina bifida/no hydrocephalus participants were within normal range and were significantly higher than mean scores generated by participants in the hydrocephalus group.54 One large study evaluated the correspondence of lesion level and qualitative brain abnormalities with cognitive impairment, academic performance, and adaptive behavior in children and adolescents with spina bifida and hydrocephalus.57 The authors found participants with thoracic- level lesions had more malformations in the midbrain and tectum, pons, and splenium compared to magnetic-resonance images of this population with lumbar- and sacral-level lesions. Upper-level lesions likewise are more likely to accompany lower ratings of intelligence, reading, math, adaptive behavior, and personal-living skills. Hispanic/Mexican American participants with thoracic lesions had significantly poorer outcomes for social communication and community-living skills, learning disabilities, and mental retardation. The lower-lesion group had significantly more diagnoses of ADHD.

ATTENTION DEFICIT HYPERACTIVITY DISORDER Burmeister, et al. studied the incidence of Attention-Deficit/Hyperactivity Disorder in children and adolescents with spina bifida and hydrocephalus and found 31% met criteria for the disorder compared to 5% of healthy controls.55 As expected, inattentive-type ADHD was more common in the spina bifida group (23%), with only 0.5% being identified with predominately hyperactive-impulsive symptoms, and 8% diagnosed with the combined-type. The authors note that typical hyperactivity is rarely seen in this population due to motor impairments, and noted that parents endorsed impulsive behaviors and excessive verbal behavior on their instrument’s hyperactivity-impulsivity scale. Additionally, participants with spina bifida had lower ratings on measures of executive functions than the control group, and most notably, the spina bifida participants who had ADHD diagnoses of any type fared significantly worse on executive functions than adolescents and children with spina bifida who did not have ADHD. Burmeister and her colleagues could not attribute differences in ADHD subtypes to cognitive function or the number of shunt revisions. Another study that evaluated attention in youth with spina bifida and their siblings showed the magnitude of attentional deficits increased for those with shunts and repeated shunt revisions.56 The authors 182 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics found history of shunts and other medical variables (agenesis of corpus callosum, high lesion level, and oculomotor abnormalities) accounted for individual differences in attention.

MEMORY Adolescents and children with shunted hydrocephalus consistently have demonstrated impaired memory span6,7,58 and often use inefficient strategies to aid recall.53 A 1995 study found the spina bifida hydrocephalus group learned words more slowly than control and spina bifida only participants, resulting in fewer total words recalled, even though they remembered as many words as those in the comparison groups on the first trial.59 Additionally, these authors found a pronounced recency effect for delayed recall, but no difference in recognition between any of the three groups. Although children and adolescents with spina bifida and shunted hydrocephalus have been found to have explicit memory deficits, implicit memory appears to be largely intact.60 The authors speculated that since implicit memory is fundamental in the acquisition of new skills and knowledge, this population retains capabilities for procedural and other learning, although they may have difficulty remembering specifically how they acquired new skills.

READING AND WRITING SKILLS A recent study that evaluated young adults with spina bifida and hydrocephalus61 found patterns of reading and writing in childhood generally persist into adulthood. This sample of young adults had better ratings of reading accuracy and decoding than reading comprehension; however, this group’s reading comprehension scores were within the normal range. Writing fluency was less developed than reading and received the lowest cognitive score for this test sequence. Like reading skills, these scores were within a standard deviation of the norm mean.

ARITHMETIC SKILLS A study that examined mathematical skills in young adults with spina bifida and hydrocephalus found children who had poor math problem- solving abilities usually also had problem-solving deficits and limited functional numeracy as adults. On average these young adults had lower The Adolescent with Spina Bifida 183 test scores on computation accuracy, speed, problem solving, and numeracy.62 Study results showed that functional numeracy decreased as lifetime shunt revisions increased. The authors note implications for this finding indicate deficits in mathematical skills needed to cope with everyday life greatly impact functional independence.

VISUO- AND FINE-MOTOR SKILLS Method of ambulation might affect some areas of school performance as well.63 A study that evaluated energy expenditure differences when using assistive walking devices (braces and crutches) or wheelchair showed that students exhibited poorer vasomotor skills, which resulted in poorer handwriting and fine motor coordination when they walked compared to days they used their wheelchair. Method of ambulation did not significantly affect reading scores for these participants.

NEUROPSYCHOLOGICAL EVALUATIONS Serial neuropsychological assessment has been found to be vital to monitor cognitive and psychomotor functions over time. Changes in these areas have been shown to detect shunt malfunctions in the absence of acute symptoms.64 This developmental approach is especially important for more severely affected individuals with complex secondary medical conditions; i.e., multiple shunt revisions, callosal agenesis, tethered cord, and seizures. A thorough assessment covers multiple cognitive domains including overall intelligence, and subdomains, which entail perceptual-motor function, attention, impulsivity, hyperactivity, memory, sequencing, organization, and reasoning abilities. Interpretation of test results that require copying or manipulation of objects must consider reduced motor control in the upper extremities for those with high-level lesions or significant psychomotor skill deficits.51 Children may have deficits in any or several of these areas, even when overall intelligence is within the average range or higher. In this population, verbal intelligence, the major portion of overall intelligence, is thought to be a better predictor of academic performance, than the full-scale IQ score. Additionally, a neuropsychological evaluation includes idiographic recommendations to improve functioning and academic progress.65 A baseline assessment should be completed well before the child enters school.12,66 Neuropsychological deficits that stem from congenital 184 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics hydrocephalus are usually evident in this initial evaluation.11,12 Perfor- mance during the baseline evaluation can be used to monitor changes throughout childhood and adolescence. Neuropsychologists who specialize in assessment and treatment of this population recommend testing approximately every 2-3 years during childhood and less frequently thereafter.11, 12 Subsequent neuropsychological testing batteries have been shown to be sensitive to deterioration of distinct cognitive processes, the source of which may be a shunt malfunction.12 The typical neurocognitive profile of children with spina bifida with hydrocpehalus includes general/verbal intelligence in the average to low- average range, with verbal skills exceeding nonverbal abilities.11,14 Common problems include perceptual-motor deficits, poor comprehension, inattentiveness, restlessness, poor memory, disorganization, poor sequencing, reduced reasoning and problem-solving skills.11, 14

TRANSITION TO ADULTHOOD The developmental process of achieving independence in adulthood begins in adolescence, and for teens with spina bifida, the journey requires more planning, coordination and diligence than is necessary for most youth. For students 14 years of age who have individualized education plans, transition planning is mandated and is finalized during annual IEP meetings.66 Adolescents who have accommodations at school via 504 plans should begin planning for college or vocational training at this time as well. Many high schools have vocational programs that offer education credits for part-time work experience (usually no more than 10 hours per week) supervised by school personnel. Vocational planning is vital for these teens: National unemployment rates for all disabled adults in the US in 2005 was a dismal 38%, compared to 77% of the nondisabled population;67 however, surveys of clinical spina bifida populations published in the 1970s and 1980s consistently show approximately 66% of these adults were employed.68 Medical care transition from parent to child typically begins when children begin catheterizing or performing other routine medical tasks independently. Pediatric multidisciplinary team members can further the adolescent’s transition to managing their own medical care by having the youth gradually take responsibility for scheduling appointments, ordering supplies, etc. This, too, is an opportune time for parents and/or team The Adolescent with Spina Bifida 185 members to check the adolescent’s understanding of the effects of myelodysplasia and his/her medical care needs. Instead of completing driver’s education before purchasing a car, as most youth do, adolescents with spina bifida who require hand controls must provide a car or van with modifications to take driver’s training for most programs in the US. Often state departments of rehabilitation or local organizations will help finance the cost of vehicle modifications. Some national private foundations also have helped with the purchase of vans or cars. Psychomotor deficits, experienced by most with spina bifida, affects driving skills. A study that examined the learning period and early driving experiences of drivers with spina bifida and hydrocephalus, cerebral palsy, and able-bodied controls found the disabled drivers took longer to learn to drive than nondisabled peers.69 Additionally, in their first year, the disabled drivers drove less, but had more accidents than the controls; they also were less likely to travel on unfamiliar routes and had more difficulties planning and reading maps. Although drivers with spina bifida and cerebral palsy reported more difficulties than able-bodied peers, the types of problems experienced were the same.

SEXUAL ATTITUDES In a self-image analysis of adolescents with spina bifida, researchers found these participants had a normal profile on body, self-image, emotion, social relationships, and coping.32 Their self-ratings on sexual attitudes, however, were well below average. Although these teens did not have depressed scores on self-worth or body image, their feelings and behavior toward the opposite sex, and perceived ability to be open to sexual relationships was low, and this effect was much more pronounced for females than for males.

SEXUAL MATURITY Studies that examined onset of sexual maturity have consistently found that early sexual maturity is common in children with spina bifida.70-72 Most studies report precocious puberty concomitant with shunted hydrocephalus with prevalence rates ranging from 4 to 18%.73, 74 Hochhaus and associates examined spina bifida patients for growth problems and found precocious puberty in 15% of those with shunted hydrocephalus 186 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics and 8% had spina bifida without hydrocephalus compared to 0.6% of the general population.71 For studies that measured early sexual maturation not early enough to be classified as precocious, 14% of males and females fell into this classification. The average age of onset of menarche for girls with shunted hydrocephalus and myelomeningocele was between 10.9 to 11.4 years compared to an average age of 12.8 years for nondisabled females in the US.75 Greene et al.70 found advanced bone age with early appearance of secondary sex characteristics, noting these indicators point to premature activation of the hypothalamic-pituitary-gonadal axis.

SEXUAL FUNCTION Recent studies have supported past findings of twice the number of females report being sexually active than males.76, 77 Sawyer and her associates found 81% of females and 88% of males in their sample reported some genital sensation was present. Although 88% of the women in this study had adequate vaginal lubrication, only 19% reported obtaining orgasm. De Vylder and colleagues reported females with spina bifida also may have erogenous zones in the nipples and periumbilical areas, sensation from which provided sexual pleasure and even orgasm.78 Cass et al. classified sexual function by level of lesion. In this group 33% of females who were sexually active with lesions L2 and above reported experiencing orgasm.79 Fifty-five percent of the women with mid-level lesions experienced orgasm, as did 60% of women with lesions S1 and below. As expected, Woodhouse found those with lesions at S1 or below usually have normal sexual function.80 This group found ambulators had normal sexual function, compared to only half of those who used wheelchairs fulltime. Sawyer found erection was achieved by 95% of males,76 although other studies have reported 25% of this population had erectile dysfunction.14 Ejaculation and orgasm reported in 67% of this sample, with Sawyer reporting that only 27% of patients with erectility were satisfied with penile rigidity. Woodhouse reported half the males with spina bifida who have posterior urethral valves experienced weak ejaculation and/or highly viscous and alkaline semen.80 The author also noted that 75% of males with ambiguous genitalia or micropenis have normal intercourse. Verhoef et al. found 79% of the adolescent males with spina bifida were able to obtain erections, with 61% reporting the ability to ejaculate.81 The Adolescent with Spina Bifida 187

Fifty-eight percent of males in the Sawyer study said they had experienced orgasm, with 88% able to achieve erection and 67% reported the ability to ejaculate.76 All of the sexually active females (37%) said they had experienced orgasm. In a similar study, 81% of the sexually active women described “a regular feeling of sexual excitement” during masturbation or other sexual activity, and 39% reported they frequently experienced orgasm.81 Joyner et al. speculated that myelomeningocele patients with hyperreflexia may experience “excruciatingly painful contractions” when orgasms occur simultaneously with an empty bladder.82 The authors further noted that orgasm with a full bladder may induce urinary incontinence. Despite partial or complete sexual functioning in a majority of this population, many list incontinence as the biggest barrier to seeking intimate relationships.76, 81 One study also found lack of self-confidence was an issue for more than half of the participants, but having a disability or being wheelchair dependent was an issue for less than one-third of this sample.81

FERTILITY, PREGNANCY, AND COMPLICATIONS Little is known about infertility rates in women with spina bifida; however, authors of a literature review stated females are thought to have normal fertility.78 The oft-repeated 1-in-25 risk of a parent with spina bifida having a child with a neural-tube defect reflects higher probability than published data. In a total of 259 pregnancies accounted for in 11 internationally published case studies, only 2 infants were born with spina bifida, reducing risk to approximately 1 in 130; however, the risk of having a child with a congenital defect is higher for this population.83 Nine infants in this group were born with other significant birth defects, which is a 1 in 29 probability. Recurrent urinary tract infections were common in case studies reporting prenatal complications, particularly for women with a history of UTIs. In a review of gynecologic issues in women with spina bifida, Jackson and Sipski noted additional complications included ureteric obstruction, ureteric stenosis, preeclampsia, and ectopic pregnancy.83 The authors posited secondary orthopedic conditions in this population (i.e., kyphosis, hip dislocation and/or subluxation, and pelvic obliquity) may alter the women’s reproductive anatomy, by displacement within the pelvis or by compression of reproductive organs. These problems often require special positioning on the exam table, and that gynecological surgical procedures may necessitate a modified approach. 188 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In a study that monitored pregnancy outcomes and shunt malfunctions in women with spina bifida, 70 women reported 138 pregnancies, 103 of which resulted in 105 live births (including two sets of twins).84 Sixteen women reported 32 incidences of miscarriage, including two ectopic pregnancies. Also in this study, five women delivered pre-term and four women underwent therapeutic abortions. Twelve infants had congenital defects, only one of which was myelodysplastic. Seven children were subsequently diagnosed with developmental disabilities, including attention-deficit/hyperactivity disorder, pervasive developmental delay, and autism. During pregnancy, four women underwent seven shunt revisions, and 15 women experienced intermittent intracranial pressure that did not require revision. No shunt malfunctions were reported for 100 of the 138 pregnancies. Arata and her colleagues found a sample of 17 women reported a total of 29 pregnancies, 23 of which resulted in live births. Eight infants were born preterm. Seven of the fullterm infants had growth retardation.85 None of the 23 had spina bifida, but three had congenital anomalies. One out of five wheelchair dependent women had a vaginal delivery, and 10 of the 18 pregnancies with mobile women also were vaginal deliveries. Recurrent urinary tract infections occurred in all women with histories of urinary infections, and stomal problems occurred, but were not considered serious. Preexisting pressure sores deteriorated further, and mobility was reduced for two ambulatory women. Only one woman did not recognize the onset of labor and delivered successfully at home. Women with spina bifida and Mitrofanoff urinary diversions have successfully delivered with either cesarean section86,87 or vaginal delivery with forceps.88 In all cases, as pregnancy progressed these woman had increasing difficulty catheterizing through their umbilical stoma and indwelling catheters were inserted.

SEXUAL EDUCATION Ignorance of the effects of myelomeningocele on sexual functioning clearly was apparent in a study published in 1999.76 Researchers quizzed adolescents with spina bifida about sexual and reproductive health knowledge and concluded that this population had an adequate level of sex education, but lacked information specific to spina bifida. Eighty- eight percent of the young people were not aware of increased risk of The Adolescent with Spina Bifida 189 latex allergy; thus, it can be assumed very few had specific knowledge that would lead them to choose vinyl condoms or diaphragms, or other latex precautions when choosing contraception methods. To emphasize the need for condition-specific information, the authors quoted a sample of questions by the respondents: “Can I catch sexually transmitted diseases even though I have spina bifida?” “Can I pass spina bifida on to my partners by having sex with them?” In this study, significantly more females than males answered sex education questions correctly. Another study found nearly all of the young people received sex education either through school, parents, or peers.81 Only 25% had received information on sexuality from their physician. Only 18% received information specific to spina bifida. Most of these respondents would liked for their physician to have initiated a discussion on the subject. One research project that examined the occurrence of sexual abuse in the spina bifida population found 37% of the females said they had experienced unwanted sexual attention, with 30% also having ‘something done’ to them sexually, two of whom said they were raped by strangers.76 One male also reported sexual abuse.

SUMMARY Medical advances over the past 40 years have dramatically increased the survival rate and quality of life of those with spina bifida. Multidisciplinary teams of specialists provide comprehensive care for patients with this complex condition. Adolescents with spina bifida have plans for employment and aspirations for marriage and family that are typical for youth of any ability. These teens are generally independent in daily living skills; however, most are not adequately participating in activities that prepare them for successful transition to adulthood (i.e., decision-making, household responsibilities, activities with peers). Most patients with spina bifida have intellectual functioning within the average range; however, hydrocephalus, repeated shunt infections, and other medical issues increase the risk of reduced cognitive function. Most have psychomotor deficits and many have specific learning disabilities whether or not their intellectual abilities are within the normal range. These students tend to perform significantly better on measures of verbal intelligence than nonverbal or performance indices, with verbal comprehension index scores as better predictors of academic performance 190 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics than full-scale scores. Youth with spina bifida may have some degree of memory impairment, and tend to struggle to learn math concepts. Several studies have found much higher rates of attention and other executive function problems, with up to almost a third having a diagnosis of attention-deficit/hyperactivity disorder. Serial neuropsychological evaluations are critical for patients with multiple shunt revisions, corpus callosum malformations, tethered cord, or seizures. Early sexual maturity occurs more often in children with spina bifida with the risk increasing for those with hydrocephalus. It appears that fertility is not affected in females, and although the risk of having a child with a neural-tube defect is higher, birth rates appear lower than previously expected. Rates have a congenital defect of any kind is higher for women with spina bifida. Approximately 25% of males have erectile dysfunction. Weak ejaculation and retrograde ejaculation are common. Although transition to adulthood begins in childhood, formal planning is mandated by law to begin no later than age 14 for students with individualized education plans. All teens with spina bifida, regardless of special education status, need to make overt vocational plans, particularly in view of low employment rates for the disabled population. Psychomotor deficits results in longer vehicle driver learning periods and more accidents for adolescents with spina bifida. Early research found adolescents with spina bifida were at risk for adjustment problems, depression, low-self esteem, and suicidal ideation. More recent studies have been contradictory with regard to these psychological problems, with emerging evidence that these patients have self-worth that is comparable to nondisabled peers. Although global self- worth is not significantly different for these teens, older adolescent girls with spina bifida had the lowest self-worth scores, and younger females with spina bifida placed more importance physical appearance than did males with spina bifida. Social acceptance for females with spina bifida of any age tended to also affect self-worth. Although it is not clear whether teens with spina bifida are more likely to have social skills deficits, social isolation remain a major concern for this group. These adolescents reported few peer contacts outside of school, and while inaccessible homes prevent many from visiting friends, teens with spina bifida should be encouraged and supported in meeting with their friends out in the community. The Adolescent with Spina Bifida 191

KEY LEARNING POINTS • Those born with spinal cord lesions have some degree of paralysis, neurogenic bladder, and Chairi II malformations. A majority of these children also have hydrocephalus, corpus callosum abnormalities, spine and hip malformations, and club foot. • A myriad possible secondary conditions may develop, i.e., seizures, cognitive deficits, attention problems, nonverbal learning disorders, strabismus, latex allergy, obesity, decubitus ulcers, obesity, scoliosis, or kyphosis; any combination of these makes treating adolescents with spina bifida a challenge in meeting their needs throughout their lifespan. • The developmental process of achieving independence in adulthood begins in adolescence, and for teens with spina bifida, the journey requires more planning, etiology of behavioral problems is multifactorial, including maladjustment at home and/or school and factors operating before pregnancy, during pregnancy, during delivery and in neonatal period. • Although global self-worth is not significantly different for these teens, older adolescent girls with spina bifida have the lowest self- worth scores, and younger females with spina bifida placed more importance physical appearance than did males with spina bifida.

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48. Andrews G, Crino R, Hunt C, Lampe L, Page A. General issues in anxiety disorders. In The Treatment of Anxiety Disorders. New York: Cambridge University Press 1994:1–17. 49. Lumenta CB, Skotarczak U. Long-term follow up of 233 patients with congenital hydrocephalus. Child’s Nerv Syst 1995;11:173–175. 50. Wills KE, Holmbed GN, Dillon K, McClone DG. Intelligence and achievement in children with myelomeningocele. J Pediatr Psychol 1990;15:161–176. 51. Hurley AD. Conducting Psychological Assessments. In Rowley-Kelly FL, Reigel DH (eds): Teaching the Student with Spina Bifida. Baltimore, MD: Paul H Brookes 1993:107–123. 52. Lollar DJ. Learning patterns among children with spina bifida. Z Kinderheilkd 1990;45:39 suppl 1. 53. Iddon JL, Morgan DJ, Loveday C, Sahakian BJ, Pickard JD. Neuropsychological profile of young adults with spina bifida with or without hydrocephalus. J Neurol Neurosurg Psychiatry 2004;75:1112-1118. 54. Barf H, Verhoef M, Jennekens-Schinkel A, Post M, Gooskens, R, et al. Cognitive status of young adults with spina bifida. Dev Med Child Neurol 2003;45:813-820. 55. Burmeister R, Hannay J, Copeland K, Fletcher J, Boudousquie A, et al. Attention problems and executive functions in children with spina bifida and hydrocephalus. Child Neuropsychol 2005;11:265-283. 56. Loss N, Yeates KO, Enrile BG. Attention in children with myelomeningocele. Child Neuropsychology 1998;4:7-20. 57. Fletcher J, Copeland K, Frederick J, et al. Spinal lesion level in spina bifida: A source of neural and cognitive heterogeneity. J Neurosurg: Pediatr 2005;102:268- 279. 58. Vachha B, Adams R. Memory and selective learning in children with spina bifida- myelomeningocele and shunted hydrocephalus: A preliminary study. Cerebrospinal Fluid Res, 2. 2005 15 November. Available from http:// www.cerebrospinalfluidresearch.com/content/2/1/10 Accessed 10 March, 2006. 59. Yeates KO, Enrile BG, Loss N, Blumenstein E, Delis DC. Verbal learning and memory in children with myelomeningocele. J Pediatr Psychol 1995;20:801-815. 60. Yeates, KO, Enrile BG. Implicit and explicit memory in children with congenital and acquired brain disorder. Neuropsychology 2005;19:618-628. 61. Barnes M, Dennis M, Hetherington R. Reading and writing skills in young adults with spina bifida and hydrocephalus. J Int Neurol Soc 2004;10:655-663. 62. Dennis M, Barnes M. Math and numeracy in young adults with spina bifida and hydrocephalus. Dev Neuropsychol 2002;21:141-155. 63. Franks CA, Palisano, RJ, Darbee JC. The effect of walking with an assistive device and using a wheelchair on school performance in students with myelomeningocele. Physical Therapy 1991;71:570–577. 64. Matson M, Mahone M, Zabel A. Serial neuropsychological assessment and evidence of shunt malfunction in spina bifida: A longitudinal case study. Child Neuropsychol 2005;11:315-332. 65. Baron J. Neuropsychological evaluations: Does your child need one? Insights into Spina Bifida 2004;3:4-5. 66. DesNoyers Hurley A. Conducting psychological assessments. In Rowley-Kelly, F Reigel, D (eds): Teaching the Student with Spina Bifida. Baltimore: Paul H. Brookes 1992:107-143. 67. Shultz, M. High joblessness plagues disabled. The Detroit News 20 December 2005;B1-B3. The Adolescent with Spina Bifida 195

68. Castree BJ, Walker JH. The young adult with spina bifida. Br Med J (Clin Res Edn) 1981;283:1040–1042. 69. Simms B. The car use of young drivers with spina bifida and hydrocephalus. Eur J Pediatr Surg 1991;1(Suppl):31–34 . 70. Greene S, Frank M, Zachmann M, Prader A. Growth and sexual development in children with meningomyelocele. Eur J Pediatr 1985;144:146-148. 71. Hochhaus F, Butenandt O, Schwarz HP, Ring-Mrozik E. Auxological and endocrinological evaluation of children with hydrocephalus and/or meningomyelocele. Eur J Pediatrics 1997;156;597-601. 72. Meyer S, Landau H. Precocious puberty in myelomeningocele patients. J Pediatr Orthop 1984;4:28-31. 73. Elias E, Sadehi-Nejad A. Precocious puberty in girls with myelodysplasia. Pediatrics 1994;23:521-522. 74. Trollmann R, Dorr H, Strehl E, Katalinic A, Beyer, R, Wenzel D. Growth and pubertal development in patients with meningomyelocele: A retrospective analysis. Acta Pediatr 1996;85:76-80. 75. Kakarla N, Bradshaw K. Disorders of pubertal development: Precocious puberty. Sem Reprod Med 2004;21:339-351. Available from: http://www.medscape.com/ viewarticle/468259 Accessed 17 March 2006. 76. Sawyer S, Roberts K. Sexual and reproductive health in young people with spina bifida. Dev Med Child Neurol 1999;41:671-675. 77. Shurtleff D, Sousa J. The adolescent with myelodysplasia: Development, achievement, sex an deterioration. Del Medl J 2003;49: 631-638. 78. De Vylder A, van Driel M, Staal A, Weijmar Schultz W, Nijman J. Myelomeningocele and female sexuality: An issue? Eur Urol 2004;46:421-427. 79. Cass A, Bloom B, Luxenberg M. Sexual function in adults with myelomeningocele. J Urol 1986;136:425-426. 80. Woodhouse CR. The sexual and reproductive consequences of congenital genitourinary anomalies. J Urol 1994;152:645-651. 81. Verhoef M, Barf H, Vroege J, et al. The ASPINE Study: Preliminary results on sex education, relationships and sexual functioning of Dutch adolescents with spina bifida. Eur J Pediatr Surg 2000;105:53-54. 82. Joyner BD, McLorie GA, Khoury AE. Sexuality and reproductive issues in children with myelomeningocele. Eur J Pediatr Surg 1998;8:29-34. 83. Jackson A, Sipski M. Reproductive issues for women with spina bifida. J Spinal Cord Med 2005;28:81-91. 84. Liakos AM, Bradley NK, Magram G, Muszynski C. Hydrocephalus and reproductive health of women: The medical implications of maternal shunt dependency in 70 women and 138 pregnancies. Neurol Res 2000;22:69-88. 85. Arata M, Grover S, Dunne K, Bryan D. Pregnancy outcome and complications in women with spina bifida. J Reprod Med 2000;45: 743-748. 86. Schumacher S, Fichtner J, Stein R, Wiedmemann A, Hohenfollner R. Pregnancy after Mainz pouch urinary diversion. J Urol 1997;158:1362-1364. 87. Natarajan V, Kapur D, Sharma S, Singh G. Pregnancy in patients with spina bifida and urinary diversion. Int Urogynecol Jliako 2002;13:383-385. 88. Roberts N, Rafferty P. Vaginal delivery in a patient with spina bifida and a Mitrofanoff urinary diversion. J Obstet Gynaecol 2004;24:576-586. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Post-Traumatic 16 Stress Disorder

GK Vankar, Khyati Mehtalia

INTRODUCTION Children and adolescents are exposed to traumatic events. In this general population sample, one-quarter experienced at least one high magnitude event by age 16, 6% within the past 3 months.1 The stressors include natural disasters like earthquakes, floods, tornadoes, fires or manmade disasters, assaults, vehicular accidents, rapes, physical and sexual abuse, medical disorders like burns or cancer, diagnostic procedures. The prerequisite for qualifying as traumatic stressor is that the person exposed should feel terrified that death or injury is imminent or there is physical or psychological vulnerability. The person’s response involves intense fear, helplessness, or horror. In children, this may be manifest in form of disorganized or agitated behavior.2 Though the traumatic stressor affects almost every person exposed to it, in some there are several characteristic symptoms that continue for significant period and lead to distress and disability. Magnitude of the problem and consequences of PTSD symptoms has led to increased efforts to diagnose the disorder accurately and to treat it effectively.

EPIDEMIOLOGY Most epidemiological studies have been of older young people and adults. Giaconia et al4 reported a lifetime prevalence of 6% in a community sample 3 of older young people. Kessler et al4 reported a lifetime prevalence of 10% using data collected from older young people and adults in the US National Comorbidity Post-Traumatic Stress Disorder 197

Survey. In contrast, the British National Survey of Mental Health of over 10,000 children and young people reported that 0.4% of children aged 11– 15 years were diagnosed with PTSD, with girls showing twice the rate of boys.5 Estimates of the incidence of PTSD are more frequently reported after various natural and other disasters. Rates vary enormously, partly as a result of different methodologies and partly as a result of different types of traumatic event. In various studies of the effects of road traffic accidents (not resulting in an overnight stay in hospital) rates of 25-30% are reported. The study of 200 young survivors of the sinking of the cruise ship Jupiter reported an incidence of PTSD of 51%. Most cases manifested within the first few weeks, with delayed onset being rare. Other disorders such as anxiety and depression were common as well.6 Where relatively standardized assessment methods have been used, the incidence of PTSD among child survivors of specific disasters ranges from 30 to 60%. As yet there are no epidemiological studies of the prevalence of PTSD among children in the general population. However, community studies in the United States have consistently indicated that around 40% of high school students have experienced some form of domestic or community violence, and between 3 and 6% have PTSD. 19 Despite this, in one study, half of the pediatricians in US considered that their knowledge and skills for diagnosis and management of PTSD were not satisfactory.7 Zeigler8 in their survey in US found Only 7% pediatricians believed that children were likely to develop symptoms of posttraumatic stress , only 18% reported giving any verbal guidance and only 3% provided any written instructions about posttraumatic stress to their patients and families.8 Ruchkin et al9 did not find any cross-cultural differences as regards manifestations of PTSD between US and Russian adolescents. PTSD has been described in the developing world also, its validity is established in India.10 Using standard screening instruments, several studies have found PTSD among Indian adolescents ranging from 20 to 22.5% within 6-8 months of earthquake and communal violence respectively.11, 12 One study found low prevalence of 4.7% PTSD, 4 years after exposure to communal violence in Gujarat.13 198 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Biological Factors PTSD is a biopsychosocial disorder. Family history of depression in first degree relatives increases risk for PTSD. This points towards genetic vulnerability. Psychobiological correlates include changes in hypothalamic–pituitary–adrenal axis functioning, increased excretion of adrenergic and dopaminergic metabolites, small cerebral volume and lower IQ as well as memory impairment. Neuroimaging findings suggest that after psychological trauma biological changes are not restricted to dysregulation of neurochemical systems but involve alterations in brain function and structure. The most replicated structural finding is hippocampal volume reduction, which may limit the proper evaluation and categorization of experience. Replicated localized functional changes include increased activation of the amygdala after symptom provocation (which may reflect its role in emotional memory) and decreased activity of Broca’s area at the same time (which may explain the difficulty patients have in labelling their experiences).14

Psychological Factors Extreme stressors cause intense negative emotions like terror, anger, sadness and hyperarousal caused by sympathetic nervous system activation. Neutral cues at the time of exposure can pair with trauma so much so that they themselves would later generate intense reaction. If a traumatic event occurred at a particular place, whenever the child visits the place later may feel terrified, scream for help and perspire profusely. If child repeatedly avoids trauma reminder cues to minimize anxiety, he will tend to further avoid such stimuli. Parental response to child’s reaction to trauma may have “modeling” effect (Box 16.1).

Box 16.1 A 5-year-old girl was brought by parents in immediate post-earthquake days. The child refused to go to her home on the fifth floor. When the psychiatrist asked ‘ who else is scared to go to the fifth floor?’ The father looked at the mother and responded: ‘she is!’

Social Factors Lack of social support, parental psychopathology are important predictors of PTSD after exposure to a traumatic event. Post-Traumatic Stress Disorder 199

Though important, family factors are not the sole etiology of PTSD. Nelson et al15 in a twin study of young adults demonstrated that twins who were sexually abused were at a higher risk of developing adverse outcomes than their non-abused twin despite shared heredity and family factors.

COMORBIDITY PTSD is associated with comorbid with other psychiatric disorders. Such comorbidity is rule rather than the exception. The psychiatric disorders- anxiety disorders, major depression, substance use disorder and attention deficit hyperactivity disorder occur at a higher rate in children and adolescents having PTSD. These comorbid conditions can have onset prior to, concomitantly or following onset of PTSD. Other anxiety disorders tend to precede onset of PTSD while major depression occurs following onset of PTSD.19

DIAGNOSIS DSMIV TR Criteria for PTSD 16 Diagnostic criteria for 309.81 Posttraumatic Stress Disorder (Cautionary Statement) A. The person has been exposed to a traumatic event in which both of the following were present: 1. The person experienced, witnessed, or was confronted with an event or events that involved actual or threatened death or serious injury, or a threat to the physical integrity of self or others. 2. The person’s response involved intense fear, helplessness, or horror. Note: In children, this may be expressed instead by disorganized or agitated behavior. B. The traumatic event is persistently reexperienced in one (or more) of the following ways: 1. Recurrent and intrusive distressing recollections of the event, including images, thoughts, or perceptions. Note: In young children, repetitive play may occur in which themes or aspects of the trauma are expressed. 2. Recurrent distressing dreams of the event. Note: In children, there may be frightening dreams without recognizable content. 200 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

3. Acting or feeling as if the traumatic event were recurring (includes a sense of reliving the experience, illusions, hallucinations, and dissociative flashback episodes, including those that occur on awakening or when intoxicated). Note: In young children, trauma- specific reenactment may occur. 4. Intense psychological distress at exposure to internal or external cues that symbolize or resemble an aspect of the traumatic event. 5. Physiological reactivity on exposure to internal or external cues that symbolize or resemble an aspect of the traumatic event. C. Persistent avoidance of stimuli associated with the trauma and numbing of general responsiveness (not present before the trauma), as indicated by three (or more) of the following: 1. Efforts to avoid thoughts, feelings, or conversations associated with the trauma 2. Efforts to avoid activities, places, or people that arouse recollections of the trauma 3. Inability to recall an important aspect of the trauma 4. Markedly diminished interest or participation in significant activities 5. Feeling of detachment or estrangement from others 6. Restricted range of affect (e.g., unable to have loving feelings) 7. Sense of a foreshortened future (e.g., does not expect to have a career, marriage, children, or a normal life span) D. Persistent symptoms of increased arousal (not present before the trauma), as indicated by two (or more) of the following: 1. Difficulty falling or staying asleep 2. Irritability or outbursts of anger 3. Difficulty concentrating 4. Hypervigilance 5. Exaggerated startle response E. Duration of the disturbance (symptoms in criteria B, C, and D) is more than 1 month. F. The disturbance causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. Specify if: Acute: If duration of symptoms is less than 3 months. Chronic: If duration of symptoms is 3 months or more. Post-Traumatic Stress Disorder 201

Specify if: With Delayed Onset: if onset of symptoms is at least 6 months after the stressor *Reprinted with permission from the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision. Copyright 2000 American Psychiatric Association.

Clinical Features Re-experiencing the Trauma Children may re-experience the trauma in a variety of ways. Flashbacks and memories: In children, intrusive memories are more common than flashbacks. Flashbacks are vivid experiences that include visual and auditory elements from the trauma; the child may feel like the trauma is happening all over again and he or she may react with intense fear. Flashbacks may be more common among children who have depression in addition to PTSD. These may be intrusive and interfere with function at home or school. Behavioral re-enacting: Children may act out aggressively toward others or do and say things that they witnessed. Children are often unaware that this behavior is connected to their abuse.

Re-enacting through play: The child may represent the traumatic experience through repetitive play (Box 16.2).

Box 16.2 After earthquake, a child repeatedly played making houses from sand and breaking them.’ This is my house, this is your house, and there is no house. After communal violence, children played ‘firing’ games, one team becoming majority community and the other, minority.

Symptoms of avoidance of memories or situations that remind the child of the traumatic event: The child may exhibit a general restriction in daily activities (e.g., avoiding activities that could prompt excitement or fear) or may present with specific fears. They may lose previously acquired skills and show regressive behavior like thumb sucking or bed wetting. 202 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Avoidance Children or adolescents with PTSD avoid thinking or talking about topics that could remind them of traumatic experiences. Some, especially young children, may refuse outright to acknowledge that the abuse occurred. Triggers: Children may react to and attempt to avoid stimuli that trigger memories of the abuse. Some common triggers include phrases, songs, scenes on television, a smell, or a person’s appearance. Anniversaries, dates, and certain places may also trigger memories. Sleep disturbance: Children may experience nightmares, fear of the dark, and fear of sleeping alone. Physical contact: Children with PTSD may have difficulty managing physical contact because of a heightened sense of vulnerability or because it may be a reminder of abuse. Emotional numbing: To manage difficult reactions to the abuse, children with PTSD may have to suppress memories and almost all emotional reactions. These children may seem emotionally numb. Normal human interactions appear not to resonate with them; they laugh less and show less human connection and empathy. Sense of foreshortened future: PTSD is associated with a sense of pessimism about the future, with affected people occasionally feeling that there is no future for them. In children, this may manifest as the belief that they will never become adults or a lack of interest in planning for the future. Dissociation: Dissociative episodes are periods of disconnection from the external environment. A dissociating child may appear to be absent and unresponsive for a few minutes. Events that remind the child of danger or threat may trigger these episodes. Children who experience dissociation soon after the disclosure of abuse are at significantly increased risk for developing PTSD. Some believe that this is because dissociation inhibits the appropriate level of experiencing and expressing their emotions concerning the abuse.

Symptoms of Increased Arousal and Hypervigilance The child may appear on edge, noticing small changes in the environment and closely tracking the behaviors of others. They may exhibit an increased startle response. Post-Traumatic Stress Disorder 203

Cognitive function: A small study of neuropsychologic function in children with PTSD found deficits in sustained attention, problem solving, and abstract reasoning. Sleep problems: The child may have much difficulty falling asleep. Many fears are experienced at night, such as imagining faces on the wall or eyes looking at the child. Many sleep disruptions, frequent nightmares, and awakenings at night can occur. Nightmares are common in children with PTSD. They may directly relate to the trauma or, more commonly, consist of frightening dreams with more generalized themes. Behavioral inhibition: Some children with PTSD are inhibited and overly pleasing and attentive to their caregivers. This may be the case, particularly if the child has reason to fear that angering or disappointing the caregiver can trigger a negative encounter. Delays in development and learning: In younger children, traumatic events, particularly long-standing trauma or high-stress living conditions, are more likely to delay the development of the child in several important domains, such as reciprocity, relatedness, cognitive abilities, and adaptive behavior in general. Traumatized children may appear almost autistic and may display great difficulties with learning. Physical: No specific physical signs of PTSD exist. The pediatrician may suspect the condition in the child who is excessively frightened of being touched or approached by the doctor. When this circumstance arises, inquire about the child’s history of traumatic experiences. In the case of physical or sexual abuse, the physician may detect the associated physical signs. Studies have found that only a small minority of sexually abused children have physical evidence of abuse.

Causes: Not every person who is exposed to trauma develops PTSD. Development of PTSD is unpredictable following a traumatic event. Onset of the syndrome may be initiated through either direct or witnessed exposure to a single or chronic trauma. Some differentiate trauma exposures into 2 types, as follows: 17 Type I trauma includes full, detailed memories, “omens,” and misperceptions. Type II trauma includes denial and numbing, self- hypnosis and dissociation, and rage. Crossover conditions often occur after sudden, shocking deaths or accidents that leave children handicapped. In 204 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics these instances, characteristics of both type I and type II childhood traumas exist side by side.

Subthreshold PTSD: It is important to note that children who do not meet full criteria for PTSD (i.e. three symptom clusters) may not differ significantly from children meeting all three cluster criteria (re- experiencing, avoidance, hyperarousal) with regard to functional impairment and distress. In one study, children with subthreshold PTSD had functional impairment that was specific to PTSD symptoms and was not due to comorbidity. This suggests that it might be more precise to make a diagnosis of PTSD in children and adolescents based on the intensity of symptoms and their relationship to functional impairment, than on the threshold number of symptoms.18

Developmental Considerations in PTSD Diagnosis Preschool children do not have cognitive and verbal skills to describe their experiences. Eight of the eighteen PTSD criteria require a verbal description of internal states and experiences. The clinician must therefore attempt to infer from behavioral observations whether the child’s thoughts and feelings are consonant with PTSD symptoms. Direct reports from parents, teachers and other observers in the child’s milieu are an important component of the evaluation, although it should be borne in mind that parents often minimize the child’s PTSD symptomatology. Preschool children often display the loss of recently acquired developmental skills (regression), the onset of new fears or the reactivation of old ones, accidents and reckless behavior, separation anxiety (often manifested in anxious clinging), and psychosomatic complaints such as stomach aches and headaches.20

COURSE AND PROGNOSIS Many studies show that immediately after a traumatic event large proportion of children and adolescents may have PTSD symptoms. These symptoms tend to persist for 1-2 years following, which they reduce. Some studies conclude that presence of severe PTSD symptoms many years after exposure to trauma. DeBellis et al21 have documented several psychobiological abnormalities like decreased intracranial volume, decreased corpus callosum volume area, low IQ which are highly Post-Traumatic Stress Disorder 205 correlated with duration of PTSD. Childhood sexual abuse is highly predictive of adult depression and suicidality. Hence there is need for early diagnosis and treatment of PTSD.21 The follow-up study of young people who survived the sinking of the Jupiter found that 15% still met criteria for PTSD 5–7 years after the event.6 More recently, a 33-year follow-up of the children who survived the Aberfan landslide disaster found that 29% of those traced and interviewed still met criteria for PTSD.22 In other words, in the absence of effective therapy, the long-term effects of life-threatening, traumatic events in childhood can be severe. Box 16.3 gives types of PTSD.

Box 16.3: Types of PTSD Acute PTSD: Duration 1 month but less than 1 month Chronic PTSD: Duration more than 3 months Delayed onset PTSD: Onset of PTSD 6 months after traumatic event Complex PTSD: Repeated, multiple and/or early childhood trauma. Severe affective modulation problems, attachment and relationship problems, aggression, substance use, dissociation features and marked impairment.23

PHARMACOTHERAPY Pharmacological agents for childhood PTSD have received little empirical investigation. Effective pharmacological agents are ideally those which: • Treat the symptoms which are distressing and disabling • Improve the quality of life of the child/adolescent, allow normal growth and development in the long-term, and • Facilitate the process of psychotherapy by enabling traumatized children to deal with emotionally distressing material and work through their distress19 Despite the lack of well-designed, randomized, controlled trials that support efficacy, drugs can be used in a rational and safe manner. Reduction in even one disabling symptom, such as insomnia or hyperarousal, may have a positive effect on a child’s overall functioning.23 • SSRIs, are a good first choice because they have broad spectrum therapeutic effects. They are effective for the core symptoms of PTSD as well as common comorbid conditions like the anxiety disorders and depression. They also improve social and occupational functioning and an individual’s perception of improved quality of life.19 Treatment response should be closely monitored and if there are continuing 206 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

symptoms that do not respond to, additional medication to improve outcome should be considered. For example, in one study though sertraline, significantly improved the core PTSD symptoms in one study comorbid anxiety and depressive symptoms did not respond well.19 A reasonable approach is to begin with a broad-spectrum agent, such as an SSRI, which should target anxiety, mood, and re-experiencing symptoms. Besides this, drug treatment considerations are important (Box 16.4). Box 16.4: Drug treatment of PTSD • Adrenergic agents, such as clonidine (0.05 to 0.1 mg/day), used either alone or in combination with an SSRI: of hyperarousal and impulsivity. • Add mood stabilizer may be necessary in severe affective dyscontrol. • Add an atypical antipsychotic drug in cases of severe self-injurious behavior, dissociation, psychosis, or aggression. • Comorbid conditions such as ADHD : psychostimulants or newer agents such as atomoxetine.

In a more naturalistic study, the use of an opiate medication (morphine) as a possible preventive agent in children with burns-related PTSD was investigated. The dose of morphine administered to children during hospitalization for burn injuries was associated with a significant reduction in PTSD symptoms over a 6-month period.

PSYCHOTHERAPY Trauma Focused Cognitive Behavioral Therapy Evidence is growing that trauma-focused cognitive-behavioral therapy (TF-CBT) is an effective treatment for sexually abused children, including those who have experienced multiple other traumatic events (Box 16.5). Box 16.5: Core components of TF-CBT • Psychoeducation about traumatic reactions and PTSD; • Stress reduction (by relaxation training, thought stopping (telling unpleasant thoughts silently ‘stop’, and cognitive coping techniques). • Gradual exposure (relief from symptoms occurs by entering into feared situations rather than avoiding them.) hence the person recalls and tells the thoughts, feelings and sensations in detail that he experienced while he actually experienced the trauma as well as while telling the experience. • Cognitive processing (relationship between thoughts, feelings and behaviors is explained, wrong and unpleasant thoughts are identified and replaced by more realistic thought). • Parental treatment component: Besides the above steps, parent is explained how to best help the child and in joint sessions with child, improve communication regarding trauma. Post-Traumatic Stress Disorder 207

This treatment combines trauma-sensitive interventions and well- established CBT principles.25,26 Most of the TF-CBT treatment studies have consisted of 12 treatment sessions. It has been given in individual, group and school settings. It is more effective than nondirective play therapy, standard community care or no care. Hence it is considered first line treatment for Children with PTSD or significant trauma related symptoms. The strongest mediator of treatment response other than type of treatment was parental emotional distress. At one-year follow-up, the strongest predictor of positive response was parental support of the child.28 Play therapy: This treatment is used for preschool children who are not able to express themselves verbally. It is assumed that with play child’s inner conflicts are expressed and the distress is relived. Usually the play is nondirective, the therapist does not tell the child what and how to play. Most important benefit of play therapy is to improve communication about traumatic experience.19 In one intervention study it was found inferior to trauma focused CBT.

Eye Movement Desensitization and Reprocessing (EMDR) Shapiro (1989) After identifying a traumatic target memory, the therapist would have the client articulate a self-referent “negative cognition” associated with the memory (e.g., “I am shameful”) and a “positive cognition” (e.g., “I am honorable”) to replace the negative one. The therapist would then move her fingers back and forth in front of the client’s eyes, instructing the client to track her fingers visually while concentrating on the distressing memory. After each set of 10-12 eye movements, the therapist would ask the client to provide ratings of distress and strength of belief in the positive cognition. The therapist would repeat this procedure until distress subsided and belief in the positive cognition increased.27 Adult studies have found equal effectiveness even if the eye movement is not included in the procedure showing that eye movements are not the effective component of EMDR.28 Psychological debriefing: This consists of sessions in which child is encouraged to talk about the traumatic event and express feelings related to the experience, psychological support and psycho-education regarding common reaction to traumatic stress is given and how to deal with them. 208 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In child adolescent populations its effectiveness is not studied, some adult studies have found worse outcome compared to no treatment.29 KEY LEARNING POINTS • PTSD is an important psychiatric sequel for children and adolescents exposed to traumatic event • Besides exposure to traumatic event, re-experiencing traumatic event, avoidance of traumatic reminders and hyperarousal are three symptom clusters needed for PTSD diagnosis • Major depression and other anxiety disorders are common comorbid conditions • For children and adolescents exposed to traumatic events, pediatrician should specifically look for the manifestations of PTSD: children and adolescents as well as parents should be interviewed • Trauma focused cognitive behavior therapy and SSRIs are first line treatments for PTSD

REFERENCES 1. Galea S, Ahern J, Resnick H, et al. Psychological sequelae of the September 11th attacks in New York City. N Engl J Med 2002;346:982-987. 2. Fairbrother G, Stuber J, Galea S, Fleischman AR, Pfefferbaum B. Post-Traumatic Stress Reaction in New York City Children After September 11th Terrorist Attacks. Final Report to William T. Grant Foundation. New York, NY: New York Academy of Medicine 2003. 3. Giaconia RM, Reinherz HZ, Silverman AB, Pakiz B, Frost AK, Cohen E. Traumas and post-traumatic stress disorder in a community population of older adolescents. J Am Acad Child Adolesc Psychiatry 1995;34:1369-1380. 4. Kessler RC, Sonnega A, Bromet E. Posttraumatic stress disorder in the national comorbidity survey. Archiv Gen Psychiatry 1995;52,1048-1060. 5. Meltzer H, Gatward R, Goodman R. Mental Health of Children and Adolescents in Great Britain. London: Stationery Office, 2000. 6. Yule W, Bolton D, Udwin O, Boyle S, O’Ryan D, Nurrish J. The long-term psychological effects of a disaster experienced in adolescence. I: The incidence and course of PTSD. J Child Psychol Psychiatry 2000; 41,503-511. 7. Laraque D, Boscarino JA, Battista A, et al. Reactions and needs of tristate-area pediatricians after the events of September 11th: implications for children’s mental health services. Pediatrics 2004;113:1357-1366. 8. Ziegler MF, Greenwald MH, DeGuzman MA, Simon HK. Post-traumatic stress responses in children: Awareness and practice among a sample of pediatric emergency care Providers. Pediatrics 2005;115:1261-1267. 9. Ruchkin V, Schwab-Stone M, Jones S, Cicchetti DV, Koposov R, Vermeiren R. Is post-traumatic stress in youth a culture-bound phenomenon? A comparison of symptom trends in selected US and Russian communities. Am J Psychiatry 2005;162:538-544. 10. Mehta Khyati, Vankar GK, Patel V. Validity of the construct of PTSD in a low income country-interview study of women in Gujarat, India. Br J Psychiatry 2005;187:585-586. Post-Traumatic Stress Disorder 209

11. Mehta K, Vankar GK, Panchal B, et al. PTSD among earthquake affected adolescents in Kutch. (Unpublished work). 12. Mehta Khyati , Vankar GK. PTSD among communal violence affected adolescent girls in Ahmedabad (Unpublished work). 13. Vankar GK, Banwari G, Parikh V, Shah H. PTSD and major depression among children and adolescents who lost a parent in communal violence (Unpublished work). 14. Alaister H. Neuroimaging findings in post-traumatic stress: Disorder-systematic review. Br J Psychiatry 2002;181:102-110. 15. Nelson EC, Heath AC, Madden PA, et al. Association between self-reported childhood sexual abuse and adverse psychosocial outcomes: results from a twin study. Arch Gen Psychiatry 2002;59:139-145. 16. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorder, 4th edn. Washington, DC: American Psychiatric Association 2000. 17. Terr L. Childhood traumas: An outline and overview. Am J Psychiatry 1991;148: 1-20. 18. Carrion VG, Weems CF, Ray R, Reiss AL. Toward an empirical definition of pediatric PTSD: The phenomenology of PTSD symptoms in youth. J Am Acad Child Adolesc Psychiatry 2002;41:166-173. 19. Cohen JA. PTSD in Children and Adolescents. In: Sadock BJ, Sadock VA (eds): Comprehensive Textbook of Psychiatry, 8th edn. Philadelphia: Lippincott Williams and Wilkins 2005. 20. Scheeringa MS, Wright MJ, Hunt JP, Zeanah CH. Factors affecting the diagnosis and prediction of PTSD symptomatology in children and adolescents. Am J Psychiatry 2006;163:644-651. 21. DeBellis MD, Keshavan MS, Clark DB, et al. Developmental traumatology. Part II: Brain development. Biol Psychiatry 1999:45:1271. 22. Morgan L, Scourfield J, Williams D, Jasper A, Lewis G. The Aberfan disaster: 33-year follow-up of survivors. Br J Psychiatry 2003;182,532-536. 23. Donnelly CL. Pharmacologic treatment approaches for children and adolescents with post-traumatic stress disorder. Child Adolesc Psychiatr Clin North Am 2003;12:251-269. 24. van der Kolk BA. The Assessment and Treatment of Complex PTSD. In: Yehuda R (ed): Treating Trauma Survivors with PTSD. Washington, DC: American Psychiatric Press 2002. 25. Cohen JA, Deblinger E, Mannarino AP, Steer RA. A multisite, randomized controlled trial for children with sexual abuse-related PTSD symptoms. J Am Acad Child Adolesc Psychiatry 2004;43:393-402. 26. Cohen JA, Mannarino AP. Factors that mediate treatment outcome of sexually abused preschool children: six- and 12-month follow-up. J Am Acad Child Adolesc Psychiatry 1998;37:44-51. 27. Shapiro F. Efficacy of the eye movement desensitization procedure in the treatment of traumatic memories. J Traumatic Stress 1989;2:199-223. 28. McNelly RJ. Research on EMDR as a treatment for PTSD. PTSD Res Quart 1999;10: 1-8. 29. Kennedy J. The current status of psychological debriefing. BMJ 2000;321:1032- 1033

GENERAL REFERENCE National Institute for Clinical Excellence. Guideline N. 26 PTSD: The Management of PTSD in Adults and Children in Primary and Secondary Care. London: Gaskell 2005. RAP Special Volume 19: Developmental and Behavioral Pediatrics 17 Dyslexia

Suraj Gupte, Utpal Kant Singh, WE Wilson

INTRODUCTION The term, dyslexia, denotes reading (sometimes even writing and spelling difficulties, both written and oral) in a child whose vision and general intelligence are otherwise normal.1-3 The original Greek word from which it is derived means “difficulty with words” i.e. communication.1 It usually coexists with other skills of learning. Intelligence is not affected.3-9 Yet, child performance remains much behind his potentials. Dyslexia is noticed in school children, usually by the third grade. They are able to see and recognize letters but find it difficult to spell and write words. There is no difficulty in recognizing the meaning of objects and pictures. Such celebrities as Edison, Einstein and Leonardo da Vinci are said to have suffered from dyslexia.1 Understandably, their achievements may have been yet greater had they not been suffering from this neurodevelopmental disorder.

ETIOLOGY Exact cause of dyslexia remains elusive. Nevertheless, there is a consensus that it results from failure to break words into sounds and assemble word sounds from written language. There are three areas in the brain that are responsible for dyslexia and other learning disabilities: 1. Wernicke’s area: It is responsible for analyzing sounds in written and spoken words. It is underactive in dyslexia. This too is underactive in dyslexia. Dyslexia 211

2. Angular gyrus: It links visuals with stored sounds of languages. 3. Broca’s area: It coordinates the movements of lips, jaws and tongue to produce speech. It helps to convert letters to sounds. In dyslexia, it is overactive. In a large majority of the cases (85%), dyslexia is inherited and familial. Chromosomes 1, 2, 6 and 15 have been implicated for the loci.

EPIDEMIOLOGY Dyslexia is a persistent chronic condition rather than a transient neurodevelopmental lag though it is responsive to focused early and intensive interventions. Prevalence is 5-10% in school and clinic-based samples and 17.5% in unselected population-based samples. Both sexes suffer equally.

CLINICAL MANIFESTATIONS The major manifestations are • Difficulties in acquiring and using language: Reading and writing letters in the wrong order. There is directional confusions as also confusion regarding capital and small letters. • Difficulties in learning to speak • Difficulties in organizing written and spoken language • Difficulties in learning letters and their sounds • Difficulties in memorizing number facts • Difficulties in learning foreign languages • Difficulties in correctly doing math operations. Though attention-deficit/hyperactivity disorder (ADHD) is altogether a distinct and separate condition, dyslexia may coexist in ADHD.

DIAGNOSIS3-6 Dyslexia is more or less a clinical diagnosis, based on thoughtful analysis of all available clinical data, including psychometric assessment. The aim of clinical evaluation is to determine • If there are unexpected difficulties in reading • If there are accompanying linguistic problems at the level of phonologic processing. 212 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

History Earliest sign is difficult to understand by reading and to express by writing. By the age of 7 years, the child has developed acute difficulty reading, writing or spelling. Since he fails to perform in keeping with his potentials, he loses confidence and self-esteem. School becomes a burden for him and he loses the joy of studying. Poor school performance provokes him to play truant. TV-addiction results as a comfortable escape from academics. Reading and writing exhausts the child. Writing is remarkably slow and clumsy with poor handwriting and bad spacing. Spellings, grammar and syntax are bad. Spelling mistakes are because of “orthographic memory problem” which means that spelling mistakes are because of visual memory problem. Interestingly, despite being a poor speller, the dyslexic may well be a very good artist, scientist, navigator or technocrat in which different type of memory is required.

Physical Examination It has the following major components: • A physical examination with special reference to neurological examination and tests of vision and hearing. • Psychological examination including tests to measure intelligence (IQ), tests of academic achievements and tests of other special abilities. • Reading assessment to find out reading word by word, omissions, reversals, insertions and guessing at words, • Spelling assessment to find out if he child can spell words that involve roots, prefixes and rules for combining. • Assessment of arithmetic skills Informal assessment that includes observation, oral interviews and error analysis is important. • Handwriting, comprehension and attention.

MANAGEMENT3,6-9 Appropriate treatment needs a multidisciplinary team comprising a clinical/educational psychologist, pediatrician, psychiatrist, special educator, social worker and speech therapist. If dyslexics are identified and provided suitable help at the initial stage, their potentialities could be exploited fully. Dyslexia 213

Action in Early Years The emphasis should be on “remediation” of the reading problem.

Action in Secondary School The emphasis should be on providing “accommodation” rather than remediation that was practiced early. Each component of the reading process is taken in turn and effective intervention program provide systematic instruction in phonemic awareness, phonics, fluency, vocabulary and comprehension strategies. Ample opportunities are provided for writing, reading and discussing literature.

Action in High School and Beyond Here the treatment is accommodation rather than remediation. Accommodation strategy include: • Providing extra time in reading and writing assignments as well as examinations • Use of laptop computers with spelling checkers • Use of taperecorders in the classroom • Use of recorded books • Access to lecture notes • Tutorial services • Alternatives to MCQs • Separate quiet room for taking tests.

KEY LEARNING POINTS • Though school failure can result from several causes, dyslexia remains on top in its etiology. • A multidisciplinary approach is called for in a child with dyslexia if diagnosis is to be reached early enough. • Management of dyslexia warrants a life-span perspective. • When recognized early in life, the focus should be on “remediation” of the reading problem. • When the child enters secondary school, the focus should shift to “accommodation” with systematic instruction in phonemic awareness, phonics, fluency, vocabulary and comprehensive strategies and opportunities for writing, reading and discussing literature. 214 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• When in high school and beyond is typically based on accommodation rather than remediation. • With application of appropriate curriculum adjustment, it is possible to help the child attain his optimal educational potential and become a productive and contributing adolescent and adult.

REFERENCES 1. Gupte S. Learning disability. In Gupte S (ed): Recent Advances in Pediatrics Vol 16. New Delhi: Jaypee Brothers 2006. 2. Lyon G. Towards a definition of dyslexia. Ann Dyslexia 1995;45:3-27. 3. Lyon GR, Shaywitz SE, Shaywitz BA. Special reading disability (Dyslexia). In: Behrman RE, Kliegmam RM, Jenson HB (eds): Nelson Textbook of Pediatrics, 17th edn. Philadelphia: Saunders 2004:110-112. 4. Shaywitz S, Shaywitz B. Dyslexia. In: Swaiman KF (ed) Pediatric Neurol: Principles and Practice, 3rd edn. 1999:576-584. 5. Bibace R, Walsh M. Development of children’s concept of illness. Pediatrics 1980;66: 912-917. 6. Cpin D. Developmental learning disorders: Clues to their diagnosis and management. Pediatr Rev 1996; 17: 284-290. 7. Kulkarni M, Kalantre S, Upadhye S, Karande S, Ahuja S. Approach to learning disability. Indian J Pediatr 2001;68:539-546. 8. Kulkarni M, Chari G, Shanbag P. Learning disability. In: Gupte S (ed) Recent Advances in Pediatrics, Vol 5. New Delhi: Jaypee Brothers 1995:314 -332. 9. Pratt H, Donell D, Orfuse M. Disorders of cognition, attention and learning. Indian J Pediatr 1999;66:404-414. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Mathematical Disabilities: 18 Developmental Aspects

E Levine, Suraj Gupte, Utpal Kant Singh

Over the decades, performance in mathematics has definitely come to stay as an index of child’s intellectual competence.1-3 Understandably, therefore, mathematics disabilities result not only in underachievement but also have a profound fallout on child’s feelings, self-esteem and academic motivation.3,4 Parents, teachers and even peers may hurt his ego by humiliating him, devastating the child who woefully feels inadequate in mathematics and develops a feeling of sadness. He begins to regard the subject as downright hostile and a perpetual nuisance.5,6 According to a conservative estimate, some 5 to 10% children suffer from one or the other mathematics disability.

MANIFESTATIONS Mathematical disability may be in relation to facts, details, procedures, manipulations, patterns, words, sentences, images, logical processes, estimations, concepts, approaches, accumulations, applications, apprehensions and affinities.

Facts Storage in memory as also prompt retrieval as and when required (the so- called “automatized recall”) of the mathematical facts, e.g. multiplication tables, simple addition and subtraction, a range of numerical equivalents, etc. form the sheet-anchor of mathematical learning. Any problem in storage and/or retrieval. Is bound to result in underachievement in problem-solving exercises. 216 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Details Attention to such minute and fine details as aligning numbers in suitable columns, operational signs (plus or minus), exact location of decimal points, etc. is important. Attention deficit, impulsivity, erratic placing and lack of self-monitoring place the child at considerable disadvantageous position in the class.

Procedures It is important for the student to recall mathematical algorithms, in other words the specific procedures—which include the processes involved in multiplication, division, reducing fractions, regrouping, converting from decimals to percentages and solving simple equations. Procedure recall depends on understanding the underlying logic, sequential memory and the capacity to recall and apply the steps in an operation in the correct serial order. Normally, with practice the procedure becomes automatic in due course.

Manipulations The ability to manipulate and maintain task subcomponents is termed “active working memory”. It is central to mathematical proficiency. Deficits in active working memory cause problems in mathematical learning exercises.

Patterns Ability to recognize a range patterns (key words or phrases that keep emerging from word problems. Providing clues about the procedure needed to solve the problem) is an important subcomponent of mathematical learning.

Words Limitations in acquisition of mathematical vocabulary (application of denominator, subtrahend, divisor, isosceles) become a bottleneck in mastering mathematics.

Sentences Interpretations and drawing inferences from sentences in word problems has a great bearing on mathematical learning. The child with language disabilities face problems when a teacher explains a procedure or a concept. Mathematical Disabilities: Developmental Aspects 217

Images Normally, a student should be in a position to effectively visualize, store, and recall visual imagery. This means that he should be able to perceive subtle differences in shape, size, and proportion of geometrical forms. He should also be able to associate language with visual imagery. In the presence of difficulty with visual memory, he may encounter serious problems.

Logical Processes Propositional reasoning (ability to appreciate and manipulate the “if…. thens” and the “either… ors”) and proportional reasoning (ability to solve problems by comparing ratios and make changes in ratios) in particular are important in mathematics. Whereas propositional reasoning develops in elementary school children, proportional reasoning is by and large a domain of the middle childhood and adolescence. It plays some role in chemistry, physics and other sciences. Lack of either reasoning causes discrepancy between abilities to perform computations and solve word problems requiring reasoning. As a result these children begin overdepending on their memory in mathematics.

Estimations The student must be in a position to form a rough impression about the likely answer in the given problem as a consequence of reasoning process. Those who are wanting in this ability are likely to cut a sorry figure in mathematics as they fail to estimate if their computational results indeed make sense.

Concepts Mastery of a concept in mathematics, including the ability to delineate its constituent elements, is quite necessary for grasping the subject. Just a working grasp of the concepts can at best contribute to attaining only fragmentary knowledge of mathematics and is not good enough. A student belonging to this category is likely to fail to conceptualize that percentages, decimals and fractions have much in common. 218 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Approaches The approach to the solution of a problem (the so-called “problem-solving skill”) should be systematic as well as strategic. Its components include • Identification of the question being asked. • Studying the problem and determining the relevant as well as the irrelevant information in the problem. • Estimating the answer. • Devising possible strategies for the problem. • Tentative selection of the best strategy. • Trying out that best strategy. • Employing alternative strategy if the first one fails to work. • Monitoring the process throughout.

Accumulations Cumulative memory is an essential subcomponent of mathematical ability. The student must build up cumulative knowledge over a span of time. When early grasp of the subject matter is insufficient, the student is likely to pay a heavy toll subsequently.

Applications Relating formal learning to everyday life constitutes the cornerstone of mathematics learning. The student who fails to discover the utility of mathematics in realistic ways is likely to regard mathematics learning as “stupid”.

Apprehensions Mathematics disability leads to mathematics anxiety or phobia. The latter further leads to mathematics disability. Thus a vicious cycle, that is difficult to break, is set up.

Affinities The student with true affinity for mathematics, usually as a result of strong role models, is likely to develop a good grasp of the mathematics concepts. In contrast, the student with little or no affinity towards mathematics which he regards as a cocktail of minimally related, isolated operations and concepts. Mathematical Disabilities: Developmental Aspects 219

MANAGEMENT OF THE CHILD WITH MATHEMATICS DEFICIENCIES Evaluation The neurodevelopmental, neuropsychological and educational testing is of paramount importance in a child suffering from mathematics underachievement. The aim is to determine the underlying dysfunction (attention deficits, visual-spatia weaknesses, language disabilities, memory problems, poor sequential organization, higher cognitive inadequacies) that may be contributing to mathematics deficiencies. Attempts should be made to identify the mathematics subcomponent(s) that are contributing to the child’s underachievement.

Intervention Strategy1-5 Remedial action for mathematics disability needs to be initiated early in the child’s career, long before he begins to be bogged down by over- whelming pressure and his underachievement. Else, since mathematics knowledge is essentially cumulative matter, he may not be able to catch up. The following remediation guidelines may be of special value: Underdeveloped subcomponents Once the subcomponent responsible for the underachievement is identified (vide supra), wholesale efforts should be directed at mastering this particular area. For instance, a student with delayed automatization of math facts needs to be given practice drills, recalling facts under timed conditions. All efforts should be made to exploit child’s developmental strengths and subjects area affinities, making use of diagrams and other graphic materials. A very productive strategy, well tried by me as well as others, is to learn math by trying to teach it. Now, we have available educational software that facility learning at the level of the deficient components. Bypass techniques In bypass technique, a deficient mathematical task subcomponent may well be circumvented. This technique requires a teacher to evaluate instructional priorities and be flexible and sensitive to the student’s struggles. Handling neurodevelopmental Dysfunction Management of the underlying neurodevelopmental dysfunction (ADHD, language disabilities, cognitive problems) assists enhancing performance in mathematics. A child with attention deficit, for example, is likely to improve his performance following use of stimulant medication such as dexedrine as also other behavioral and cognitive management techniques. 220 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Changing Attitudes of the Family and the Teachers1,3,5 In the first place, parents must appreciate that mathematics disability is none of child’s fault. It is a developmental problem that can be overcome employing remedial measures. They must never humiliate the child for his deficiency. • They should be supporting and nonjudgemental, offering considerable praise and positive reinforcement while offering help to him in homework. • The affected child should be given mathematics privacy and not embarrassed by asking questions that he is unlikely to reply correctly. Equally importantly, he should not be exposed to the wrath of his peers. This means that efforts should be made to avoid having other students correct or even see the papers of peers with poor mathematics performance. • Grade retention should be avoided as it is unlikely to help the child. • The affected child should sustain a degree of optimism, aiming at reasonable level of proficiency as an attainable goal in the field.

KEY LEARNING POINTS 1. Mathematical, generally considered an index of child’s intellectual competence, results not only in underachievement but also have a profound fallout on child’s feelings, self-esteem and academic motivation. 2. Mathematical disability may be in relation to facts, details, procedures, manipulations, patterns, words, sentences, images, logical processes, estimations, concepts, approaches, accumulations, applications, apprehensions and affinities. 3. Management includes: • Evaluation to identify the components and subcomponents affected • Bypass (accommodation) in which the deficient component is circumvented • Changing attitudes of parents who must appreciate that mathematics disability is none of child’s fault; it is a developmental problem that can be overcome employing remedial measures Mathematical Disabilities: Developmental Aspects 221

REFERENCES 1. Levine MD, Lindsay RL, Reed MS. The wrath of math. Pediatr Clin North Am 1992;39:525-536. 2. Brainerd CJ. Young children’s mental arithmetic errors: A working memory analysis. Child Dev 1983;54:812-817. 3. Levine E, Whitehead EW, Gupte S. Dyscalculia. Eur Bull Dev Pediatr 2006;13:178- 186. 4. Shalves RS, Gross-Tur V. Developmental dyscalculia. Pediatr Neurol 2001;34:337- 342. 5. Levine MD. Developmental Variations and Learning Disorders, 2nd edn. Cambridge: Educators Publishing Service 1999. 6. Whitmore K, Hart H, Willems G (eds). Neurodevelopmental Approach to Specific Learning Disorders. Cambridge: MacKeith Press 1999. RAP Special Volume 19: Developmental and Behavioral Pediatrics

19 Behavioral Problems: Overview

Utpal Kant Singh, AE Laurence, Suraj Gupte

INTRODUCTION The term, behavior problem, denotes a disorder that represents significant departure or deviation from the so-called normal behavior. These disorders are relatively stable, internalized and difficult to treat than the adjustment reactions but less so than neurosis and pychosis.1-4 The hyperkinetic reactions, runaway reactions and group delinquency are examples of behavioral problems. The root cause of the problem can usually be traced out to the home and/or school environment. The term, conduct disorder, denotes to a behavioral problem that becomes persistent source of difficulty at home, at school or in the community at large. Since a situation aberration (usually in the family or at school) is a common denominator of the background of adjustment reactions, behavior problems and special symptoms, it is customary to label as these as “behavioral disorders.” In pediatric practice, almost one-quarter infants and children suffer from one or the other behavioral problem. Table 19.1 lists the common behavioral disorders according to the nature of deviation in behavior and Table 19.2 according to the age of occurrence of the disorder.

ETIOPATHOGENESIS Etiology of behavioral problems is multifactorial, including maladjustment at home and/or school and factors operating before pregnancy, during pregnancy, during delivery and in neonatal period.2-4 A faulty emotional Behavioral Problems: Overview 223 environment (constituted by parental attitudes, siblings, neighborhood, school and mass media, including television (the so-called “idiotbox”) radio and periodicals) is, however, most important. Of the environmental factors, faulty parental attitudes (rejection, overprotection, dominance, unrealistic expectations, overcriticism, discrimination, unfavorable comparisons, overdiscipline or underdiscipline, dominance by the parents, marital disharmony, etc.) are the single most important cause of behavioral difficulties. Overprotection means more than excessive protection of the child against danger with the result that the child is not allowed to take care of himself and grow up like his peers who are given balanced protection. It may happen in the only child, the only son in particular, a child born after many years of waiting, the first born, especially in a joint family, a very good-looking child, a mentally retarded child or a physically handicapped child, in case of loss of a spouse or failure on the part of the spouse to play his or her role well, or a child who is supposed to have brought fortune to the parents. Rejection, on the contrary, usually is accompanied by failure to attend to the normal needs of the child. Unfavorable comparisons, admission to an institution when the circumstances do not really demand it, unnecessary scolding, excessive punishment or failure to please the child consistently with a reward as and when appropriate. There is invariably accompanying favoritism to the other child. Rejection may happen in an unwanted child, a stepchild, a mentally or physically handicapped child, a child who is supposed to have brought bad luck to the family, or a child who is chronically mischievous and is a constant source of troubles for the family. Not infrequently, parental attitudes may well be a pot purrie of overprotection and rejection at different times, depending on such diverse factors as parents’ mood, financial position, job satisfaction and interparental relationship. Lack of consistency on the part of parents consensus towards the child, leading to conflict, anxiety and insecurity.

CLASSIFICATION Some behavioral problems may well belong to more than one category. Enuresis, for instance, is regarded as an undesirable “habit disorder” resulting from poor training by a developmental psychologist, a “personality disorder” by a psychologist or psychiatrist.” 224 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 19.1: Categorization of common behavioral problems

Habit problems • Thumb-sucking, nailbiting, bruxism, tics, enuresis, encopresis, breath-holding spells, trichotillomania, aerophagia Eating Problems • Pica, food fads, food refusal/overeating, vomiting, aerophagia, anorexia Personality Problems • Shyness, timidity, fears, anger, jealousy Antisocial Problems • Juvenile delinquency, e.g. stealing, lying, destructiveness, cruelty, gang activities. Sleep Problems • Night terrors/mares, somnambulism, insomnia, sleep-talking, hypersomnia, narcolepsy, cataplexy Speech Problems • Stuttering, mutism, phonation and articulation disorders Scholastic Problems • Reading, writing or mathematical disability, repeated failures, absenteeism, truancy, school phobia, aggressiveness Sexual Problems • Masturbation, homosexuality, incest, sodomy, precocious sexuality, hypersexuality

Table 19.2: Categorization of behavioral problems in relation to age group

Infancy • Feeding problems (rumination), colic, stranger anxiety, breath-holding spells, temper tantrum Preschool Age • Head banging, body rocking, thumb-sucking, nailbiting Midchildhood • Stuttering,, pica, sleep problems, enuresis, encopresis, tics Adolescence • Juvenile delinquency

COMMONLY ENCOUNTERED BEHAVIORAL PROBLEMS

PICA The Latin term, pica, meaning magpie (a bird that eats anything and everything), refers to compulsive eating of substances other than food (nonedible, non-nutritive items), e.g. earth, dust, dirt, clay, chalk, sand, paint flakes, plaster from wall, fabrics, hair, etc. (Table 19.3)4-8 • It is an eating disorder, encountered usually in first 5 years of life. • Familial occurrence is common, particularly in the mother. Behavioral Problems: Overview 225

Table 19.3: Nonfood articles employed in pica

Common Frequent Infrequent

Earth Tooth paste Sand Clay Paper Soap Dust/dirt Paint chips Coffee ground Chalk Hair Cornstarch, laundry starch, baking soda Plaster Wool Cigarette ashes Threads Cigarette buts String Glue Ice Rust

Types 1. Pica as a physiological phenomenon During oral phase, children until 18 to 24 months often try to eat non-food items. It is not necessarily considered abnormal at this age. 2. Pica as a behavioral problem This usually occurs in children aged 2-5 years. 3. Pica associated with mental retardation Pica is most common in people with developmental disabilities, including autism and mental retardation

Etiology As a manifestation of inclination for mouthing or tasting, pica until 2 years of age may be taken as normal, provided that there are no associated problems such as mental retardation or behavioral problems. Else, it needs to be considered a deviant behavioral problem. Association of pica with mental retardation, including autism and such brain-behavior disorders such as Kleine-Levin syndrome, is altogether a different entity.7, 8 Else, pica usually is seen in children from lower strata of society with suggestion of parental neglect, poor supervision or adequate attention. Accompanying behavioral problems are common. Perverted appetite may well be a manifestation of psychologic cause which should be searched. Associated malnutrition with worm infestation and vitamin and mineral deficiencies (usually iron and calcium) is common. Whether these are the cause or effect of pica is not clear. Predisposing factors in pica may be shortlisted as: • Poor socioeconomic status • Poor parenting 226 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Emotional deprivation • Iron-deficiency anemia (IDA) • Mental retardation.

Etiologic Hypotheses Nutritional Hypothesis The nutritional hypothesis suggests that nutritional deficiency, such as iron deficiency, trigger specific cravings. Some evidence supports the hypothesis that at least some pica is a response to dietary deficiency. Nutritional deficiencies are often associated with pica and correction of that deficiency improves symptoms. Many children, for example, stop eating non-food items after they are treated for iron deficiency anemia, common condition among children with pica. However, not everyone responds when a nutritional deficiency is corrected, indicating that may be a consequence of the behavior (rather than the cause). Furthermore, there are also children with pica who don’t have a documented nutritional deficiency. Geophagia, eating earth substances such as clay or dirt, is a form of pica that can cause iron deficiency.

Cultural Hypothesis Another hypothesis to explain pica is that in some cultures, eating clay or dirt may help relieve nausea (and therefore, morning sickness), increase salivation, remove toxins, and alter odor or taste perception; some people actually claim to enjoy the taste and texture of dirt or clay. Some people eat clay or dirt as part of a daily habit. Pica is a cultural feature of certain religious rituals, folk medicine, and magical beliefs. Some people in various cultures believe that eating dirt helps them incorporate magical spirits into their bodies.9

Psychologic Hypothesis Some psychological theories regard pica as a behavioral response to stress—an indication that the individual has an oral fixation (is comforted by having things in his or her mouth). Despite the wide variety of hypothesize, not one of them explains all forms of pica. The attending pediatrician must treat every case on its merit Behavioral Problems: Overview 227 and attempt to find out what may be causing or contributing to the disorder.

Clinical Features Infants and children with pica are invariably anemic secondary to iron deficiency. Mineral and vitamin deficiencies are common. • Intestinal parasitosis is by and large always present • Occasionally, pseudomotor cerebri may develop • Predisposition to chronic lead poisoning is a real risk • Associated behavioral problems are frequent. In trichotillomania, the child pulls his hair and swallows them. A large collection of hair in the stomach may cause a palpable lump in the epigastrium, especially after main meal. This is called trichobezoar.7-9

Risks Table 2 lists the major risks of pica.

Diagnosis Psychologic and environmental assessment are important. The latter should include not only child’s home but also additional locations where he spends time, say grandmother’s house, nursery school, etc. Laboratory tests should include screening for: • Nutritional anemia, usually IDA. • Worm infestations. • Lead intoxication: Blood lead level on blood sample from finger puncture or, preferably, venous blood.

X-Ray Studies • Large radio-opaque flakes of paint seen on abdominal radiologic examination strongly suggest pica. • Radiography of long bones for lead lines.

Management In view of the common association between pica and intestinal parasitosis as also mineral (especially iron, calcium and zinc) and vitamin deficiencies, these accompaniments should be treated without loss of time. 228 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

In case of chronic lead toxicity, appropriate corrective therapy is needed. Subjects suffering from pica in the presence of psychosomatic problem(s) qualify for evaluation for underlying psychiatric illness such as depression and separation anxiety and, if warranted, psychotherapy. Additional therapeutic interventions include: • Physical and persuasive restraint. • Aversive and non-aversive behavioral therapy. • Enrichment of environment. • Time out.

BREATH-HOLDING SPELLS This common situational disorder, also termed “infantile syncope”, accounts for 4-13% of psychosomatic disorders in pediatric age group. It occurs to some degree in up to 27% of otherwise normal children. In a vast majority, onset is before 18 months of age. Two types are recognized: Pallid and cyanotic. Cyanotic type is thrice as frequent as the pallid type. In 20% of cases, both types coexist.

Etiology The time-honored belief is that BHSs result from frustration. A disciplinary conflict between parents and the child is the basic underlying cause, the child uses the attack or its threat to assert himself and to express his anger or protest. According to a recent explanation, genetically determined dysregulation of autonomic nervous system reflexes is responsible for BHSs. Different autonomic dysregulatory mechanisms are responsible for the two types of BHSs. The pallid type is supposed to be secondary to cardiac asystole, similar to a vasovagal attack. It can be induced by ocular compression. The cyanotic type results from a rise in the intrathoracic pressure when breath is held in expiration (as, for example, during crying), leading to decrease in cerebral circulation. The mechanism involves an interplay among hyperventilation, Valsalva maneuver, expiratory apnea and intrinsic pulmonary mechanisms. Cerebral anoxia from an autonomic Behavioral Problems: Overview 229 dysfunction is responsible for loss of consciousness. Though the beginning of the attack is voluntary, subsequent loss of consciousness is involuntary. Role of iron-deficiency anemia (IDA) too is speculative. The association between BHSs and IDA is well-known. Anemia seemingly adversely affects the compensatory functions of autonomic nervous system and contributes to cerebral anoxia in severe BHSs. Treatment of IDA promptly and fully stops the spells in many of them.

Clinical Features In a classical attack of BHSs (cyanotic type), the child cries, hyperventilates and holds his breath (usually in expiration) followed by cyanosis in a few seconds. There may occur momentary loss of consciousness and convulsive twitchings. Finally, he becomes limp. In the pallid type, the child develops characteristic pallor rather than cyanosis. The onset in both types is between 6 and 18 months of age. The frequency is usually one to three attacks a day.

Diagnosis Clinical picture is usually so characteristic that little difficulty should be encountered in recognizing the condition. When spells are accompanied by tonic and clonic convulsions, differentiation from epilepsy becomes essential. In the former, an obvious precipitating factor can invariably be elicited. Secondly, cyanosis in spells precedes convulsions whereas in epilepsy it follows these. Thirdly, EEG in spells is invariably normal. BHS should also be differentiated from cyanotic attacks (the so-called “hypercyanotic spells”) seen in congenital heart disease.

Treatment It is directed at determination of the causative and precipitating factors and treating these by psychotherapy. Drug therapy is of insignificant value. Attention must be directed to coexisting IDA, if any, and prompt treatment offered for its correction. Very frequently occurring pallid BHS may respond to atropine sulfate, 0.01 mg (O) thrice daily. 230 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Response to piracetam, an expensive drug, too is quite gratifying.

Prognosis As the child grows, frequency of BHSs decreases. Finally, almost all such children are symptom-free by the age of 5 or 6 years. Incidence of temper tantrum and other behavioral disorders in these children is high. There is no evidence that epilepsy occurs in greater proportion in them than in normal population.

THUMBSUCKING AND NAILBITING Over 20% children are expected to exhibit this conduct disorder sooner or later. It should be considered a behavioral problem only when it shows chronicity. Thumbsucking though fairly normal in early infancy in which oral phase is important, it may well be a behavioral problem in older children. It is child’s way of securing extra self-nurturance. Ignoring the symptom by the parents often resolves the problem. Its persistence beyond the age of 3 years may adversely affect the teeth (causing malocclusion) in a proportion of children. Nailbiting is a phenomenon demonstrated by children beyond 4 years of age. It may continue upto adolescence and even beyond right into adulthood. The cause is a kind of insecurity, a conflict or hostility. The child seemingly draws a sense of pleasure from such self-stimulation. Treatment consists in reassurance to the parents and guidance that they need not be “fussy” over these benign problems.

TEETH GRINDING (BRUXISM) Teeth grinding, especially during sleep, is a common conduct problem. In case of infants, parents need not bother about it. In case of older children, it may be a manifestation of disturbed sleep, disturbing dreams, pentup tension and aggression. Apart from this, bruxism may occur in mental retardation and in unconscious patients, more so those suffering from meningitis or encephalitis. Treatment consists in improving the environmental situation responsible for tension and conflict. Attempts should be made to make bedtime more relaxed and enjoyable. Behavioral Problems: Overview 231

TICS These are repetitive movements of muscle groups (eye blinking, tongue thrusting, lip smacking, grimacing, throat clearing) representing discharge of tension originating in emotional and physical states. These are absent during sleep and not accompanied by unconsciousness. Unlike involuntary movements, these can be controlled by voluntary effort. Tics usually occur in school-going children and usually represent emotional disturbance or maladjustment. Generally, they may act as an outlet for suppressed anger and wearisomeness following forcible control of anger by parents or the teacher. Critical comments by elders often worsen the situation rather than stop their occurrence. I know of a child who would kept spitting every now and then. The overindulgent mother, not realizing these were, in a way, parents’ creation, kept criticizing and rebuking him for the “bad habit”. Did it make a difference ? Yes, of course it did! But, mind you, it simply aggravated his tics. The child only recovered from it once he was away from parents to a boarding school. Such examples can be multiplied. A peculiar tic disorder, Gilles de la Tourette syndrome, is characterized by tics in the form of varied bodily movements accompanied by vocalization in the form of as compulsive barking, grunting or shouting obscene words. Though the intensity of manifestation tends to diminish with advancing age, it usually persists throughout life. Haloperidol, 1-5 mg (O) as such or together with an antiparkinsonian agent is indicated in this disorder. Tice, when severe, need psychiatric evaluation. Treatment is mainly in the form of dopamine antagonists such as haloperidol. Other effective agents include clonidine, carbamazepine and clonazepam.

RHYTHMIC HEADBANGING/ROCKING A neglected child (hungry for human touch or interaction) may indulge in rhythmic head banging or rocking to obtain sensory solace. The condition is encountered more often in mentally retarded children or children with emotional or maternal deprivation. Practically always, in my experience, they also exhibit failure to thrive. 232 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

TEMPER TANTRUMS This condition is characterized by bouts of tantrums in the form of shouting, screaming, crying, kicking the furniture, breaking windowpanes, pounding the floor or wall with fists, etc. When he is very angry and offended. It is a sort of outlet for his anger and efforts aimed at exercising autonomy and assertiveness, occurring during the period of negativism. The underlying factors include inconsistency, unreasonable expectations, excessive strictness, overprotectiveness, overindulgence and marital disharmony on the part of parents. It is an attention-seeking device, occurring most often in children who are active, determined and assertive. Associated behavioral problems include thumbsucking, headbanging, sleep disturbances, enuresis and hyperkinesis. Persistence and worsening tantrums are an indication for excluding postnatal brain injury, organic brain disease and seizure disorders. Management should revolve around finding their cause and correcting it. The child should be taught to express his negative feelings in an acceptable way rather than tantrums. Undue attention, both positive and negative, needs to be discouraged. During the course of tantrums, it should be ensured that he does not hurt himself.

STUTTERING (Stammering) Preschoolers, usually between 3-5 years of age, may start stammering. The cause in a large majority of the cases is the neurotic attitude of the mother, leading to a conflict in child’s personality. No treatment is generally needed. If stuttering persists, breath-control exercises and miniaturized metronome that is of value in pacing the rhythm of speech need to be restored to under care of a speech therapist.

ENURESIS The term, enuresis, denotes normal urinary bladder emptying at a wrong place and time at least twice a month after the age of 5 years. Nocturnal enuresis refers to bed-wetting. It is a fairly common pediatric problem, occurring in about one-fourth of children, and is a potential cause of embarrassment to the child as well as the parents. A proportion of the children suffering from this disorder may wet their garments during Behavioral Problems: Overview 233 waking hours as well. Boys suffer more often than girls. Remarkable familial pattern is observed.

Clinical Features Two clinical types are recognized: primary (persistent) and secondary. In the primary (persistent) enuresis, the child has never been dry at night. It is usually the result of erratic bladder training either by parents who are overanxious for prompt control, or those who are not reasonably close to the child’s needs, or chronic psychological stress not related to bladder training. Secondary (regressive) enuresis is characterized by initial control of bladder that later gets disrupted by stressful environmental events like marital conflict, death, arrival of a sibling, or shifting to a new house. It is usually intermittent and transitory. Four types are recognized based on daytime symptoms: • Type 1: Monosymptomatic nocturnal enuresis • Type II: Diurnal enuresis without daytime frequency • Type III: Nocturnal enuresis with daytime frequency • Type IV: Nocturnal diuresis with daytime frequency and voiding dysfunction

Etiology The causes are: i. Psychologic Enuresis may be a manifestation of family conflict and maladjustment, e.g. too strict parents, rejection, sibling rivalry, etc. An erratic handling of the problem by the parents causes further anxiety to the child. His condition, therefore, gets more aggravated. ii. Too late, too early or improper training by the parents regarding the bladder control is also an important factor in the causation of bed- wetting. iii. Physical factors like threadworm infestation, genitourinary infections, anatomic defects, etc. may be responsible for enuresis in some cases. In both types (primary and secondary), an organic pathology is present in less than 5% cases. Dysuria, frequency, straining, dribbling, gait disturbances and poor bowel control suggest an underlying organic cause. 234 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Diagnosis This should include a detailed interview with the parents as well as the child to find the etiologic or, at least, associated emotional factors, together with a complete physical examination. Intestinal parasitosis, especially threadworm, genitourinary infection and anatomic defects should be excluded. Urine analysis and urine culture should be performed at the initial visit to exclude UTI. An X-ray of lumbosacral spine, ultrasonography, voiding cystourethrogram and urodynamic studies are often required.

Treatment A prompt treatment is essential or the child may continue to have enuresis plus added emotional problems in adolescence. Treatment is, as a rule, not required before 6 years of age. If the underlying disease is detected it should be treated. In others, treatment consists of: 1. Psychotherapy and training (behavior modification) in the form of: a. Reassurance to the parents and the child. Parents should be told to encourage the child in having dry nights. In fact, they should offer special pat and even reward on occasions when the child does not wet the bed. b. Restriction of too much of water and drinks at bed time and insisting on his voiding before retiring. c. Waking him up once or twice to void during night. d. Rewarding the child for the dry nights (which should be charted on a calendar) assists in enlisting the cooperation of the child. e. Discouragement of punishment or humiliation of the child by the parents. f. Ridicule by siblings and friends should not be allowed. Parents need to spend at least half an hour of quality time with the child. 2. Bladder-strengthening exercises. This include emptying the bladder before sleeping, drinking large quantity of water during daytime and holding urine as long as possible and practise repeatedly starting and stopping the stream in the flush. 3. Using an electric alarm (buzzer) device which is designed in such a way that the child wakes up as soon as he is about to wet the bed. The Behavioral Problems: Overview 235

device is based on the condition reflex response. It consists of a sensor fixed to child’s underwear and an alarm placed at bedside. 4. Drugs Imipramine hydrochloride, 0.9 - 1.5 mg/kg/day (O) at bedtime for 3-6 months, gives good results. It acts by altering the arousal-sleep mechanism. The success rate considerably improves, if it is supplemented with a small dose of diazepam. Anticholinergic agent, oxybutinin, 10-20 mg/day (O), for 3-6 months, is useful for daytime enuresis with urgency and urge incontinence. It acts by reducing the uninhibited bladder contractions. Desmopressin (DDVP), which is a synthetic antidiuretic agent, is a relatively expensive modality for enuresis. The basis for use of this drug is that immediate triggering factor for enuresis is the overdistended bladder resulting from reduced secretion of the ADH rather than the abnormal bladder capacity or function. It is given in full dose (10-40 mcg/day) as spray until child is dry for 28 successive nights. Thereafter, dose is tapered over 3 weeks period. At times, a combination of modalities (say electric alarm device plus drug therapy) works best. The pediatrician must also develop a positive relationship with the child to allay feeling of guilt, resentment or shame. He should motivate the child for “independent control”.

Encopresis Compared to enuresis, encopresis constitutes a more serious emotional disturbance. It is characterized by passage of feces into inappropriate places at any age (usually after 5 years) when bowel control is expected to be accomplished.

In primary encopresis, chronic soiling persists from infancy onward. In secondary encopresis (regressive), soiling occurs after attaining bowel control at appropriate age. Accompanying symptoms include chronic constipation, fecal impaction, overflow incontinence and poor school attendance as well as performance. The cause is unconscious anger and defiance on the part of the child. Treatment is similar to that of enuresis as far as supportive measures are concerned. Primary encopresis is more difficult to treat than secondary form. 236 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

School Phobia (School Refusal; School Withdrawal)10 School phobia refers to absolute refusal by the child to go to school. Generally two types are recognized, namely: • Anxiety-related: It usually occurs in reasonably good students who become fearful of separation from parents, have school phobia with a specific fear and feel insecure. Girls suffer more frequently than boys. • Secondary Gains Type: It usually occurs in “inadequate” students, usually boys who prefer comforts at home. The major underlying factor is anxiety about separation from parents. Most often the mother. The parents are responsible for giving the child an impression that they won’t indeed mind his staying at home. There usually is a far-too-close tie between the child and the parents. The mother, in particular, is overindulgent, overprotective and domineering type and the father is ineffective and disinterested. Such school factors as bullying or teasing, unreasonable punishment or dislike of the teacher may also contribute to the problem. Treatment consists in weaning the child and the parents from each other with the help of a child psychiatrist and family physician. The cooperation of the school teacher(s) should be obtained as and when warranted.

Substance Abuse Four types of substance abuse by, especially by adolescents, are: 1. Experimental: Weekend beer or marijuana use at parties 2. Recreational: Weekday use and progression to harder drugs, liquor, etc. 3. Problematic: Daily use; personality changes noted; difficulties at school and with family 4. Addictive: Majority of time under the influence of drugs or alcohol; frequent legal problems. An oft-asked question is: When should a drug abuser be referred for professional evaluation and counselling ? Well, the answer is: • Recreational, problematic or addictive substance abuser, regardless of age • In case of a younger adolescent, evaluation at experimental stage may assist in postponing such behavior. Most of the addicts demonstrate significant evidence of conflict, confusion, mental tension and remarkable deterioration in academic performance. Shoplifting, stealing and, at times even trafficking are Behavioral Problems: Overview 237 resorted to by the diehard addicts to make enough money for procuring their supply of the drugs.

The causes of drug abuse include frustration at home: • A poor performance at school • Keenness to burn midnight oil at the time of examination • To gear up stamina for better performance in sports and athletic • Jut for kicks, jut for the heck of it • As an adventure • To hit back at parents, teachers or society in protest The suggested preventive measures include: • Provision of adequate facilities for recreation and entertainment, especially in the hostels. • Proper canalization of energies of the adolescents into constructive activities. • Inoculation of the dangers of drug abuse among students, their teachers and family members. • Provision of periodical psychiatric guidance facilities in schools. • Strict implementation of drug control measures.

Child Abuse and Neglect Maltreated and abused children are likely to suffer from such emotional problems as fearfulness, low self-esteem, delinquency, substance abuse, hyperactivity, aggression, denial, lack of trust, projection, hypervigilance, etc. In the long run, they may end up as delinquent, violent and antisocial adults and potential child abusers.

Anorexia Nervosa In a pursuit for “slimness” and “weight loss”, many adolescent girls impose foolish dietetic restrictions on themselves. This is termed “anorexia nervosa of restrictive type. Some adolescent girls may eat in binges and then get rid of the food intake by self-inducing vomiting or using cathartics. This is called “bulimia”. In both cases, they are likely to become grossly malnourished with disturbances related to almost all organs, i.e. electrolyte imbalance, postural hypotension, cardiac arrhythmias, CCF, hypothermia, amenorrhea, constipation, dry skin with lanugo hair, peripheral edema, rise in BUN, bone marrow hypoplasia, etc. Interestingly, the subjects are notably resistant to infection. 238 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

An important observation is that the disorders occurs more often in sisters and mothers of anorexics than in general population. It must not be confused with anorexia accompanying psyhopathologic disorders such as schizophrenia, depression, hysteria and hypochondriasis. Though it is generally believed to be a eating disorder, its exact etiology remains elusive. It is noteworthy that the subjects have such characteristics as developmental immaturity, isolation and excessive dependence. The family background is overprotective. Management revolves around psychotherapy (including pharmacotherapy with antidepressant agents), behavior modification and nutritional rehabilitation. Results following adoption of an integrated multimodal strategies are gratifying.

Sibling Rivalry The term refers to aggressive or regressive behavior or passive mischievousness on the part of the elder child on the arrival of another sibling. The contributory factors include a gap of less than 2 years between the two children and favoritism on the part of the mother. Both these factors result in jealousy which initiates the behavioral problems. Aggressive behavior may manifest as an assault on the sibling or inanimate objects. Regressive behavior may be in the form of temper tantrums or secondary enuresis. Management consists in helping the child to cope with feelings of jealousy. The parents must ensure the child that arrival of the younger sib in no way undermines his importance. A careful mental preparedness of the child before the birth of the younger baby helps to safeguard from rivalry. He may be involved in preparation for the new arrival. After 2-3 months, sibling rivalry usually resolves. However, it stands good chances to reappear.

Suicidal Tendencies in Adolescents Suicidal tendency in adolescents is far more frequent than is generally held. That for every death by suicide, there are some 50-200 attempts that fail. Signals of such tendency are: Behavioral Problems: Overview 239

• Depression as manifested by sadness, anorexia, sleep disturbances, fatigue, etc. • Psychosomatic complaints • Juvenile delinquency, truancy, sexual promiscuity, substance abuse, running away from home and other “acting out” behaviors • Previous suicidal attempt in the preceding 2 years • History of a suicide in the family.

Adverse Effects of Heavy Television Viewing Excessive TV viewing by children is becoming quite a problem in India and other developing countries too. Its probable adverse effects include: • Increased aggressive behavior and acceptance of violence • Difficulty in distinguishing between fantasy and reality • Distorted perception of reality • Trivialization of sex and sexuality • Increased passivity and disengagement • Negative effects on cognitive learning • Loss of time and potential to inform and to teach prosocial behavior.

Periodic Syndrome The disorder refers to periodic occurrence of certain symptoms such as colicky abdominal pain (periumbilical), nausea and vomiting, headache (often of migrainous variety), diarrhea or constipation, marked pallor or flushing, fever and prostration. These manifestations may be present in various combinations. A characteristic feature of the syndrome is that recurrences occur at different periodicity of weeks or months. In between the attacks, the child is all right. The subjects are usually emotional, highly strung, obsessional and perfectionists. The expectations of their parents are far too lofty. A quarrel in the family or school examination often precipitates the attack. There is, as a rule, no evidence of infection. In a small proportion, there may be evidence of epilepsy. Treatment consists in providing assistance with emotional stress in the family or at school. Regular psychotherapy may be warranted in “hardcore” cases. 240 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

HYSTERIA (CONVERSION REACTION/ HYSTERIA) In hysteria, now recognized as a dissociative (conversion) disorder, the child (usually a preadolescent or an adolescent) with a psychopathic personality present with manifestations simulating an organic disease, say recurrent abdominal pain, sensation of compression of the throat (globus hystericus), blindness, gait disturbances, paralysis, sensory loss, urinary retention, seizures or dyspnea. A good history-taking and clinical workup usually lead to the correct diagnosis without resorting to painstaking investigations. The patient has a tendency to be indifferent to the queries and relates manifestation in a detached manner.

Common Presentations Hysterical seizures, a common presentation, are remarkable by absence of tongue-biting, apnea and incontinence. The patient tends to forcibly hold the eyes closed and seizure activity is bizarre. Quite often, seizures are marked by rhythmic thrusting and writing of trunk. Nocturnal seizures, stereotyped aura, cyanotic skin changes and postictal confusion are infrequent in pseudoseizures seen in hysteria. Moreover, serum prolactin level remain normal after the pseudoseizures. EEG too shows no spike and wave forms or postictal slowing. Hysterical blindness is characterized by tunnel vision and absence of pupillary abnormality and fundoscopic abnormality. Hysterical ataxia is characterized by inability to stand or walk without any deficit on neurologic examination when tested in lying position. The gait is bizarre and there is extreme lurching on the sides. In cerebellar ataxia, on the other hand, the patient walks on a wide base and has difficulty in maintaining balance. Hysterical paralysis is characterized by presence of normal muscle tone, tendon reflexes and plantars, and positive Hoover test. The last-named consists in keeping hand under the allegedly paralysed leg and asking the patient to raise the normal leg against resistance. As the patient forcefully lifts the leg, the examiner’s hand can feel downward pressure of the affected leg against the examiner’s hand. This occurs only in hysteria. Hyperventilation syndrome is a state characterized by dyspnea, tightness or stabbing pain in chest, headache, abdominal pain, muscle pains, paresthesia, palpitations, dryness of mouth, vertigo, choking, weakness, Behavioral Problems: Overview 241 blurred vision, confusion and syncope. The syndrome occurs in episodes. The causes include acute anxiety state, uremia, salicylate poisoning, hypernatremic dehydration, diabetic ketoacidosis, and Rye syndrome. Treatment is primarily of the cause.

Management Treatment of hysteria is primarily early detection and symptom removal. The symptom removal (normalization) can be attained by insisting on adherence to routine and contracting differential reinforcing and removal of secondary reinforces. The child should never be accused of feigning the symptoms. Appropriate antidepressants and anxiolytics may be warranted.

Parental Divorce and Child Behavior Response of children to parental divorce varies with the age group (Table 19.4).

Table 19.4: Response of different age groups to parental divorce

Preschool age (30 months to 5 years) • Regression in developmental milestones such as toilet training • Increased separation anxiety • Sleep disturbances • Preoccupation with fear of abandonment • Demanding with the parent the child is living with • Aggressiveness with siblings and peers Early school age (6-8 years) • Open grieving • Preoccupation with fear of rejection and of being replaced • Torn by guilt because of conflicting loyalties Later school age (9-12 years) • Profound anger at one or both parents • Distinguishes one parent as the culprit for the divorce • Deterioration in school performance and peer relationship • Sense of loneliness and powerlessness Adolescence • Significant potential for acute depression and even suicidal ideation • Acting out behavior in the form of substance abuse, truancy, sexual activity • Self-doubts about own potential for marital success 242 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

BEHAVIORAL ASSESSMENT AND THERAPY Behavioral assessment is discussed in details in the very next chapter of this volume. In a large majority of the behavioral problems, behavioral approach which aims at either decreasing odd behavior or improving behavior, can be practiced by the pediatricians, parents, and teachers. The characteristics of this approach are listed in Table 19.5.

Table 19.5: Characteristics of behavioral therapy

1. It looks at treating the current problem and not the past. 2. It changes behavior irrespective of the etiology. 3. At any particular time, a single problem-behavior is handled. 4. It involves identifying the problem and understanding its ABC i.e. — Antecedents — Behavior per se — Consequences of behavior 5. It is important to hypothesise the reasons for the cause and the factors that serve to perpetuate the said behavior. 6. After conducting the analysis, the individual-oriented program is chalked out to tackle the problem behavior. 7. A baseline data about the frequency and severity of the behavior is needed before initiating behavioral therapy. 8. After initiation of behavioral therapy, constant evaluation is essential.

Techniques to decrease behavior in such situations as disturbing group activity, temper tantrum, disruptive behavior, nail-biting, enuresis, etc., are: • Time-in and time-out • Extinction and planned ignoring • Punishment • Habit reversal • Positive practice Techniques aimed at improving behavior are: • Time-in and time-out • Incidental learning • Modeling • Token economy • Shaping For details, readers are referred to an excellent review by Tulu et al.8 Behavioral Problems: Overview 243

KEY LEARNING POINTS • Etiology of behavioral problems is multifactorial, including maladjustment at home and/or school and factors operating before pregnancy, during pregnancy, during delivery and in neonatal period. • Overprotection and rejection are two most important underlying factors; often both may operate in a single child. • Some behavioral problems belong to more than one category. • Common behavioral problems include pica, breath-holding spells, thumb-sucking, nail biting, teeth grinding, tics, rhythmic headbanging, temper tantrum, stuttering, enuresis, etc. • In a large majority of the behavioral problems, behavioral approach, which aims at either decreasing odd behavior or improving behavior, can be practiced by the pediatricians, parents, and teachers.

REFERENCES 1. Levy SE. Pediatric evaluation of the child development delay. Child Adolesc Psychiatr Clin North A 1996;5:809. 2. Finney JW, Weist MD. Behavioral assessment of children and adolescents. Pediatr Clin North Am 1992;39:369-378. 3. Block RW, Rash FC. Handbook of Behavioral Pediatrics. Chicago: Year Book 1985. 4. Gupte S, Singh T. Child psychiatry. In: Gupte S (ed) The Short Textbook of Pediatrics, 10th edn. New Delhi: Jaypee 2004: 445-454. 5. Gupte S. Pica. Indian Pediatr 1982;19:1044-1045. 6. Crossby WH. Food, pica and iron-deficiency. Arch Int Med 1971;127:960-962. 7. Illingworth RS. Common Symptoms of Disease in Children, 7th edn. Oxford: Blackwell 1983. 8. Tulu MS, Shah R, Bavdekar SB. Common behavior disorders. In: Gupte S (ed) Recent Advances in Pediatrics-12. New Delhi: Jaypee 2002:473-485. 9. Robinson BA, Tolan W, Golding-Beecher O. Childhood pica: Some aspects of the clinical profile in Manchester, Jamaica. West Ind Med J 1990;39:20-26.8 10. Berg I. School avoidance, school phobia and truancy. In: Lewis M (ed): Child and Adolescent Psychiatry: A Comprehensive Textbook. Balimore: Williams and Wilkins, 1991:1093. RAP Special Volume 19: Developmental and Behavioral Pediatrics 20 Tic Disorders

MS Bhatia, Ravi Gupta

INTRODUCTION Tics are repetitive, involuntary non-rhythmic movements or vocalizations that appear in childhood. It is a multidimensional disorder and can be scaled depending upon—duration of symptoms (from fewer than 1 year to lifelong), intensity of symptoms (from mild to severe), frequency of symptoms (from rare to constant), variety of symptoms (from single motor groups/single phonic tic to multiple occurrences of both types), complexity of tics (from simple motor/phonic tic to multiple motor groups or the production of multiple sounds, including involuntary phrases), response to treatment (from good to minimal or no response), and lastly comorbid psychiatric disorders (from none to multiple).1 Tics interfere with the person’s working and sufferer may feel difficulty in social or occupational dealings. Children suffering from tics are usually distinctly noticed in the school and other children may tease them, making the child more anxious and further aggravating the tics. Hence, timely diagnosis and adequate treatment is of utmost importance. Otherwise, sufferer may develop depression or occupational deterioration.

EPIDEMIOLOGY According to the western literature, tic disorder affects approximately 1 percent school children at a time with a lifetime prevalence of 4-5/10,000.3 Tic disorder has gender bias and is more common in boys than the girls. Transient tics can be seen in approximately 10% children4 and motor tics usually appear before vocal tics. Tic Disorders 245

ETIOLOGY Despite its high prevalence rates, exact pathology could not be elucidated till date. Available literature suggests that basal ganglia and limbic circuits are important in the pathogenesis. Neurochemical studies have implicated dopamine, serotonin and norepinephrine in the development of tics and family studies observed that relatives of TS patient show high degree of tics as well as comorbid illnesses.

Genetic Factors The concept of tics as a developmental disorder is suggested by the sex ratio, early age of onset and the marked tendency to remission after adolescence. Concordance rate of Tourette’s disorder is 53% for monozygotic pairs and 8% for dizygotic pairs.5 It is probably inherited in an X linked or autosomal dominant manner.6 When one parent is a carrier or has Tourette’s disorder, there is 50% chance that each child will receive the genetic vulnerability from the parent. However, not every child who inherits the gene will develop symptoms of disorder, and it indicates incomplete penetrance. The penetrance is higher in males than in females. There is a 99% chance of males but only a 70% chance of females showing small clinical expression of the gene. Recent studies have described that not only the TD, but also comorbidities, e.g. OCD and ADHD are significantly increased in children with TD parents. Furthermore, this risk is even greater when both parents are affected.7 With the development of molecular research techniques, few studies in the recent past have been conducted to find out chromosomal abnormalities associated with TS. They reported that chromosomal regions 18q22,8 17q25,9 7q3110 and balanced translocation t (6;8) are associated with increased risk of TD.11 However, none of the study could be replicated to provide sufficiently strong evidence. Other researches went one step further to find out the possible gene abnormalities in subjects with TS. They suggested a complex mode of inheritance involving contributions of several genes, but with different effect size. Identified genes that increase the risk of TS are—DRD4 gene, MAO-A gene12 and disruption or decreased expression of CNTNAP2 gene. Contactin associated protein gene 2 can lead to a disturbed distribution of the K(+) channels in the nervous system, thereby influencing conduction and/or repolarization of action potentials, causing unwanted actions or movements and clinically expressed as TS.13 In summary, though the evidences for the heritability are present, none of the evidence has substantial ground to be replicated. More research is required in this area. 246 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Neuroanatomical Theories Neuroradiological study described that tics patients show increased number of hyperintensities in the subcortex, particularly basal ganglia in thalamus. This fact shows that subcortex is important in development of pathology in these disorders.14 Different parts of the basal ganglia and thalamocortical system, with a focus on the premotor and prefrontal cortices, are connected with each other via parallel, functionally segregated basal ganglia-thalamocortical systems. These parallel circuits, representing sensorimotor, cognitive and emotional-motivational behavioral processes, are connected with each other through specific pathways that serve to integrate these various functions. The pathways that lead from the ventral striatum via the dopaminergic substantia nigra to the dorsal striatum are important.15 Abnormal activity has been reported in clusters of striatal neurons (matrisomes) during inappropriate contexts leading to inhibition of GPi or SNpr neurons that would normally be active to suppress unwanted movements. The inhibition of these GPi or SNpr neurons would then disinhibit thalamocortical circuits, leading to the production of tics. Activity-dependent dopamine effects would inappropriately reinforce these activity patterns leading to stereotyped repetition. Over the time, overactive striatal neuronal clusters may change under various influences so that the produced movement also changes over time16 (Figure 15.1).

Neurochemistry Many neurotransmitters have been implicated in the production of tics- most of the theories are byproduct of pharmacological interventions. These patients may show a low basic dopamine turnover and an increase in the availability of dopamine D2 receptors in the caudate body,17 deficits in serotonin transporter binding capacity.18 Level of neurotransmission may also influence the severity of tics. Serotonergic neurotransmission in the midbrain and serotonergic or noradrenergic neurotransmission in the thalamus influence severity of vocal tics.19 Moreover, increased availability of D2 receptors in caudate is associated with the severity of motor tics in Tourette’s syndrome.17 Improvement in TS caused by THC implicates the central cannabinoid receptor system to play a role in TS pathology.20 Tic Disorders 247

Neuroimaging Children with Tourette’s disorder have smaller overall corpus callosum size, whereas adults with Tourette’s disorder on average have larger corpus callosum size, yielding a prominent interaction of diagnosis with age. Corpus callosum size correlates positively with tic severity and inversely with dorsolateral prefrontal and orbitofrontal cortical volumes in both the subjects with Tourette’s disorder and the comparison subjects. Given prior evidence for the role of prefrontal hypertrophy in the regulation of tic symptoms, these findings suggest that neural plasticity may contribute to smaller corpus callosum size in persons with Tourette’s disorder, which thereby limits neuronal trafficking across the cerebral hemispheres and reduces input to cortical inhibitory interneurons within the prefrontal cortices. Reduced inhibitory input may in turn enhance prefrontal excitation, thus helping to control tics and possibly contributing to the cortical hyperexcitability reported previously in patients with Tourette’s disorder.21 Semi-quantitative analysis in brain SPECT perfusion showed that, compared with children with chronic tic disorder, children with Tourette’s syndrome had significantly lower perfusion in the left lateral temporal area and asymmetric perfusion in the dorsolateral frontal, lateral and medial temporal areas.22 Metabolic derangements have been demonstrated within regions of the basal ganglia, limbic system and sensorimotor cortex and are in keeping with the concept of TS as both a motor and behavioral disorder.23 Other studies reported normal global and regional metabolic rates in TS with a specific brain network characterized by a reduction in the activity of limbic basal ganglia-thalamocortical projection systems. fMRI studies showed activation in the left middle frontal gyrus, caudate nucleus, cingulate gyrus, cuneus, left angular gyrus, left inferior parietal gyrus, occipital gyri and right precentral gyrus in TS patients.24 In summary, neuroimaging studies have implicated the limbic system and basal ganglia in the development of tics. These findings are in accordance with clinical features and lie behind repetitive movements and emotional content of the disorder.

Other Theories Low levels of plasma ferritin and iron have been reported in the Tourette’s syndrome (TS) patients. Low iron stores may contribute to hypoplasia of 248 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics the caudate and putamen, thereby increasing the vulnerability or severity of tics. It simultaneously also contributes to smaller cortical volumes and consequently reduced inhibitory control of tics.25 Prenatal maternal smoking increases the severity of tics and contributes to presence of comorbid OCD in these subjects. Other variables, such as paternal age and subject’s birth weight are also significantly but less strongly associated with increased symptom severity. However, symptom severity may not be related with hypoxia, forceps delivery, or hyperemesis during pregnancy, as suggested previously.26 Autoimmunological mechanisms seem to be important in some subtypes of TS that are triggered or exacerbated by infections with hemolytic streptococci. In these cases, immune modulatory therapy proved to be efficient.3 Relative short stature appears common in Tourette syndrome, and its presence with other features may implicate a neurotransmitter system that interacts with neuroendocrine pathways, controlling height.27 Endogenous opioids, including dynorphin and met-enkephalin are concentrated in structures of the basal ganglia are known to interact with central dopaminergic neurons, and may play an important role in the control of motor functions. The neuropathological study reported decreased levels of dynorphin A immunoreactivity in striatal fibers projecting to the globus pallidus in the brain of a patient with severe TS. This observation, taken with the neuroanatomic distribution of dynorphin and its broad range of motor and behavioral effects, has led to speculation concerning its role in the pathobiology of TS.28

CLINICAL FEATURES Children with tic disorders are generally placed into one of three diagnostic categories as listed in the American Psychiatric Association Diagnostic and Statistical Manual of Mental Disorders, 4th edition, (DSM-IV).29 1. Transient tic disorder 2. Chronic motor or vocal tic disorder 3. Tourette disorder These categories are based on the types of tics as well as their duration. Certain features are common to all three categories. They all begin before the age of 21, are more common in males and include tics that occur many times a day. Motor and vocal (phonic) tics may be classified as either simple Tic Disorders 249 or complex simple motor tics typically begin with brief bouts of transient tics involving the face or head and there is often a rostralcaudal progression, with tics of the face, head and shoulders appearing earlier and in a higher proportion of patients than motor tics of the trunk or limb. Simple motor tics are sudden, brief meaningless movements such as eye blinking, facial grimacing, nose twitching, lip pouting, neck jerking, shoulder shrugging and abdominal tensing. In contrast, complex motor tics are of longer duration and appear more purposeful. Examples include hitting to self, jumping, hopping, touching to self and others, rolling the eyes upwards or side to side, thrusting out arm, squatting. Other varieties of complex tics may include imitating gestures or movements of other people (echopraxia) or the performance of obscene gestures (copropraxia). Complex motor tics are rarely present in the absence of simple motor tics. Vocal tics may occur in isolation, but they occur more commonly in person who also have motor tics, and they usually appear after the onset of motor tics. As is true of motor tics, vocal tics are classified as simple and complex. The range of possible vocal symptoms is extraordinary and virtually any noise or sound has the potential of evolving into a tic. Example of simple vocal tics include common and natural vocalizations such as throat clearing, sniffing, grunting, coughing, snorting, hissing, shouting, clicking etc. Complex vocal tics involve linguistically meaningful words, phrases or sentences that may be shouted out at inappropriate times. During conversation, vocal symptoms may interfere with the smooth flow of speech and resemble stammering or stuttering. Vocal tics can include repeating the sounds or words of another person (Echolalia), repeating one’s own sounds or words (Palilalia) and involuntary use of obscene language (corprolalia). A transient tic disorder consists of single or multiple motor and/or vocal tics that occur many times a day, nearly every day for at least 4 weeks, but for no longer than 12 consecutive months. The most common forms of tics are eye blinking, facial movements, throat clearing, or sniffing. Age of onset is always during childhood or early adolescence, and may be as early as two years of age. By definition, these tics disappear permanently after being present for less than 1 year. A chronic tic disorder consists of either motor or vocal tics, but not both, lasting for more than 1 year but no tic-free period of more than 3 250 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics consecutive months.29 The other characteristics of the disorder are generally the same as Tourette’s disorder, except that the severity of the symptoms and the functional impairment are usually much less. Tourette’s disorder is the diagnosis reserved for those children who have both multiple motor and vocal tics that have been present for more than 1 year. The tics range in severity from mild to severe. Since the diagnosis of Tourette’s disorder is based, in part, on the presence of symptoms for more than 1 year, children seen earlier in their course can not be diagnosed with certainly until a sufficient period of time has passed. Tourette syndrome and chronic tic disorder are part of the same disease entity, with Tourette syndrome being a more severe and complex form of tic disorder.30 All forms of tics may be exacerbated by anger or stress, but they are usually markedly diminished during sleep. Though, TS is an involuntary movement disorder. However, many patients have stated that without the premonitory sensation, there would be no tics. For this reason, it has been suggested that the premonitory urge may be considered the involuntary component of TS and the performance of the tic merely a voluntary response.23 Premonitory feelings or “sensory experiences,” which are distinct from the actual motor or phonic tics and precede the tics, occur in over 80% of tic-patients and in 95% of patients with Gilles de la Tourette Syndrome (GTS).4 Anatomical regions with the greatest density of urges were the palms, shoulders, midline abdomen, and throat.28 Another study reported that the most common sensation was an urge to move and an impulse to tic (“had to do it”).32 Intensification of premonitory sensations, if prevented from performing a motor tic, was reported with relief of premonitory sensations after performing the tic.32 A substantial majority of patients indicate that their tics were either fully or partially a voluntary response to the premonitory urges challenging the involuntary nature of tics.31-32 The “just right” sensation correlated with the presence of comorbid obsessive-compulsive disorder.

COMORBIDITY ADHD as a comorbid disorder has been reported in 35-90% of children with TS. The ADHD seen in TS is similar to ADHD in children without tics, although attentional difficulties seen in TS sufferers are influenced by tics as well as by internal distractions that are common in comorbid Tic Disorders 251 anxiety or OCD.33 Besides ADHD, other comorbid illnesses are OCD, learning disorder and other behavior disorders.34 These children are also prone to develop mood disorders particularly, depression.35 Sleep disturbances are common in patients with Tourette’s syndrome, with decreased sleep efficiency and slow wave sleep percentage, increased sleep latency, percentage of stage I, percentage of awakeness, number of awakenings, and sleep stage changes and more overall movements during sleep. Severity of Tourette’s syndrome during the day correlates significantly and positive with number of awakenings and sleep stage changes and negatively with sleep efficiency. In addition to an increased number of regular movements patients show tics in all sleep stages. Tic frequency as well as frequency of regular movements was significantly higher in REM than in non-REM sleep. The increased motor activity may be attributable to a state of hyperarousal rather than a disturbed cholinergic system.36

COMPLICATIONS Complications include physical injury, such as blindness due to retinal detachment (from head banging or striking oneself), orthopedic problems (from knee-bending, neck jerking or head trauma), skin problems (from picking) and in rare cases, self mutilation (from head banging). Person with this disorder have no shortening of their life span and many persons have now been described, who have Tourette disorder and, are in their 70s and 80s. Repeated air swallowing, in turn, causing abdominal cramping, eructation, and flatus, eventually leading to aeroenteria was reported as a complication of vocal tics.37

ASSESSMENT OF CHILD WITH A TIC DISORDER Diagnosis must be based on the history and physical examination since there are no diagnostic laboratory studies. The anatomical location, number, frequency, intensity, complexity, and degree of disruption associated with motor and phonic tics should be documented. The age of onset should be noted along with associated stressors and toxic insults such as stimulant medication. Clinicians should delineate the course of tics over the past year and over the past several months prior to presentation. Assessment should substantiate clinical waxing and waning of the disorder, duration of severe tic symptoms, and factors associated 252 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics with exacerbation or improvement. Questions should be asked regarding the possibility of associated sensory urges or mental phenomenon associated with tics and what impact on the individual’s self esteem, social adaptation or academic performance do tic symptoms have? Finally an assessment should be made for risk to self injury as a result of presence of tics. Such finding will serve as a record of tic symptom severity at the time of initial presentation and for documentation of impairment associated with motor and phonic phenomenon. Associated behavioral phenomena should be assessed in addition to tics. These include the presence or absence of obsessive compulsive symptoms and behaviors, such as simple rituals and full fledged OCD. Specific question should be asked about the possibility of attentional problems, mood liability, irritability, depression and anxiety. How does the patient get along with family and friends? What is his and her premorbid history and adjustment before tic onset. What are the life events associated with onset and exacerbation of tic, symptoms, coping skills, and social support available to the patient? A genetic family history should be taken about other relatives or family members, who have unusual movement problems or obsessive compulsive behaviors. A past medical or developmental history may be relevant to the presence of tics such as prenatal birth history, developmental delays, medication exposures and injuries. Except for the tics, physical and neurological examinations are normal in persons with Tourette’s disorder or any other form of a tic disorder, although the presence of soft signs has some times been emphasized. Soft signs generally reflect the maturity and degree of development of central nervous system. Many scientists now believe that soft signs are not a reliable or important part of the evaluation of persons with tic disorder. Since tic can be suppressed during the time that the child is in the doctors office, no tics may be seen during the interview and examination. Nevertheless, the history can be considered reliable if the description is typical for tics. In the usual practice, in a child with a tic disorder, laboratory testing is unnecessary. All clinically available tests are normal and laboratory testing is ordered only when other causes for involuntary movements are being considered. EEG is normal, although some reports have described minor, Tic Disorders 253 non-diagnostic abnormalities.39 Computerized Tomography (CT) scans and magnetic resonance imaging (MRI) scans are normal in patients with tic disorders. Psychological testing does not diagnose Tourette’s disorder and tic disorder, but it may identify associated conditions such as learning disabilities or attention deficits.

TREATMENT No cure exists for Tourette’s disorder or any other tic disorder. Potential treatments are symptomatic, and there is no evidence that early treatment alters the natural course of the disorder. Treatment is recommended if the symptoms impair psychosocial, educational or occupational functioning. Aims of the treatment should focus primarily on the alleviation of tics, while associated disorder should be evaluated separately and treated if indicated.

Pharmacotherapy The physician should not attempt to begin medication at the initial visit. The first few visits should be used to help establish a baseline of symptoms and to determine what difficulties is present in addition to tics. The time is also used to educate child and family about the disorder and helping them cope with the impact of the diagnosis. In child’s tics are mild and of little functional significance, these children do not require medical treatment, and their family can be reassured that medications are available if tics worsen in future. Pharmacotherapy is at present the main stay of treatment for Tourette’s disorder and other tic disorders. The patients are always started on the smallest possible dose of medication. The dosage is increased gradually with keeping in mind to both the development of side effects and the improvement in symptoms. It may take several weeks of treatment before the beneficial effect of medication is noted. Once effective medication is found, the lowest effective dosage is maintained, but it is must to change the dosage time to keep up with the natural fluctuation of symptoms. Haloperidol, a dopamine-blocking agent, remains the best known and most widely used medication in the treatment of Tourette’s disorder and other tic disorders. Haloperidol improves symptoms in 86 percent of patients.38 Excess sedation and other side effects can be avoided by starting with dose of 0,25-0.5 mg/day and increasing by 0.5 mg every week until 254 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics a point is reached with maximum benefit and minimum side effects after as small a dose as 2-3 mg daily is adequate for symptoms’ relief. It is usually administered orally, once a day at bed time, many have started using intramuscular haloperidol decanoate (25 mg every fortnight) with success. Pimozide is also effective in a large proportion of patients39 and produces less sedation or lethargy than haloperidol. Doses of 0.5 to 9 mg/ day once at bedtime are adequate to control the symptoms. In everyday practice it is preferable to start with an “atypical” neuroleptic drug—for example, olanzapine (5-10 mg/day), risperidone or clozapine.4 However, the problems associated with the use of neuroleptic agents have led to a search for alternative medications. Clonidine, an alpha- adrenergic agonist most frequently used for hypertension is an alternative medication. Although it is relatively free of severe side effects, reports on the effectiveness of clonidine vary. Some studies have indicated that upto 70 percent of treated patients respond favorably.40 Clonidine is started at 0.5 mg once a day and this is increased by 0.5 mg a day on weekly basis until there is improvement or until tolerance is reached. Clonidine is best taken in small doses, three times each day because of short half life. Because of the lowered incidence of side effect, many people begin medication with clonidine and reserve the use of neuroleptic for refractory cases. Other drugs that can be tried with varying success are neuroleptics such as fluphenazine, penfluridol, the antidepressant clomipramine and the anticonvulsant clonazepam. Recent successful treatment of Tourette disorder with calcium antagonists such as nifedipine has been documented as has that with naloxone, lithium, tetrabenazine in combination with pimozide, progabide and physostigmine. Treatment of Tourette syndrome combined with ADHD is often challenging. Alpha 2 agonists, such as guanfacine, or non stimulant options such as atomoxetine, where available, are worth considering, although many individuals with Tourette syndrome and ADHD can be successfully treated with judicious doses of stimulant medications.41 Psychostimulants in general do not worsen the coexisting tics in ADHD subjects though individual patients may experience exacerbation of tics. SSRIs (sertraline, citalopram, fluoxetine, fluvoxamine) or other antidepressants (clomipramine) have been used in treatment of psychiatric comorbid conditions, too. Botulinum toxin injections have proved useful in tics, targeting at the symptoms of blepharospasm, in neck and facial muscles.4 THC is also effective and safe in the treatment of tics.20 Tic Disorders 255

In most cases, the disorder is self-limited or can be treated by medication or behavioral therapy. In a small percentage, however, symptoms are intractable to any conservative treatment. Since 1955, various attempts have been made to treat these patients through neurosurgical procedures. The target sites have been diverse and include the frontal lobe (prefrontal lobotomy and bimedial frontal leukotomy), the limbic system (limbic leukotomy and anterior cingulotomy), the thalamus, and the cerebellum. Combined approaches have also been tried such as anterior cingulotomies plus infrathalamic lesions. The results have often been unsatisfactory or major side effects have occurred, such as hemiplegia or dystonia.42

Psychosocial Management What is vitally important is supportive psychotherapy, counseling and advice to both patient and family as the illness can be disabling not only to affected individual, but to family members as well. Support in helping the patient and family to cope are important as is guidance to teachers. Formal psychotherapy, hypnotherapy, behavior therapy and psychoanalysis have limited or no place in treatment of the symptoms of Tourette disorder and other tic disorders.

KEY LEARNING POINTS • Tics, a multidimensional entity, are repetitive, involuntary non- rhythmic movements or vocalizations that appear in childhood • Basal ganglia and limbic circuits are important in the pathogenesis. Neurochemical studies have implicated dopamine, serotonin and norepinephrine in the development of tics and family studies observed that relatives of TS patient show high degree of tics as well as co-morbid illnesses. • The concept of tics as a developmental disorder is suggested by the sex ratio, early age of onset and the marked tendency to remission after adolescence. • Three diagnostic categories as listed are transient tic disorder, chronic motor or vocal tic disorder and Tourette disorder. • Besides ADHD, other comorbid illnesses are OCD, learning disorder and other behavior disorders. These children are also prone to develop mood disorders particularly, depression. 256 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Complications include physical injury, such as blindness due to retinal detachment (from head banging or striking oneself), orthopedic problems (from knee-bending, neck jerking or head trauma), skin problems (from picking) and in rare cases, self mutilation (from head banging. • In most cases, the disorder is self-limited or can be treated by medication or behavioral therapy. • Treatment is recommended if the symptoms impair psychosocial, educational or occupational functioning. • Haloperidol, a dopamine-blocking agent, remains the best known and most widely used medication in the treatment of Tourette’s disorder. • Supportive psychotherapy, counseling and advice to both patient and family are vital as the illness can be disabling. • Formal psychotherapy, hypnotherapy, behavior therapy and psychoanalysis have limited or no place in treatment of the symptoms of Tourette disorder and other tic disorders.

REFERENCES 1. Kuperman S. Tics and Tourette’s syndrome in childhood. Semin Pediatr Neurol 2003;10:35-40. 2. Stern JS, Burza S, Robertson MM. Gilles de la Tourette’s syndrome and its impact in the UK. Postgrad Med J 2005;81:12-19. 3. Riederer F, Stamenkovic M, Schindler SD, Kasper S. Tourette’s syndrome- A review. Nervenarzt 2002;73(9): 805-819. 4. Czaplinski A, Steck AJ, Fuhr P. Tic syndrome. Neurol Neurochir Pol 2002; 36:493- 504. 5. Pauls DL, Cohen DJ, Heimbuch R, Deltor J, Kidd KK. Familial pattern and transmission of Gilles de la Tourette syndrome and Multiple tics. Arch Gen Psychiatry 1981;38:1091-1093. 6. Cabrera-Lopez JC, Marti-Herrero M, Fernandez-Burriel M, Toledo-Bravo de Laguna L, Dominguez-Ramirez S, Fortea-Sevilla S. Paroxysmal stereotype-tic- dystonia syndrome. Rev Neurol 2003;36(8): 729-734. 7. McMahon WM, Carter AS, Fredine N, Pauls DL. Children at familial risk for Tourette’s disorder: Child and parent diagnoses. Am J Med Genet B Neuropsychiatr Genet 2003;121:105-111. 8. Cuker A, State MW, King RA, Davis N, Ward DC. Candidate locus for Gilles de la Tourette syndrome/obsessive compulsive disorder/chronic tic disorder at 18q22. Am J Med Genet A 2004;130:37-39. 9. Paschou P, Feng Y, Pakstis AJ, Speed WC, DeMille MM, Kidd JR, Jaghori B, Kurlan R, Pauls DL, Sandor P, Barr CL, Kidd KK. Indications of linkage and association of Gilles de la Tourette syndrome in two independent family samples: 17q25 is a putative susceptibility region. Am J Hum Genet 2004;75:545-560. Tic Disorders 257

10. Diaz-Anzaldua A, Joober R, Riviere JB, Dion Y, Lesperance P, Chouinard S, Richer F, Rouleau GA. Association between 7q31 markers and Tourette syndrome. Am J Med Genet A 2004;127:17-20. 11. Crawford FC, Ait-Ghezala G, Morris M, Sutcliffe MJ, Hauser RA, Silver AA, Mullan MJ. Translocation breakpoint in two unrelated Tourette syndrome cases, within a region previously linked to the disorder. Hum Genet 2003; 113:154-161. 12. Diaz-Anzaldua A, Joober R, Riviere JB, Dion Y, Lesperance P, Richer F, Chouinard S, Rouleau GA; Montreal Tourette Syndrome Study Group. Tourette syndrome and dopaminergic genes: A family-based association study in the French Canadian founder population. Mol Psychiatry 2004; 9: 272-277. 13. Verkerk AJ, Mathews CA, Joosse M, Eussen BH, Heutink P, Oostra BA. Tourette Syndrome Association International Consortium for Genetics. CNTNAP2 is disrupted in a family with Gilles de la Tourette syndrome and obsessive compulsive disorder. Genomics 2003;82:1-9. 14. Amat JA, Bronen RA, Saluja S, Sato N, Zhu H, Gorman DA, Royal J, Peterson BS. Increased number of subcortical hyperintensities on MRI in children and adolescents with Tourette’s syndrome, obsessive-compulsive disorder, and attention deficit hyperactivity disorder. Am J Psychiatry 2006;163:1106-1108. 15. Groenewegen HJ, van den Heuvel OA, Cath DC, Voorn P, Veltman DJ. Does an imbalance between the dorsal and ventral striatopallidal systems play a role in Tourette’s syndrome? A neuronal circuit approach. Brain Dev 2003; 25 Suppl 1: S3-S14. 16. Mink JW. Neurobiology of basal ganglia and Tourette syndrome: basal ganglia circuits and thalamocortical outputs. Adv Neurol 2006;99:89-98. 17. Kienast T, Heinz A. Dopamine and the diseased brain. CNS Neurol Disord Drug Targets 2006; 5:109-131. 18. Muller-Vahl KR, Meyer GJ, Knapp WH, Emrich HM, Gielow P, Brucke T, Berding G. Serotonin transporter binding in Tourette Syndrome. Neurosci Lett 2005; 385:120-125. 19. Heinz A, Knable MB, Wolf SS, Jones DW, Gorey JG, Hyde TM, Weinberger DR. Tourette’s syndrome: [I-123]beta-CIT SPECT correlates of vocal tic severity. Neurology 1998; 51:1069-1074. 20. Muller-Vahl KR, Schneider U, Prevedel H, Theloe K, Kolbe H, Daldrup T, Emrich HM. Delta 9-tetrahydrocannabinol (THC) is effective in the treatment of tics in Tourette syndrome: a 6-week randomized trial. J Clin Psychiatry 2003; 64: 459-465. 21. Plessen KJ, Wentzel-Larsen T, Hugdahl K, Feineigle P, Klein J, Staib LH, Leckman JF, Bansal R, Peterson BS. Altered interhemispheric connectivity in individuals with Tourette’s disorder. Am J Psychiatry 2004;161:2028-2037. 22. Chiu NT, Chang YC, Lee BF, Huang CC, Wang ST. Differences in 99mTc-HMPAO brain SPET perfusion imaging between Tourette’s syndrome and chronic tic disorder in children. Eur J Nucl Med 2001;28:183-190. 23. Adams JR, Troiano AR, Calne DB. Functional imaging in Tourette’s syndrome. J Neural Transm 2004;111:1495-1506. 24. Gates L, Clarke JR, Stokes A, Somorjai R, Jarmasz M, Vandorpe R, Dursun SM. Neuroanatomy of coprolalia in Tourette syndrome using functional magnetic resonance imaging. Prog Neuropsychopharmacol Biol Psychiatry 2004;28:397-400. 25. Gorman DA, Zhu H, Anderson GM, Davies M, Peterson BS. Ferritin Levels and Their Association With Regional Brain Volumes in Tourette’s Syndrome. Am J Psychiatry 2006;163:1264-1272. 258 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

26. Mathews CA, Bimson B, Lowe TL, Herrera LD, Budman CL, Erenberg G, Naarden A, Bruun RD, Freimer NB, Reus VI. Association between maternal smoking and increased symptom severity in Tourette’s syndrome. Am J Psychiatry 2006;163:1066-1073. 27. Zelnik N, Newfield RS, Silman-Stolar Z, Goikhman I. Height distribution in children with Tourette syndrome. J Child Neurol 2002;17:200-204. 28. Leckman JF, Riddle MA, Berrettini WH, Anderson GM, Hardin M, Chappell P, Bissette G, Nemeroff CB, Goodman WK, Cohen DJ. Elevated CSF dynorphin A [1-8] in Tourette’s syndrome. Life Sci 1988;43:2015-2023. 29. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders, 4th edn. Washington DC: American Psychiatric Association 1994. 30. Saccomani L, Fabiana V, Manuela B, Giambattista R. Tourette syndrome and chronic tics in a sample of children and adolescents. Brain Dev 2005;27:349-352. 31. Leckman JF, Walker DE, Cohen DJ. Premonitory urges in Tourette’s syndrome. Am J Psychiatry 1993;150:98-102. 32. Kwak C, Dat Vuong K, Jankovic J. Premonitory sensory phenomenon in Tourette’s syndrome. Mov Disord 2003;18:1530-1533. 33. Erenberg G. The relationship between tourette syndrome, attention deficit hyperactivity disorder, and stimulant medication: A critical review. Semin Pediatr Neurol 2005;12:217-221. 34. Benarroch F, Warman O, Gross-Tsur V. Tourette syndrome: from a neurological clinic to a multidisciplinary approach. Harefuah 2006;145(4):292-297,317. 35. Marcus D, Kurlan R. Tics and its disorders. Neurol Clin 2001;19:735-58. 36. Cohrs S, Rasch T, Altmeyer S, Kinkelbur J, Kostanecka T, Rothenberger A, Ruther E, Hajak G. Decreased sleep quality and increased sleep related movements in patients with Tourette’s syndrome. J Neurol Neurosurg Psychiatry 2001;70:192- 197. 37. Frye RE, Hait EJ. Air swallowing caused recurrent ileus in Tourette’s syndrome. Pediatrics 2006;117:e1249-1252. 38. Shapiro AK, Shapiro E. Tourette syndrome history and present status. In: Friedhoff AJ, Chase TN (eds): Advances in Neurology, Vol. 35. New York: Raven Press 1982. 39. Verma NP, Syrigov Papavasilliov A, Lewitt PA. Electroencephalographic finding in unmedicated, neurologically and intellectually intact Tourette syndrome patients. Electroencephal Clin Neurophysio 1986;64:12-20. 40. Cohen DJ Dettor J, Young JG, Shaywitz BA. Clonidine ameliorates Gilles de la Tourette Syndrome. Arch Gen Psychiatry 1980;37:1350-1357. 41. Castellanos FX, Acosta MT. Tourette syndrome: An analysis of its comorbidity and specific treatment. Rev Neurol 2004;38 Suppl 1:S124-130. 42. Temel Y, Visser-Vandewalle V. Surgery in Tourette syndrome. Mov Disord 2004; 19:3-14. RAP Special Volume 19: Developmental and Behavioral Pediatrics 21 Risk-taking Behavior

Satish K Tiwari

INTRODUCTION The current “hightech” age, urbanization, industrialization and globalization has lead to innumerable social changes. In most cultures, adolescence is considered a turbulent period. The adolescent who undergoes rapid changes in physical, biological, psychological and social sphere has to fulfill not only his own expectations but also those of his parents, peer groups and society. The prolonged period of adolescence is resulting in newer and newer ranges of psychosocial behavior and development. The problems like depression, violence, criminal thinking, abnormal sexual activities, vandalism etc. are increasing. The incidences of alcoholism, drug abuse, sex abuse, attempted suicides etc. are increasing at an alarming rate. Demand of acceptance in the peer group often makes the adolescent indulge in risk behaviors. More adolescents today than in past generations are involved in a behavior that threatens their health. Many adolescents are involved in multiple risk behaviors.1

NORMAL CHANGES There are lot of upheavals and restructuring during adolescence. The transformation from child to adolescence can present many ups and downs and obstacles in the life of a teenager. Emotional and psychosocial changes are most important and vital as they influence the rest of the life of the individual.2,3 Many factors decide the psychological development during this important phase of life. During this process of learning, experimentation and growing up, they make some mistakes. Self-esteem, 260 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

FIGURE 21.1: Flowchart showing emotional and psychological changes during adolescence self-confidence, self-identity along with critical/abstract thinking and decision-making are important developmental issues. The behavior many times may be perplexing and provocative. The important changes may be summarized in brief as in flow chart (Figure 21.1).

PREDISPOSING CIRCUMSTANCES There are many environmental and social circumstances, which predisposes the children or adolescents towards the risk taking behavior. a. Age of experimentation: Childhood is the age of natural experimentation and exploration when they are curious and adventurous. b. The Family Environment: The parents, the family, friends and peers and psychosocial environment shape the personality of every individual. The family environment including relations with parents, working parents, parental discord, broken families, loss of parents, decreasing family influence etc. predisposes to risk taking behavior. c. School Environment: Higher expectations and resulting stress, school failures, poor skill developments, living in boarding school or hostels may lead to risky behavior. The teachers as a role model in using Risk-taking Behavior 261

tobacco or imparting the knowledge of influence of tobacco use may alter the behavior of students. d. Modernization: The present age of advertisement, modernization, urbanization, globalization, increasing travel, migration, refugee population, widespread telecommunication, modern gadgets/ instruments, access to potentially harmful substances may result in risk-taking behavior. The pressure of modern society, peer-group acceptance, escapism and rebellion against establishment are prime factors influencing the gullible young mind getting sucked into vortex. e. Role of Media: The entertainment media is an important aspect of the environment that greatly influences the adolescents. Rap music and hip-hop, with their particular emphasis on sex and demeaning depiction of women, were blamed for encouraging early sexual behavior, leading to spread of disease and underage pregnancies. The glamorization of reel life and real life may provoke the adolescent to risky behavior. The media blitz, information explosion and technological advances have resulted in many unwanted, unlawful, antisocial and immoral activities. f. Social circumstances: Extreme poverty, exploitation, physical/mental disability, unemployment, homelessness influence the behavior of young people. The social instability caused by erosion of standards and values may lead to sociopathic behavior. g. Other factors: Addiction, abuse of drugs, criminal tendencies, involvement in terrorist activities, warfare and other emergencies etc. also leads to risky behavior.

VARIOUS ACTS/BEHAVIOR These include aggressive or disruptive behavior towards parents, siblings, teachers, peers and friends. These types of behavior are expressed either individually or in a group. Risk behaviors tend to be associated with each other in predictable ways. The onset of one behavior may indicate that another behavior has a greater likelihood of being initiated in the near future.4

Drug Abuse Nowadays drug abuse is becoming a global phenomenon. Drug abuse has reached an alarming proportion in recent years. “Drug culture” is fast 262 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics making in roads into the lives of young people from all walks of life. Many teenagers take it as a proof of maturation and entry to “pseudo-adulthood.” The increasing trend of drug abuse in girls can be due to the misconception that “modern femininity” means behaving or adopting male life-style. According to the Global Youth Tobacco Survey,5 the use of tobacco is gradually increasing in adolescents. It may start as early as 8-10 years of age. The risk of tobacco use is highest among those who start early and continue its use for a long period. The risk is more in those children who are asked by their parents or teachers to buy tobacco for them.

Sexual Activities In ancient India, there was a trend of child marriage. But, there were lots of discussions regarding these customs and its ill effects. In fact, child marriages were made punishable offence by enacting Child Marriage Restraint Act. Such restrictions may be one of the reasons for increasing incidences of high-risk sexual behavior amongst the adolescent population. An unwelcome outcome of delayed marriage and increasing literacy is the prolonged adolescence with a higher risk of premarital sexual activity. In India, although traditional norms oppose premarital sex, some studies indicate a growing trend towards premarital sexual activities among adolescents. The higher the exposure to sexual content in movies, TV, music and magazines, the more likely teens were to have intercourse.

Cyber Sex The incidence of cyber sex including ‘online dating’ has increased in last few years. More and more adolescents are engaging in online friendship and subsequent sexual activities. Some such relationships also end in sexual crimes. Last few years have witnessed uprising in cyber crimes and online delinquent activities. There has been ‘SMS galore’ involving voyeuristic and sexual abuse/behavior. The ‘Internet’ has further exacerbated the problem. Pornography through mobile phones is rising at an alarming speed. There is widespread and continuing practice of ‘sex tourism’, to which children are especially vulnerable, as it directly promotes the child prostitution and child pornography. Risk-taking Behavior 263

Live-in-relationship The era of modernization, busy-life, false sense of autonomy etc. is leading to increasing incidences of live in relationships amongst younger generation. There are many drawbacks associated with this, like emotional disturbances, familial disturbances, legal status of children etc., as there is no social acceptance for such style of living. One of the major problems of risk-taking behavior in the present scenario is explosion in the incidence of HIV/AIDS. It is observed that today’s youngsters have lost the traditional moral values. It is difficult for the parents to accept that their own little ones are at risk of HIV infection. But the hard fact and ground reality is that the incidences of these are gradually increasing in adolescence at an alarming rate. The rising rates of sexually transmitted infections (STIs), including HIV/AIDS, demand that young people be educated about the dangers of unsafe sex and STI symptoms, as well as prevention and treatment of STIs.

Delinquent Acts Any child may break the law and indulge in antisocial activities occasionally. But, when a child or adolescent indulges in premeditated, purposeful, unlawful activities habitually and repeatedly, he is considered to be a juvenile delinquent.

“Super Brat Syndrome” It is easy for children of powerful parents to step out of the line. Because, they are aware that in case of trouble, the parents will inevitably bail them out. Most rich kids believe money can buy power. Many times the individual believes that he was invincible. He doesn’t think that things will go so completely out of hand. Unfortunately for children of rich parents, this is a way of life. A power-puffed kid believes that absence of norms is a way of life.6

Street Children According to UNICEF, there are more than 5,00,000 street children in India who live and work in inhuman conditions and are involved in high-risk behaviors. There are particular problems in the provision of health and welfare services to street children with regards not only to health care but 264 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics also to housing, educational opportunities and employment. They struggle for their survival, alone, without any family support. While often called “abandoned” they might have also abandoned their families. Tired of insecurity, rejection, and grown up with violence, their ties with home have now been broken.7

Violent Behavior It is astounding how violence related events are increasing among adolescents globally. Violence is abusive or unjust exercise of power. It affects the lives of significant portion of adolescents regardless of social class, race, ethnicity, culture or country. Our role is to ease this journey as much as possible for our youth while dealing with a violent world ourselves. Some of them may indulge in violent criminal activities.

Terrorism Violence due to war and political unrest is fairly common in several countries. Juvenile terrorism is the extreme end on the scale/spectrum of juvenile delinquency. Some of the terrorist organizations are misguiding, compelling and incriminating the teenagers/youths in terrorist activities on the grounds of caste, religion, nationality, etc.

Accident Proneness An accident is unexpected, unpremeditated, unplanned event resulting in recognizable unintended damage. Adolescents by nature are rash and adventurous. They indulge in vehicular driving before the specified age and without proper training. Their mind is in state of turmoil. Hence the chances of accidents are increased.

Suicidal Tendencies The conflicts and pressures during adolescent changes, poverty and socioeconomic problems, failures in expectations, etc. predisposes to suicidal tendencies. Girls lead boys in the incidence of attempts, whereas boys outnumber girls in completed suicides. Suicide is becoming one of the leading causes of adolescent death and the rate of suicide is increasing in last few decades. Statistics shows that 12-15 years old ones or even younger children do make serious suicide attempts. Many times it is a Risk-taking Behavior 265 last step to counter helpless or lost situation in the life of an individual. Thus, it is a “cry for help” and all concerned must recognize the emergency and give a prompt help to the affected teenager. Fictional depiction of suicides in films or television serves as stimuli for imitative behavior using similar modes.

Childhood Obesity The increasing proportion of fat and energy dense food in the diet, a lot of junk foods together with reduction in the level of physical activity and rise in sedentary behaviors are the major factors responsible for the increasing average body weight of the population. Decrease in physical activities, increased television viewing, availability of video/computer games and other indoor activities also predispose to childhood obesity.8 The insidious, creeping pandemic of obesity is now engulfing the entire world. Complications arising from obese conditions could, however, heighten risk factors that would create conditions for hypertension, cardiovascular diseases, diabetes, stroke and some forms of cancers. Since obesity is more likely a lifestyle disorder, treatment should involve lifestyle corrections rather than intensive medical care except in extreme cases.

IMPACT OF HIGH RISK BEHAVIORS On Individual They become touchy, sensitive and irrationally emotional. Adolescents get confused many a times over their own behavior and have a confused identity of their own body, which causes changes in emotions and behavior. There is a risk that children’s best interests are getting lost in the debate over teen sexuality. Risk-taking sexual activity can result in sexually transmitted diseases, AIDS/HIV infection, adolescent pregnancy, unlawful abortions, maternal deaths, abandoned babies etc. Early marriage and early pregnancy can interrupt physiological growth of the mother and can result in birth complications/ low birth weight babies. Drug abuse may result in drug addiction, gastritis, hepatitis, chronic liver disease, increased chances of malignancy, global dementia, chronic disability, etc. The use of drug may adversely and seriously affect the health of individual many times resulting in premature death in early middle life. A drug addict adolescent engages in robbery, burglary, drug 266 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics dealing or prostitution to acquire money to buy the drugs and other high- risk behaviors. The school performance deteriorates, family conflicts intensify and the child may lose interest in day-to-day activities. They get frustrated, irritated or depressed. Many adolescents experience a sense of futility, personal disorganization and aimlessness. They feel inadequate, impersonalized and alienated. The impact of violence on mental health is invariably detrimental. It may result in psychological distress and poor academic performance and various behavioral problems. It may result in rejection by parents, peer groups and school teachers.

On Family There are increased chances of emotional and physical isolation/rejection and detachment from the family. Risk-taking behavior may result in increased family discord, parent-child disputes, etc. It may affect the emotional development and behavior of all the members in the family.

On Community and Nation Violence is a major threat to the welfare and prosperity of any society. The other members in the society are the ‘silent or invisible victims’. The ‘fragile states’ are characterized by weak institutions with high levels of corruptions, political instability and weak rule of law. Drug addiction may result in loss of constructive power and activities of the younger generation. If the “gen-next” of any state is addicted, it will hamper the development of the entire nation. Various health-related problems not only burden the exchequer of the country but also result in unhealthy future generation. HIV/AIDS is wreaking havoc with children’s lives in the worst affected countries. In addition to orphaning and the loss of caregivers, there is also lack of access to essential services and increased risk of survival of children and young people.

THE ROLE OF Pediatrician The pediatricians must identify the major problem areas and divide them according to priorities. While interviewing a teenager, don’t forget to establish rapport, create an atmosphere of comfort and confidentiality. Be Risk-taking Behavior 267 attentive, receptive, empathetic, understanding and helpful to the adolescent. Discuss and share the feelings. Encourage the strengths, positive aspects and avoid negative points or weaknesses in the personality of the adolescent. The pediatricians must be patient, nonjudgmental, noncritical and without a personal bias. The teenager and the parents must be interviewed separately if needed. Be open, honest and fair. Emphasize on dangers of irresponsible sexual behavior. A pediatrician may organize health awareness and sex education programs. The help of other colleagues like obstetrician and psychiatrist may be taken whenever required. There is need to sensitize the health professionals so as to be responsive to the needs of adolescents in difficult circumstances. Adolescent Sexual and Reproductive Health (ASRH) clinics may be established.

Parents The parents must accept the child as she is and shouldn’t try to live their own dreams through their child. Don’t compare the child with other children; remember that every child is different. If the parents and teachers are too dominating or possessive, problem may become extremely difficult to solve. The parents must be clear about their own sexual values and attitudes. They should talk early and often with their children about sex. They need to teach children about dating, drugs, friendship and other things which teenager may try to experiment. Know what your kids are watching, reading and listening to. Supervise and monitor your children. Know your children’s friends and their families.9 The parents should be made aware of positive points and should be advised to reinforce these qualities by verbally mentioning them or by rewarding them. Parents should be educated about the negative impact of the TV on physical and mental health of the children and how to prevent it. Communicate freely with the children and let them enjoy their childhood.

Voluntary Organizations Sex education programs may help in encouraging how to avoid early sex (especially penetrative sex), drug abuse, extramarital sex etc. The information regarding use of contraceptives, prevention of unwanted pregnancy, protection from STDs including HIV may be imparted. Any drug control program in children to be successful should involve friends, 268 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics peer groups and teachers. The qualities of responsible sexual behavior are: • Do with a willing partner, monogamous partner • No premarital sex • Use of a condom • Avoid high risk sexual behavior and • It should not hurt anybody nor hurt yourself.10

Government The government can enact and enforce specific laws and policies to promote adolescent health programs. According to the Convention on the Rights of the Child (part I, Article 34), the state shall protect the child from all forms of sexual exploitation and abuse. It has been suggested and stressed that there should be a closer cooperation and partnership between Government and Internet industries so that worldwide criminalization and penalization action may be taken effectively against childhood pornography and sexual exploitation.

Media The media can be employed in a positive and critical way to address different aspects of the adolescent behavior, social norms, attitude and environment. Media programmers and journalists should be educated regarding minimizing of the imitating behavior. The adolescent morbidity and mortality is usually related to life-style (social, environmental and behavior) practices and their consequences. The risk-taking behavior may fulfill the developmental needs such as self- esteem, identity, autonomy and intimacy. But one must not forget the potentially negative consequences of unwise, unprotected high-risk behavior. These dangers when initiated in adolescence can persist well into adult life culminating in significant degrees of mortalities and morbidities. Thus, there are more chances of “social morbidities.” The real work of meeting the needs of adolescents could be done in homes, communities, villages, towns and cities in countries around the world. It would require a huge commitment from government and all sections of societies like youth activists, civic leaders, teachers, medical professionals, parents, schools and colleges, government agencies, NGOs and peoples Risk-taking Behavior 269 from all walks of life. Reducing the health-risk behaviors of the adolescent is a challenge that is best accomplished with the support of other preventive services. Planning should preferably be decentralized with due recognition to local culture and the need to maximize community participation. Proper counseling and interventions are needed to tackle these disturbing situations. Children of rich parents believe that they are above the law because, in most cases, their parents actually break the law with impunity. The prolonged period of adolescence is resulting in newer and newer ranges of psychosocial behavior and development. Children represent the most important asset and wealth of a nation. There is need to protect and nurture this wealth through proper growth and development. There is a need for “youth friendly services” if young people are to be adequately provided with preventive, promotive and curative care. WHAT TO DO All adolescents must have best possible start to adult life. Helping adolescent begins with good health care, good nutrition, quality education, psychosocial support, healthy and accident-free environment. Sensitize media including television, press and websites on adolescent issues by making special programs and giving proper guidance and information to the community. The government and voluntary organizations should come forward with a helping hand to prevent social ills and exploitation. Framing of national policy on adolescent care with its proper implementation and monitoring will go a long way in developing an “adolescent friendly community.” Empower younger generation to refuse unwanted, unprotected sex and teach them responsible sexual behavior. We have to reaffirm our commitment to complete the unfinished agenda of the world summit for the children and to readdress other emerging issues vital to the achievement of the long-term goals and objectives endorsed at recent major United Nations summits and conferences, in particular the United Nations Millennium Declarations.11 Overall the child is disorganized internally and has feeling of insecurity. KEY LEARNING POINTS • These include aggressive or disruptive behavior towards parents, siblings, teachers, peers and friends • The adverse effect is not only in relation to the individual but also on family, community and the nation. 270 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Pediatricians must identify the major problem areas and divide them according to priorities. Teenager and the parents must be interviewed separately if needed. Be open, honest and fair. Emphasis should be on dangers of irresponsible sexual behavior. Health awareness and sex education programs may be organized. • The parents should be made aware of positive points and should be advised to reinforce these qualities by verbally mentioning them or by rewarding them. • Voluntary Organizations should Sex education programs may help in encouraging how to avoid early sex (especially penetrative sex), drug abuse, extramarital sex etc. • Media should act in a positive and critical way to address different aspects of the adolescent behavior, social norms, attitude and environment. • Government can enact and enforce specific laws and policies to promote adolescent health programs

REFERENCES 1. Tiwari SK. Juvenile delinquency. In: Gupte S (ed): Recent Advances in Pediatrics (Special Vol.17: Adolescence). New Delhi: Jaypee Brothers 2006:305-315. 2. Gupte S. Adolescence. In: Gupte S (ed): The Short Textbook of Pediatrics, 9th edn. New Delhi: Jaypee Brothers 2004. 3. Ghai OP. Adolescence. In: Ghai OP, Gupta P, Paul VK (eds): Ghai’s Essential Pediatrics 5th edn. New Delhi: Interprint 2000: 41-46. 4. Aric V, Marcell and Charles E, Irwin Jr. Risk taking behaviors. In Rudolph AM, Rudolph CD, Hostetter MK, Lister G, Siegel NJ (eds): Rudolph’s Pediatrics 21st edn. New York: McGraw-Hill 2002:226-231. 5. The Global Youth Tobacco Survey Collaborative Group. Tobacco use among youth: A cross-country comparison. Tobacco Control 2002;11:252-270. 6. Sawhney A. Superbrat syndrome. The Times of India 2006; 11th June. Mumbai p.11 (Col.3-8). 7. Sethi GR. Street children: A window to the reality! (Editorial). Indian Pediatr 2004; 41:219-220. 8. Walayat N, Yadeo S. Adolescent risk-taking behavior: Indian perspectives. In: Bhave SY (ed): Course Manual for Adolescent Health, Part II. New Delhi: IAP 2002;385-391. 9. Let’s talk about teen sex. The Times of India, Mumbai 2006; 21st May. p.15 (Col.1- 2). 10. Nair MKC. Conducting question answer session. In: Pejaver RK, (ed): Adolescent Care 2000 and Beyond. Bangalore: Prism Books 2001:e 224-244. 11. UNICEF. A World Fit for Children. New York: UNICEF 2002. RAP Special Volume 19: Developmental and Behavioral Pediatrics 22 Disruptive Behavior Disorders

Sapna Gill, Nitin Gupta

INTRODUCTION Conduct disorder (CD) is probably the most common psychiatric childhood disorder.1 It is also the most frequent reason for referral to child and adolescent mental health facilities. CD and oppositional defiant disorder (ODD) are classified under the broad rubric of disruptive behavior disorders (DBD). Many children and adolescents can display aggression, disobedience and rebellion at various stages in their life. However, if such behaviors tend to persist, they assume pathological dimensions. Yet, for some clinicians, DBD can be seen as not being traditionally medical, but more of a psychosocial problem. Nevertheless, it is important to be able to have some understanding about etiology and management of such ‘out- of-control’ children for psychiatrists and pediatricians. This chapter shall attempt to provide an overview of these issues at hand, and hopefully help the reader gain insight into this complex and common clinical issue.

HISTORY Understanding about CD goes back to as early as the 1930s. This was primarily based on work by clinicians from the USA and UK. Psychoanalytic movement contributed to further development of its understanding, but were restricted by the lack of systematic evaluation of such an approach.2 Cleckley’s 3 influential monograph provided detailed case descriptions and cardinal features of psychopathy which could be linked with such behaviors in childhood. Some examples of the cardinal features listed by Cleckley3 were absence of responsibility/regret/shame, refusal to accept blame, lying and cheating, chaotic life-style, excessive 272 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics alcohol use, lack of insight, etc. Later, Bowlby4 gave the concept of ‘affectionless character’. Probably, the most important contribution came from the work by Robins5 who provided a reasonably convincing link between childhood conduct problems and psychopathology in adults, i.e. antisocial personality disorder. A major influence on the understanding of DBDs has been the involvement of law and courts. In summary, the law defines as deviant not only children who break the law set for adults, but also those who break any of a separate set of rules for the behavior of children.6 However, it was only in the 1960s that the medical model of illness was applied to DBDs. Both major classificatory systems, i.e. ICD and DSM included CD in their eighth and second editions/versions respectively. Probably, more focus on DBDs has been provided in the DSM classificatory system over the years. During the course of the gradual evolution of the DSM classificatory system, the most influential and major change occurred in the DSM-IV. In DSM-IV.7 Attention deficit hyperactivity disorder (ADHD) was separated from the DBD group based on the primary differentiating factor of ‘disruptive’ as it was felt that including children with ADHD and no disruptive behaviors would be incorrect.8 Additionally, the subcategories of CD were modified and reduced in DSM- IV, but no major changes were made to ODD. Hence, DBD as a group has been subjected to frequent and major changes; probably still to be an ongoing process.8

THE CONCEPT OF “AGGRESSION” As one talks about DBD, the single term that probably stands out is- ‘disruption’. Within the concept, definitions, and criteria (as can be seen later on) of CD, aggression is an important and key theme. Hence, it is pertinent to mention briefly about the concept, types, and relevance of aggression here. Aggression in humans tends to be controlled by developmental and social stimuli, and many aggressive behaviors may be acceptable depending upon the context or circumstances in which they are carried out.6 Problems tend to occur when children fail to apply, or to learn, about the appropriateness of aggressive behavior according to the circumstances.6 Nowadays, aggressive and antisocial behavior are often synonymous or aggregated as a single concept. Antisocial behavior can be defined as- Disruptive Behavior Disorders 273 behavior by which people are disadvantaged and basic norms and values are violated.9 Aggression is defined popularly as- behavior deliberately aimed at harming people and/or objects.10 Although this is more implicitly aimed at physical hurt, other form of harms, i.e. psychological harm, is equally important.9 Aggression in children is conceptualized differently from that in adults. In children, there are different classifications proposed for aggressive behavior. But, probably the most common dichotomy used is-‘reactive’ (response to a perceived threat or provocation) versus ‘proactive’ (behavior that anticipates a reward).11 Although both can be distinguished easily at a theoretical level, behavioral distinction is not that easy. Attempts have been made through direct observations and use of questionnaires, but more research is needed to reliably differentiate between these subtypes.9 Unfortunately, studies have been mainly carried out in the general population, rather than in a clinical population of children with aggressive behavioral disorder.9,12 To summarize, this current sub-typing does not overlap or match up with the clinical distinction of DBD into CD and ODD. Nevertheless, an in- depth understanding of aggression is still pertinent and necessary to further our understanding about DBDs.

CLINICAL FEATURES AND CLASSIFICATION We shall provide an overview of the clinical presentation in brief, and discuss about the criteria as used in the two major classificatory systems across the world, i.e. DSM-IV7 and ICD-10.13

Oppositional Defiant Disorder (ODD) Clinical Features Children with ODD are typically diagnosed between 6-10 years of age; juveniles with ODD having persistent and intentional antagonizing behavior and blaming others for their mistakes.14 Adolescents with ODD present with a persistent pattern of hostility towards authority; manifesting in the form of ‘defiant behavior’. This misbehavior can also be in the form of temper tantrums or episodes of rage. Though not easily angered adolescents with ODD hold grudges easily and are vindictive. Most common places for manifestation of ODD behavior is in familiar surroundings like home and school.14 Overall, ODD is rarely diagnosed before 3 years of age and generally does not persist beyond adolescence.14 274 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

The DSM-IV specifies the following criteria, i.e. Criterion A: There should be presence of at least four of the following symptoms: • Loses temper • Argues with adults • Actively denies or refuses adult requests • Deliberately does things that annoy other people • Blames others for his or her mistakes or misbehavior • Is touchy or easily annoyed by others • Is angry and resentful • Is spiteful and vindictive. Criterion B: The symptoms should have been present for at least 6 months.

Criterion C: This disturbance in behavior causes clinically significant impairment in social, academic, and occupational functioning. The ICD-10 lists the essential feature of ODD as a pattern of persistently negativistic, hostile, defiant, provocative and disruptive behavior which is clearly outside the normal range of behavior for a child of same age in the same sociocultural context. But these do not include the more serious violations of the rights of others or law as listed under the criteria for CD. The clinical features are similar to those outlined in the DSM-IV. The behavior is most evident in interactions with adults/peers known well to the child, and such signs may not be seen during a clinical interview. The symptoms need to be present for at least 6 months.

Comparison of ICD-10 and DSM-IV In the DSM-IV, ODD has to be excluded if CD is present. This reflects probably that ODD is a milder version of CD.2 In ICD-10, ODD is viewed as a subtype of CD, thereby ODD being seen as a precursor for CD later on in childhood/adolescence.15

Conduct Disorder (CD) Clinical Features CD can arise in either childhood (commonly before 10 years of age) or in adolescence (between 10-17 years of age). Typical features of childhood CD include temper tantrums, hitting, kicking people, aggression, property Disruptive Behavior Disorders 275 destruction (deliberately breaking things, setting fires), disobeying rules, lying, stealing and poor peer relationships. In adolescence it is characterized by bullying, skipping school, shop lifting, intimidation of others, frequent fighting, and sometimes using a knife, cruelty to people or animals, mugging, extensive drug use, running away from home and arson. This severe, persistent, recurring behavioral pattern violates the basic rights of others and defies societal norms and rules.14 Adolescents with CD have low self-esteem, show marked misery, unhappiness and are isolated. The DSM-IV specifies 3 criteria, i.e.

Criterion A: The basic rights of others or major age appropriate societal norms or rules are violated as evident from the following clinical features. There should be presence of at least three symptoms in the previous 6 months. Aggression • Often bullies, threatens or intimidates others • Often initiates physical fights • Has used a weapon that can cause serious physical harm to others • Has been physically cruel to people • Has been physically cruel to animals • Has stolen while confronting a victim • Has forced someone into sexual activity. Destruction of Property • Has deliberately engaged in fire-setting with the intention of causing serious damage • Has deliberately destroyed others’ property (other than fire-setting). Deceitfulness or Theft • Has broken into someone else’s house, building or car • Often lies to obtain goods or favors or to avoid obligations (i.e. ‘cons’ others) • Has stolen items of nontrivial value without confronting a victim (e.g. shoplifting). Serious Violation of Rules • Often stays out at night despite parental prohibitions, beginning before the age of 13 years 276 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Has run away from home overnight at least twice while living in the parental home or parental surrogate home (or once without returning for a lengthy period) • Often truants from school, beginning before age 13 years.

Criterion B: This disturbance in behavior causes clinically significant impairment in social, academic, and occupational functioning

Criterion C: In older individuals, the criteria for antisocial personality disorder are not met. CD can be classified on the basis of severity into mild, moderate, and severe types. The ICD-10 defines CD as being characterized by a repetitive and persistent pattern of dissocial, aggressive, or defiant conduct. This extreme behavior should amount to major violations of age-appropriate social expectations. Isolated dissocial/criminal acts do not amount to contributing towards a diagnosis as there has to be an enduring pattern of behavior. The symptoms listed are quite similar to that provided under the DSM-IV criteria. The duration of overall behavior should have been present for at least 6 months.

Comparison of ICD-10 and DSM-IV DSM-IV classifies CD on the basis of age of onset, i.e. adolescent-onset and childhood-onset (after or before 10 years respectively). ICD-10 classifies into socialized and non-socialized subtypes (based on integration or lack of integration with the peer group). Hence, ICD places a higher priority on the ‘quality of socialization’ than the ‘severity of offence’. This probably links up with research showing a link between prognosis and ‘severity of offence’.2 It is important to consider the patient within a developmental framework and perspective, as what may be pathological at one age may be appropriate developmentally at another age.14 The diagnostic categories of DSM-IV and ICD-10 do take into consideration age-appropriateness and societal/legal norms. However, it has been highlighted that the comparative nosology of the categories of CD and ODD is still problematic, as the diagnoses fail to take into account the strong influence of environment on behavior and vice versa.14 Disruptive Behavior Disorders 277

EPIDEMIOLOGY CD is one of the most common disorders in both outpatient and inpatient settings.16 In the last decade, there has been an increase in its prevalence; reported rates varying from 1-15%.8 Rates tend to be higher for boys than girls by 3-5 times, though the gap narrows with increasing age,17 and more in urban areas (9% compared to reported rates of about 4% in rural population).18 There is a trend and general consensus that poverty and low socioeconomic status are commonly associated with CD.19, 20 The prevalence rates for ODD range from 2-16%. It is more common in boys; however, this difference tends to equalize out after puberty.8 Data on the prevalence of CD related problems in adolescents are limited, and it is said that the prevalence tends to decrease as a function of age and normal maturation process.21 Numerous methodological issues influence the available prevalence rates. Some of these are diagnostic criteria used, instrument for assessment, time frame for assessment, location of study sample, number of informants, etc.22 In a comprehensive review, Loeber et al 23 reported community prevalence rates for both CD and ODD. Prevalence by age: Loeber et al 23 concluded that no firm conclusion could be reached regarding the prevalence of CD and ODD as a function of age. However, there appeared to be an increasing prevalence of non-aggressive CD behaviors from childhood to adolescence24 with an increase of covert conduct problems,23 and serious forms of aggression.25 Hence, the relationship of prevalence with age appears quite complex. Prevalence by gender: It has been seen that the odds of CD were 3-4 times greater for boys than girls; however, these sex differences in disruptive behaviors do not emerge until after 6 years of age.23 Prevalence by socioeconomic status: Both ODD and CD are more prevalent in youths from families of low socioeconomic status.22 However, it was also concluded that the current data on possible differences in the prevalence of CD and ODD in rural and urban settings is mixed and not well delineated.22 It will be pertinent to mention an important observation here that there is much less information and data available on the prevalence and correlates of CD and ODD from developing countries.2 Some authors think 278 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics that prevalence and manifestations of CD may vary in developing countries due to cultural differences of family cohesion and child-rearing methods. 26, 27

COMORBIDITY The majority of work related to comorbidity is available on CD rather on ODD. However, there was a community-based study that reported presence of comorbid depression (9%), anxiety disorder (14%), and ADHD (14%).28 Research has shown considerable evidence to suggest an increased risk of other disorders with CD in childhood. 29 Several disorders that are commonly associated are—ADHD, substance abuse, mood disorders, anxiety disorders, somatoform disorders, etc.

ADHD This is probably the most studied and the most complex comorbid condition associated with CD. Longitudinal studies show that children with ADHD display increased levels of antisocial behavior during adolescence and adulthood.30 ADHD has been found to influence the development, course and severity of CD.29 Children with CD and comorbid ADHD have an earlier age of onset of CD, and display more physical aggression and persistent features of CD.31, 32

Learning Difficulties Deficits in verbal skills are a well-recognized phenomenon,33 though it is not clear whether they act as key precursors to developing CD.34 Specific language impairment is associated with impaired social cognition (i.e. inability to understand other’s humor/sarcasm/intentions, etc.),15 and may lead onto unsocialized CD.35 Learning disorders can coexist in 50-70% of CD children.8 Children with CD experience academic difficulties (including suspension) and low self-esteem.8

Substance Abuse There is a strong and reciprocal relationship between CD and substance abuse, with each disorder increasing the manifestation of the other.29,36 Disruptive Behavior Disorders 279

Mood Disorders Depression and CD coexist to a great degree; especially depressive symptoms.29 CD can be a precursor to depression in childhood.37 Such children are at a high risk of substance abuse and suicide.38 Adolescents are prone to bipolar patterns. The chronology of development of mood disorders and CD is not very clear, and sub-syndromal symptoms can be present in childhood only.29

Anxiety The interplay and association of anxiety disorders with CD is also complex.29 Childhood anxiety disorders tend to protect against future antisocial behavior when they occur alone, but adolescents who develop CD are at risk for developing anxiety disorders.29,39

Somatoform Disorders There are only a few studies available, but they tend to show some link between these two disorders.29,40

ETIOLOGY There is a considerable overlap of research carried out on CD, ODD, delinquency, and violence.29 Hence, the discussion shall be based on looking at the overall construct of DBDs rather then either CD or ODD. The etiology of DBDs (especially CD) cannot be isolated to a single factor, but can be attributed to the interaction among these factors. For the sake of convenience they have been categorized in keeping with the framework suggested by Burke et al 29 as follows:

Biological Factors Genetics and Family Genetic effects are inferred from twin studies wherein monozygotic twins (MZ) are more similar than dizygotic twins (DZ). Twin concordance rates for adolescent delinquency have been reported to be 87% for MZ twins and 72% for DZ twins.41 Adoption studies suggest that genetically vulnerable children, whose parents were antisocial, may be especially susceptible to unfavorable family conditions.42 Genetic elements are reported to be stronger for adulthood criminality than childhood CD.43 280 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Pike et al44 found evidence that genetic factors primarily explained the association between familial negativity and adolescent antisocial behavior. Evaes et al 45 reported a high genetic correlation in twins, which suggested a common underlying condition. Although a lot of twin and adoption studies on antisocial behavior have been published, it is difficult to draw clear conclusions regarding the magnitude of genetic and environmental influences. A history of parental antisocial behavior is associated with a preadolescent onset of CD.46 Also, a strong linkage has been seen between mother and daughter antisocial behavior, more likely arising out of parental psychological distress than parenting behaviors.47

Neuroanatomy and Neuropsychology Reviews of research on children and adolescents suggest that antisocial behavior is characterized by neuropsychological impairments. 48, 49 Some researchers have put forward a case for antisocial and delinquent children having specific deficits in executive functions.50 Executive dysfunction is hypothesized to relate to antisocial and aggressive behavior by decreasing behavioral inhibition and impairing one’s ability to generate socially acceptable responses in challenging situations.51 Frontal lobe damage has been associated with aggression and CD.49,51-54 Davidson55 reported that impairment in connections between the amygdala and prefrontal cortical regions serves to aid in the suppression of negative emotions. Hence, frontal lobe damage can lead to non-suppression of negative emotions. All these findings do suggest an association between brain structures and antisocial behaviors, but further research is needed for replication of available results.

Neurotransmitters and Neurochemicals Considerable research has gone into studying neurotransmitters, mainly serotonin.29 Low levels of serotonin metabolite (5-hydroxyindoleacetic acid) in cerebrospinal fluid have been linked to concurrent56 and future57 aggression. However, there is no evidence to suggest a link of serotonin dysfunction with non-aggressive DBD symptoms.29 Serotonin function has also been linked to the regulation of mood and impulsive behavior.55 Low salivary cortisol levels are associated with ODD.58 Salivary cortisol levels have been negatively associated with both CD and parental antisocial Disruptive Behavior Disorders 281 personality disorder (APD).59 In childhood onset of CD, testosterone has been associated with aggression.54

Under Arousal of the Autonomic Nervous System Studies show that general physiological under arousal and lower heart rate are associated with ODD and CD.54 This has been seen with both boys and girls.60 Also, lower skin conductance is found in boys with disruptive behaviors.61

Others Prenatal and perinatal problems including low birth weight, 62 maternal poor nutrition, viral infections and delivery complications have also been linked to DBD and aggression,29 Prenatal exposure to teratogens, i.e. maternal cigarette smoking63 and substance abuse64 are seen to be correlated with aggression. A study by Needleman et al 65 found high levels of lead in bones of children scoring high on aggression and somatic complaints.

Functional/Individual Factors Temperament and Attachment Dysregulated temperament may facilitate the progression from early disruptive problems to CD.66 A review by Sanson and Prior 67 concluded that early temperamental traits of inflexibility, negative emotionality, and intense responses predicts externalizing behavioral problems (like DBDs) by late childhood. However, boys and girls can differ temperamentally, and further research is needed for determining such gender-specific relationship of temperament and DBD.29,67 The overall findings regarding attachment styles do not show a clear definitive relationship, and further research is warranted.29

Reading Problems Reading disorders are related to CD- they being predictive of teenage disruptive behaviors in girls.68 In boys, disruptive behavior is a risk for later reading problems, but not vice versa.69 282 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Impulsivity and Behavioral Inhibition Impulsivity has been associated with both early onset70 and presence of antisocial behavior.71 Kerr et al 72 reported that socially withdrawn boys were at greatest risk for delinquency.

Social Cognition Numerous studies have reported significant correlation between one or more social information processing patterns and measures of aggressive behavior.73, 74 Aggressive behavior has been found to be correlated with various aspects of children’s encoding of social cues, including selective attention to relevant cues,75 total attention cues,76 and recall of hostile cues.75 Aggressive behaviors have also been correlated with hostile attribution biases concerning peer’s intent,75,77 with attributions of anger in teachers,78 accuracy of interpretations of others’ intent75 and social problem solving patterns including the overall number of solutions that they are able to generate.79 Aggressive behavior has also been linked with children’s response-decision processes, including their evaluation of the consequences of aggressive behavior as being favorable,80 their judgements of aggressive responses as being good,81 their perceptions of suffering that victims experience from aggression82 and their positive valuing of the outcomes that occur to aggressive behavior.83 Children with CD are lower in empathy and identification of interpersonal cues.84

Puberty and Adolescent Development Early pubertal onset has been associated with increased problem behaviors in girls but not in boys.29 Williams and Dunlop85 have suggested that either early or late onset of puberty is associated with deviant social behaviors.

Psychosocial Factors Child Rearing Styles/Parenting Numerous aspects of parenting style, such as poor supervision, erratic and harsh discipline, parental dysharmony, child rejection, low involvement, parent-child conflict management have been associated with children’s disruptive or delinquent behavior.86-88 Disruptive Behavior Disorders 283

Parent-child Interaction Children model the behavior of the adults around them. The way a parent behaves towards a child, the way he/she handles the problems arising between them is very important. There is an association between aggressive behavior in children and the way their parents behave towards them.1 The family’s behavior, whether it is positive or negative, is easily adapted by the child. Parental rejection, negligence and ignorance of the child further contribute to the child’s disturbance.

Peer Rejection and Peer Effects Children spend considerable time with their peers and lay a lot of importance on their opinions. Peer rejection is one of the major predictors of later growth in aggressive behavior; though the question is still debatable whether it is a causal factor or just a marker. Peer rejection typically ensues when children due to their lack of participation withdraw, become aggressive and then associate with other aggressive children who share similar set of values.89 Dodge et al90 hypothesized two ways that rejection may lead to growth in aggressive behavior. Firstly, being rejected by the peers denies a child important opportunity for social growth and the development of socio-cognitive skills. Certain researchers91-93 lay importance to the development of these skills in free exchange with an accepting peer group. A child who does not have an access to this group has difficulty in developing these skills; thereby when confronted with a socially challenging situation that child is more likely to resort to aggressive behavior. Secondly, peer rejection can have stressful effect on children. Rejecting peers can be mean and cruel leading the child to be socially isolated, lonely and angry. Exposure73 and association94-96 with delinquent peers can initiate and enhance preexisting delinquent behavior.

Child Maltreatment/Child Abuse Review of literature shows that the experience of physical maltreatment in early life to be a major predictor of conduct problems in adolescent, whether measured by retrospective accounts from adolescents96 or by prospective follow-ups of children identified by social services as “maltreated”. 97 Harsh or abusive parenting behaviors increase the risk of 284 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

CDs in children. The effect of child abuse depends on its severity, duration, frequency, relationship with the victim and the perpet,rator, etc. but there is a need of more research in this area.

Socioeconomic Factors Research has shown that poor and disadvantaged neighborhoods98 where there is an exposure to poverty, drugs, adult criminals, disorganization99 violence and racial prejudice100 are associated with disruptive behaviors.

Predisposing Child Characteristics. The interplay between nature and nurture are inseparable. In infants the nurture has an upper hand and as one grows up the interaction between nurture and nature takes a stronghold. Exposure to stressful life events101 and maladaptive coping strategies102 are correlated with behavior problems especially in children who have predisposed personality characteristics such as hyperactivity, irritability, explosiveness, lack of social awareness, social anxiety and a slightly lo,w IQ, i.e. 8-10 points lower than law abiding peers. 103 To conclude and reiterate, no single factor results in DBD but it is the combination of numerous factors which increases the likelihood for the disorder.

ASSESSMENT The diagnostician needs to be very objective while making an assessment in order to provide better basis of prediction and further referrals. The success or failure of assessment is linked with the quality of the assessor’s judgement during the evaluation process, as the assessment is the objective extension of the initial clinical impression. The assessment of ODD and CD should be made keeping in mind the diagnostic criteria (as laid down in either DSM-IV or ICD-10) and the age of onset. One should take a thorough history with additional supplementary information from other sources, i.e. parents, teachers, or social/legal authorities. There is a need to conduct a detailed physical (including neurological) examination. Special attention needs to be paid to ruling out neurological disorders, endocranial disorders, and substance abuse (by simple urine drug screens). One needs to assess the individual for any Disruptive Behavior Disorders 285 underlying/associated psychopathology, as sometimes treating the underlying/associated psychopathology helps in controlling/treating DBDs.14 If there is a sudden increase in the severity of behaviors and symptoms, a detailed risk assessment needs to be done to determine the potential intent for harm to self or others, and also to determine whether hospitalization is necessary for ensuring safety.104 Where there is a strong suspicion of comorbidity, additional referrals to concerned specialists (e.g. psychologist, speech therapist, audiologist, endocrinologist, neurologist, etc.) should always be sought.8 Along with self-report scales for such children, it has been seen that including reporting scales for family members and teachers adds value and makes the assessment quite comprehensive.14 These rating scales and standardized measures help in assessing and determining-severity of the DBDs, nature of aggression, confirmation of diagnosis, concomitant psychological/temperamental factors, and presence of comorbid psychiatric illnesses that may antedate or coexist with DBDs. There are several standardized diagnostic interviews, rating scales, and projective techniques available. Some of the common ones are—NIMH Diagnostic Interview Schedule for Children-Version IV,105 Child and Adolescent Psychiatric Assessment,106 Diagnostic Interview for Children and Adolescents,107 Hare’s Psychopathology Checklist,108 Pictorial Instrument for Children and Adolescents-III-R,109 Dominic-R,110 etc. Observation in their natural setting should also be used, in which the therapist leaves his/her interview rooms and goes to the natural environment where the child lives. Field observations with well designed coding systems have provided evidence that antisocial children do not simply march to their own rhythms. Rather, much of their behavior is in direct response to the stimuli and reinforcing contingencies provided by the behave of others. Parents, siblings, peers, teachers and significant others inadvertently elicit and maintain the very problem behaviors they find repugnant. Several scoring systems have been developed in a variety of setting such as home, school, family, problem solving discussions, treatment session’s, etc. It is an important measure of treatment outcome. To summarize the assessment should be based on assessing the externalizing problems, the internalizing problems, prosocial skills and cognitive skills. Any single method or source of assessment can potentially be susceptible to inherent biases and distortions. Diversity of relevant and 286 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics accurate measures that tap the essential behaviors related to arriving at a diagnosis of DBDs is required. Hence, there is a need for development of a multi-method approach. It can be seen the evaluation for DBDs (ODD and CD) is not only a time-consuming, detailed process, but can be reasonably complicated if an attempt is made at a comprehensive assessment.

TREATMENT There have been numerous treatment approaches adopted with not very heartening outcome. The results probably also vary due to the type of setting in which the intervention is carried out. There is not definite evidence to suggest that interventions in individual (one-to-one) setting are superior to the group setting. We are outlining the most commonly used and reasonably effective treatment strategies used for managing DBDs. For a more in-depth, detailed discussion on treatment modalities the reader is advised to refer to reviews by Kazdin,111 Scott,1 Burke et al,29 Hengegler and Sheidow.112

MULTISYSTEMIC THERAPY (MST) This is based on the premise that no single treatment approach is effective for DBD. In MST, a comprehensive multidisciplinary team plan and approach is developed to meet specific needs for each case. Implementation of a treatment plan includes various groups of people that are associated with the patient and in different physical settings like the school, home, physician and the therapist’s office. The goal of the treatment plan should be directed towards addressing various psychosocial aspects related to the DBD. These would include-alleviation of poverty, improved housing and employment, improvement of educational and recreational facilities, strengthening of community agencies, individually applied behavioral methods which are community based, social support for the child and his family, positive therapist qualities which suggest appropriate but high expectations, understanding of delinquent characteristics for the appropriate choice of therapy. As children with DBD commonly come to notice via the parents or/and the school, it is equally important that additional attention should be provided by the treating team to management of the child in the school setting. The therapists place emphasis on developing links with schools. The intervention in school Disruptive Behavior Disorders 287 setting should be made considering learning disorders, special education needs, reducing bullying, group composition, playground behavior, systematic communication between parents and teachers, peer rejection, decreasing the disruptive behavior, resolving the peer conflict in a non- aggressive way. Approaches to intervention in the school setting may include behavior management techniques, social skill training, emotional control strategy and cognitive or emotional perspective taking. There is also a role for use of pharmacological treatment in MST. Efficacy has been demonstrated for severely disturbed children with CD.113

PARENT MANAGEMENT TRAINING (PMT) Studies111,114 suggest PMT to be a successful intervention strategy in DBD and are associated with improvement across setting. Parents have a sustained impact on their children’s adjustment.115 Review of literature shows that parents of antisocial youngsters have serious deficits in parenting practices. Therefore, it is necessary to reprogram the child’s social environment by teaching parents effective family management skills. The Oregon Social Learning Centre (OSLC) treatment program is based on teaching parents a set of specific parenting practices and has been described as a successful intervention in various studies.116, 117 The treatment employs a step-by-step approach, i.e. each skill learned forms the base upon which a new skill can be laid. Five management practices, i.e. tracking, positive reinforcement, discipline, monitoring and problem solving are the core of OSLC parent training program. First, the parent learns to track or pay attention to specific child behaviors, e.g. compliance versus non compliance. Based on this the parent applies the contingent consequences whether positive or negative. When parents see the child doing something praiseworthy, e.g. complying with a parental request they learn to respond with positive reinforcement, e.g. approval, smile, increase the privileges, etc. When parents see their children behaving inappropriately, e.g. non complying they then resort to mild consequences, e.g. time out or short term privilege removal, i.e. no watching of TV or playing a video game. This is ‘discipline’. In monitoring, the parents provide close supervision for their children even when their children are away from home. This involves knowing where the child is, what is he/she doing while there, with whom is he, when is he coming home, etc. Finally, the problem solving strategy helps 288 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics them to plan ahead to prevent problems before they arise and to manage difficulties in a rational manner. Kazdin 111 concluded that the PMT is the best-documented treatment and it offers the most promising procedure for the antisocial behavior.

COGNITIVE BEHAVIORAL TREATMENT (CBT) This approach includes in it an amalgam of cognitive, behavioral, emotive and developmental strategies. The CBT model focuses on management of contingencies (reward and response cost, i.e. children also learn that a cost has to be paid for misbehavior), modelling, role-playing, affective education (i.e. helping children to develop an understanding about their emotions and feelings, so that they are able to relate in a positive fashion with events), homework, self-evaluation and perspective–taking activities.118 Another important part of this therapy is to make an outline of rules and their consequences, and being consistent with them. When the rules are broken and contingencies put forward to the child, it is very valuable to prepare the child’s expectations for these contingent outcomes so that they can experience and process the event rationally and hold reasonable expectations for later contingent consequences. In effective modelling, the therapist takes a role of a coping model, i.e. he does not always display socially appropriate behaviors but makes mistakes like those seen in the child’s repertoire and models a strategy for correcting the misbehavior. He also, at times, models the strategy of catching the act before it is completed and at times be a problem solver model, i.e. he does not have a ready made answer to the situations and proposes different solutions, examines them on the bases of their consequences and then utilizes them. In role playing, the child is presented with different social situations that provide him an opportunity to exercise his newly learnt skills. It is here the therapist assess and modifies the child’s cognitive processing errors. In the perspective-taking tasks, the child plays one role and the therapist the other following the discussion of how each person processed the experience and then the roles are reversed. So, the child has now both the experiences and examines the situations from both the perspectives. Disruptive Behavior Disorders 289

As children are not very clear of their feeling and emotions various projective techniques should be used to assess their emotional states. Identifying these emotional states in themselves and others will help the child empathize and take others perspective. Using the self evaluation exercises, the child is taught to stop and re-examine his activities and consider how various people in the situation felt and thought about it. This would further enhance the perspective-taking. Homework here refers to doing things when not in therapy. Show that I Can (STIC) 119 are activities that allow the child to do an activity outside therapy and report back to the therapist to earn rewards. This therapy approximately runs for 20 weeks. It is highly effective for mild to moderate aggressive problems but more research is needed for its relevance in severely disturbed DBD children.

SOCIAL SKILL TRAINING (SST) Research has laid the importance of the social cognition in the aggressive behavior and suggests that these children have biases and deficits in their social problem solving leading them to exhibit aggressive behavior. Dodge120 proposed five cognitive operations that formed the basis of his social information model of competent social responding related to aggressive behavior. The first step is encoding of social cues in the environment by selecting, attending to and storing relevant social information. The main emphasis here is to help a child learn to attend to the person and environment while taking in the information. To simplify the child is taught to know his feelings and looking into the environment and try to identify the cues which suggest these feelings and their cause, e.g. he feels the other person is angry by looking at his frowning gesture. Further he is taught to look and listen to the person he is interacting with and think what he is saying. The interpretation process involves in it interpreting the encoded cues keeping in mind the past experiences and attended cues correctly. The response research includes in it divergent thinking, i.e. to think of a number of different ways to generate possible behavioral responses to the situation. Response decision includes in it considering the possible consequences of the responses generated in the previous step and choosing a response that fits the situation. The last step involves the enactment of the selected response as a result of the earlier operations which they learn through problem solving, role playing, self- 290 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics control skill, and behavior rehearsal. In addition to these steps, “evaluation”, was suggested by other researchers.121-123 This involves considering the consequences of the chosen response keeping in mind self and others. This approach has been beneficial in developing programs that will improve the behavior problems at home, school, and with their peers. Research has laid the importance of social cognition in the aggressive behavior and suggests that these children have biases and deficits in their social problem solving leading them to exhibit aggressive behavior. Dodge proposed five cognitive operations that formed the basis of his social information model of competent social responding related to aggressive behavior.The first step is encoding of social cues in the environment by selecting, attending to and storing relevant social information, e.g. facial expression to gather the information on intent. The interpretation process involves in it integrating the encoded cues in the light of past experiences and attended cues.The response research includes in it divergent thinking to generate possible behavioral responses to the situation. Response decision includes in it considering the possible consequences of the responses generated in the previous step and choosing a response that fits the situation. The last step involves the enactment of the selected response as a result of the earlier operations. In addition to these steps, “evaluation”, was suggested by others (Novaco, Perry et al, Rubin and Krasnor). This involves considering the consequences of the chosen response keeping in mind self and others. This approach has been beneficial in developing programs that will improve the behavior problems at home, school, and with their peers.

PHARMACOLOGICAL TREATMENT Psychopharmacological treatment of symptoms of CD has been a relatively neglected area.2 It can be safely said that CD as a disorder cannot be managed by medicines. However, symptoms of CD can be addressed to by use of medications; even though the benefits may be only partial.29 The symptoms that require looking at use of medicines are—aggression, explosive temper, destruction of property, impulsivity.2 However, the database is not very large or promising as only a few randomized controlled trials have been carried out.2,29 Amongst the psychotropics- lithium, antipsychotics, and stimulants may be useful in treating symptoms of CD.29 However, great caution should be exercised in the use of stimulants Disruptive Behavior Disorders 291 as children with CD are at high risk for developing comorbid substance abuse; stimulants being preferred when ADHD coexists with CD.

COURSE AND OUTCOME ODD can be a precursor of CD, though many children with ODD do not develop CD.8 ODD tends to remit with age.14 CD tends to be a stable diagnosis, especially when presenting in adolescence.14 Around 30-50% of children with CD go onto develop antisocial personality disorder (ASPD); this being less common in adolescent-onset CD.124 40% of 7-8 year old children with CD become recidivist delinquents as teenagers.1 Psychiatric disorders as outcomes of CD can be—alcohol and drug dependence, and ASPD.125 Non-psychiatric antisocial behaviors include –theft, violence to people and property, drunk driving, vandalism, group violence, unemployment, marital violence, divorce, and abuse of children.1 Early onset CD runs a more severe course and is associated with a poorer outcome. Gender plays an important role; with early-onset CD in males having a poorer prognosis.2

INDIAN DATA There is a surprising paucity of focus on Indian studies from psychiatrists, psychologists, and pediatricians alike. Although there have been prevalence studies carried out in children in community and schools, we could come across only one study126 which reported a 11.1% prevalence rate of CD in the school children studied. Some phenomenological work assessing temperamental characteristics of children with CD has also been reported.127,128 Work has also been done in determining a model for being able to predict and differentiate internalizing/emotional disorders from externalizing disorders like CD, ADHD, etc.129-131 In terms of etiological link of stressful life events with CD, Malhotra et al (1992)132 found comparable number of life events and stress scores between hyperkinetic/ CD children and those with neurotic and emotional disorders. Attempt has also been made to examine the subclassification of CD and hyperactive CD (HCD) in a retrospective, comparative clinic-based study of children with CD and HCD. It was suggested that there were some differences in the etiopathogenesis and comorbidity of CD compared to HCD.133 292 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

KEY LEARNING POINTS • Disruptive behavior disorders are conduct disorder and oppositional defiant disorder. • Aggression, defined as behavior deliberately aimed at harming people and/or objects, is the most common component of disruptive disorders. • Aggression and antisocial behavior are currently often considered as a single concept. • Comorbidity of disruptive behavior, especially conduct disorder, include several disorders such as ADHD, substance abuse, mood disorders, anxiety disorders, somatoform disorders etc • Assessment should be made keeping in mind the diagnostic criteria (as laid down in either DSM-IV or ICD-10) and the age of onset. • Interventions in individual (one-to-one) setting are superior to the group setting. • Treatment strategies include multisystem approach, parent management training, cognitive behavior treatment, social skill traing and pharmacolotherapy. • Amongst the psychotropics- lithium, antipsychotics, and stimulants may be useful in treating symptoms such as aggression, explosive temper, destruction of property, impulsivity. • ODD tends to remit with age whereas CD tends to be a stable diagnosis, especially when presenting in adolescence.. • Pediatricians should keep a high index of suspicion for identifying children with potential or possible DBD and, subsequently, help parents and teachers in their management, ensuring appropriate and timely referral to psychiatrists and psychologists as and when needed.

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46. Lahey BB, Loeber R, Quay HC, et al. Validity of DSM-IV subtypes of conduct disorder based on age of onset. J Am Acad Child Adolesc Psychiatry 1998; 37: 435- 442. 47. Kaplan HB, Liu X. Explaining transgenerational continuity in antisocial behaviour during early adolescence. In: Cohen P, Slomkowski C, Robins LN (eds): Historical and Geographical Influences on Psychopathology. Mahwah: Erlbaum 1999: 163-191. 48. Moffitt TE. The neuropsychological of conduct disorder. Dev Psychopathol 1993; 5: 135-151. 49. Raine A, Lencz T, Bihrle S, La Casse L, Colletti P. Reduced prefrontal grey matter volume and reduced autonomic activity in antisocial personality disorder. Arch Gen Psychiatry 2000; 57: 119-127. 50. Lueger RJ, Gill KJ. Frontal lobe cognitive dysfunction in conduct disorder adolescents. J Clin Psychology 1990; 46: 696-706. 51. Giancola PR. Evidence of dorsolateral and orbital prefrontal cortical involvement in the expression of aggressive behaviour. Aggressive Behav 1995; 21: 431-450. 52. Hux K, Bond V, Skinner S, Belau D, Sauger D. Parental report of occurrences and consequences of traumatic brain injury among delinquent and non- delinquent youth. Brain Injury 1998; 12: 667-681. 53. Max JE, Koele SL, Smith WL, et al. Psychiatric disorders in children and adolescents after severe traumatic brain injury: A controlled study. J Am Acad Child Adolesc Psychiatry 1998; 37: 832-840. 54. Plszaka SR. The psychobiology of oppositional defiant disorder and conduct disorder. In: Quay HC, Hogan AE (eds.): Handbook of Disruptive Behaviour Disorders. New York: Kluver Academic /Plenum, 1999: 371-395. 55. Davidson RJ, Putnam KM, Larson CL. Dysfunction in the neural circuitry of emotion regulation: A possible prelude to violence. Science 2000; 289: 591-594. 56. Kruesi MJP, Hibbs ED, Zahn TP, et al. A 2-year-prospective follow-up study of children and adolescents with disruptive disorders: Prediction by cerebrospinal fluid 5-hydroxyindoleacetic acid, homovanillic acid, and autonomic measures? Arch Gen Psychiatry 1992: 429-435. 57. Clarke RA, Murphy DL, Constantino JN. Serotonin and externalizing behaviour in young children. Psychiatry Res 1999; 86: 29-40. 58. van Goozen SHM, Matthys W, Cohen-Kettenis PT , Gispen-de Wied C, Wiegant VM, Engeland HV. Salivary cortisol and cardiovascular activity stress in oppositional defiant disorder boys and normal controls. Biol Psychiatry 1998; 43: 531-539. 59. Vanyukov MM, Moss HB, Plial JA, Blackson T, Mezzich AC, Tarter RE. Antisocial symptoms in preadolescent boys and in their parents: Associations with cortisol. Psychiatry Res 1993; 46: 9-17. 60. Rogerness GA, Cepeda C, Macedo CA, Fischer C, Harris R. Differences in heart rate and blood pressure in children with conduct disorder, major depression, and separation anxiety. Psychiatry Res 1990; 33: 199-206. 61. Harden PW, Pihl RO, Vitaro F, Gendreau PL, Tremblay RE. Stress response in anxious and nonanxious disruptive boys. J Emotional Behav Disord 1995; 3: 183- 190. 62. Raine A, Brennan P, Mednick SA. Interaction between birth complications and early maternal rejection in predisposing individuals to adult violence: Specificity to serious, early onset violence. Am J Psychiatry 1977; 154: 1265-1271. 63. Wakschlag LS, Lahey BB, Loeber R, Green SM, Gordon RA, Leventhal BL. Maternal smoking during pregnancy and risk of conduct disorder in boys. Arch Gen Psychiatry 1997; 54: 670-676. 296 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

64. Loukas A, Fitzgerald HE, Zucker RA,von Eye A. Parental alcoholism and co- occurring antisocial behaviour: Prospective relationships to externalizing behaviour problems in their young sons. J Abnorm Child Psychol 2001; 29: 91-106. 65. Needleman HL, Reiss JA, Tobin MJ, Biesecker GE, Greenhouse JB. Bone lead levels and delinquent behavior. JAMA 1996; 275: 363-369. 66. Kingston L, Prior M. The development of patterns of stable, transient, and school- age onset aggressive behavior in young children. J Am Acad Child Adolesc Psychiatry 1995; 34: 348-358. 67. Sanson A, Prior M. Temperament and behavioural precursors to oppositional defiant disorder and conduct disorder. In: Quay HC, Hogan AE (eds): Handbook of Disruptive Behavior Disorders. New York: Kluwer Academic/Plenum 1999: 397- 417. 68. Manghan B, Pickles A, Hagell A, Rutter M, Yule W. Reading problems and antisocial behaviour: Developmental trends in comorbidity. J Child Psychol Psychiatry 1996; 37: 405-418. 69. Sanson A, Prior M, Smart D. Reading disabilities with and without behaviour problems at 7-8 years: Prediction from longitudinal data from infancy to 6 years. J Child Psychol Psychiatry 1996; 37: 529-541. 70. Trembley RE, Phil RO, Vitaro F, Dobkin PL. Predicting early onset of male antisocial behaviour from preschool behaviour. Arch Gen Psychiatry 1994; 51: 732-739. 71. White JL, Moffit TE, Capsi A, Bartush DJ, Needles DJ, Stouthamer-Loeber M. Measuring impulsivity and examining its relationship to delinquency. J Abnorm Psychol 1994; 103: 192-205. 72. Kerr M, Tremblay RE, Pagani L, Vitaro F. Boys’ behavioural inhibition and the risk of later delinquency. Arch Gen Psychiatry 1997; 54: 809-816. 73. Coie JD, Dodge KA. The development of aggression and antisocial behaviour. In: Eisenberg N (ed.) Handbook of Child Psychology, Vol. 3: Social, Emotional and Personality Development. New York: Wiley 1998: 779-861. 74. Crick NR, Grotpeter JK. Relational aggression, gender and social psychological adjustment. Child Dev 1995; 66: 710-722. 75. Dodge KA, Pettit GS, Bates JE, Valente E. Social information processing patterns partially mediate the effect of early physical abuse on later conduct problems. J Abnorm Psychol 1995; 104: 632-643. 76. Slaby RG, Guerra NG. Cognitive mediators of aggression in adolescent offenders: I. Assessment. Dev Psychol 1988; 20: 580-588. 77. Milich R, Dodge KA. Social information processing patterns in child psychiatric populations. J Abnorm Child Psychol 1984; 12: 471-490. 78. Trachtenberg S, Viken RJ. Aggressive boys in the classroom: Biased attributions or shared perceptions? Child Develop 1994; 65: 829-835. 79. Rabiner DL, Lenhart L, Lochman JE. Autonomic versus reflective social problem solving in relation to children’s sociometric status. Develop Psychol 1990; 26: 1010- 1016. 80. Boldizar JP, Perry DG, Perry LC. Outcome values and aggression. Child Develop 1989; 60: 571-579. 81. Astor RA. Children’s moral reasoning about family and peer violence: The role of provocation and retribution. Child Develop 1999; 65: 1054-1067. 82. Perry DG, Williard JC, Perry LC. Peers’ perceptions of the consequences that victimized children provide aggressors. Child Develop 1990; 61: 1310-1325. 83. Fontaine RG, Burks VS, Dodge KA. Response decision process and externalizing behaviour problems in adolescents. Dev Psychopathol 2002; 14: 107-122. 84. Cohen D, Strayer J. Empathy in conduct disordered and comparison youth. Dev Psychol 1996; 32: 988-998. Disruptive Behavior Disorders 297

85. Williams JM, Dunlop LC. Pubertal timing and self-reported delinquency among male adolescents. J Adolesc 1999; 22: 157-171. 86. Frick PJ. Family dysfunction and the disruptive disorders: A review of recent empirical findings. In: Ollenduick TH, Prinz RJ (eds): Advances in Clinical Child Psychology. New York: Plenum 1994: 203-226. 87. Wasserman GA, Miller LS, Pinner E, Jaramilo B. Parenting predictors of early conduct problems in urban, high-risk boys. J Am Acad Child Adolesc Psychiatry 1996; 35: 1227-1236. 88. Farrington DP. A critical analysis of research on the development of antisocial behaviour from birth to adulthood. In: Stoff DM, Breiling J, Maser JD (eds): Handbook of Antisocial Behaviour. New York: Wiley 1997: 234-240. 89. Kupersmidt KB, Coie JD, Dodge KA. The role of poor peer relationships in the development of disorder. In: Asher SR, Coie JD (eds): Peer Rejection in Childhood. Cambridge: Cambridge University Press 1990. 90. Dodge KA, Lansford JE, Burks VS, et al. Peer rejection and social information processing factors in the development of aggressive behaviour problems in children. Child Develop 2003; 74: 374-393. 91. Piaget PJ. The Moral Judgement of the Child. New York: Free Press 1965. 92. Hartup WW. Peer relations. In: Mussen PH, Hertherington EM (eds): Handbook of Child Psychology, 4th edn. New York: Wiley 1983: 103-196. 93. Youniss J. Parents and Peers in Social Development. Chicago: University of Chicago Press 1980. 94. Elliot DS, Menard S. Delinquent friends and delinquent behaviour: Temporal and developmental patterns. In: Hawkins JD (ed): Delinquency and Crime: Current Theories. New York: Cambridge University Press 1996: 28-67. 95. Keenan K, Loeber R, Zhang Q, Stothamer-Leober M, van Kammen WB. The influence of deviant peers on the development of boys’ disruptive and delinquency: A temporal analysis. Dev Psychol 1995; 7: 715-726. 96. Simons RL, Wu C, Conger RD, Lorenz FO. Two routes to delinquency: Differences between early and later starters in the impact of parenting and deviant peers. In: Greenburg DF (ed): Criminal Careers. Brookfield, VT: Dartmouth 1996: 105- 133. 97. Widom CS. Does violence beget violence? A critical examination of the literature. Psychol Bull 1989; 106: 3-28. 98. Wikstrom PO, Leober R. Do disadvantaged neighbourhoods cause well adjusted children to become adolescents? A study of male juvenile serious offending, risk and protective factors, and neighbourhood content. Criminology 2000; 38:1109-1141. 99. Herrenkohl TI, Maguin E, Hill KG, Hawkins JD, Abbot RD, Catalano RF. Developmental risk factors for youth violence. J Adolesc Health 2000; 26: 176-186. 100. Hawkins JD, Herrenkohl T, Farrington DP, Brewer D, Catalano RF, Harachi TW. A review of prediction of youth violence. In: Loeber R, Farrington DP (eds): Serious and Violent Juvenile Offenders: Risk Factors and Successful Intervention. Thousands Oaks: Sage 1998: 107-146. 101. Mathijssen JJJP, Koot HM, Verhulst FC. Predicting change in problem behaviour from child and family characteristics and stress in referred children and adolescents. Dev Psychopathol 1999; 11: 305-320. 102. Hastings TL, Anderson SJ, Kelly ML. Gender differences in coping and daily stress in conduct-disordered and non-conduct-disordered adolescents. J Psychopathol Behav Assess 1996; 18: 213-226. 298 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

103. Moffitt TE, Brammer GL, Caspi A, et al. Whole body serotonin relates to violence in an epidemiological study. Biol Psychiatry 1998; 43: 446-457. 104. Steiner H. Practice parameters for the assessment and treatment of children and adolescents with conduct disorder. J Am Acad Child Adolesc Psychiatry 1997; 36 (Suppl.10): 122S-139S. 105. Shaffer D, Fisher P, Dulcan MK, et al. The NIMH Diagnostic Interview Schedule for Children version 2.3 (Disc 2.3): description acceptability, prevalence rates and performance in MECA study. J Am Acad Child Adolesc Psychiatry 1996; 35: 865-877. 106. Angold A, Costello EJ. The child adolescent psychiatric assessment. J Am Acad Child Adolesc Psychiatry 2000; 39: 39-48. 107. Reich W. Diagnostic Interview for Children and Adolescents (DICA). J Am Acad Child Adolesc Psychiatry 2000; 39: 59-66. 108. Hare RD, Clark D, Grann M, Thornton D. Psychopathology and predictive validity of PCL-R: An international perspective. Behav Sci Law 2000; 18: 623-645. 109. Ernst M, Cookus BA, Moranec BC. Pictorial instrument for children and adolescents (PICA-III-R). J Am Acad Child Adolesc Psychiatry 2000; 39: 94-99. 110. Valla J, Bergeron L, Smolla N. The Dominic-R: A pictorial interview for 6 to 11 year-old children. J Am Acad Child Adolesc Psychiatry 2000; 39: 85-93. 111. Kazdin AE. Parent management training: Evidence, outcomes and issues. J Am Acad Child Adolesc Psychiatry 1997; 36: 1349-1356. 112. Henggeler SW, Sheidow AJ. Conduct disorder and delinquency. J Marital Fam Ther 2003; 29: 505-522. 113. Henggeler SW, Rowland MD, Halliday-Boykins C, et al. One-year follow-up of multisystemic therapy as an alternative to the hospitalization of youths in psychiatric crisis. J Am Acad Child Adolesc Psychiatry 2003; 42: 543-551. 114. Breston EV, Eyberg SM. Effective psychosocial treatment of conduct disordered children and adolescents: 29 years, 82 studies and 5272 kids. J Clin Child Psychol 1998; 27: 180-189. 115. Patterson GR. A social learning approach to family intervention: III. Coercive family process. Eugene: Castalia 1982. 116. Forgatch MS, Patterson GR. Parents and adolescents: Living Together. Part II: Family Problem Solving. Eugene: Castalia 1989. 117. Patterson GR, Chamberlain P. Treatment process: A problem at three levels. In: The State of the Art in Family Therapy Research: Controversies and Recommendations. New York : Family Process Press 1988: 189-223. 118. Kendall PC. Toward a cognitive-behavioural model of child psychopathology and critique of related interventions. J Abnorm Child Psychol 1985; 13: 357-372. 119. Kendall PC, Kane M, Howard B, Siqueland L. Cognitive Behavioural Therapy for Anxious Children: Treatment Manual. Philadelphia: Department of Psychology, Temple University, 1989. 120. Dodge KA. A social information processing model of social competence in children. In: Perlmutter M (ed): Minnesota Symposia on Child Psychology. Hillsdale: Lawerence Erlbaum Associates 1986. 121. Novaco RW. Anger and coping with stress: Cognitive- behavioural intervention. In: Foreyt JP, Rathjen DP (eds): Cognitive Behavioural Therapy: Research and Application. New York: Plenum1978. 122. Perry DZG, Perry LC, Rasmussen P. Cognitive social learning mediators of aggression. Child Dev 1986; 57: 700-711. 123. Rubin KH, Krasnor LR. Social cognitive and social behavioural perspectives on problem solving. In: Perlmutter M (ed): Minnesota Symposia on Child Psychology. Hillsdale, NJ: Lawerence Erlbaum Associates 1986. Disruptive Behavior Disorders 299

124. Loeber R. Antisocial behavior: More enduring than changeable? J Am Acad Child Adolesc Psychiatry 1991; 30: 393-397. 125. Robins LN, Price RK. Adult disorders predicted by childhood conduct problems: Results from NIMH epidemiologic catchment area project. Psychiatry 1991; 54: 116-132. 126. Deivasigamani TR. Psychiatric morbidity in primary school children-an epidemiological study. Indian J Psychiatry 1990; 32: 235-240. 127. Malhotra S. Temperament characteristics of children with conduct and conversion disorder. Indian J Psychiatry 1989; 31: 168-172. 128. Malhotra S, Varma VK, Verma SK. Temperament as determinant of phenomenology of childhood psychiatric disorders. Indian J Psychiatry 1986; 28: 263-276. 129. Reddy MV, Kaliaperumal VG, Channabasavanna SM. Cluster formation in child psychiatry; Part I: Some methodological evaluation. NIMHANS J 1997; 15: 7-17. 130. Reddy MV, Kaliaperumal VG, Channabasavanna SM. Cluster formation in child psychiatry; Part II: Some empirical classification. NIMHANS J 1997; 15: 18-30. 131. Changulani M, Malhotra S, Chakrabarti S. Externalizing and Internalizing Disorders: Validation of a broad classificatory approach. In: Malhotra S, Sharan P, Gupta N, Malhotra A, Gill S (eds): Mental Disorders in Children and Adolescents: Need and Strategies for Intervention. New Delhi: CBS 2005: 17-36. 132. Malhotra S, Kaur R, Nehra R. Life events in psychiatrically sick children. Indian J Psychiatry 1992; 34: 222-230. 133. Malhotra S, Aga VM, Balraj, Gupta N. Comparison of conduct disorder and hyperkinetic conduct disorder: A retrospective study from north India. Indian J Psychiatry 1999; 41: 111-121. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Anger, Hostility and 23 Aggression Syndrome

Akash Bang, Satish K Tiwari

INTRODUCTION We are living in the “hightech” era of modernization. The physical comfort associated with modern lifestyle has many negative impacts on human life and behavior. The so-called “busy life” of modern age individuals has blurred many emotions, which were part of normal human being. Despite availability of ever increasing new and newer amenities, the individuals have no time to enjoy these. The human being is forgetting the art of relaxation due to stress and strain of life. These changes have resulted in increasing incidences of anger, hostile and aggressive (including extremist and terrorist) behavior. We are in a stage where many individuals are unable to control their anger/aggression for trivial reasons. An individual feels that he knows/understands almost each and everything as he can get any information from Internet, friends or peers. The tolerance has decreased resulting in increasing incidences of hostile behavior or conduct disorders. The frustration, anger and aggressive outbursts are experienced during all stages of life.

DEVELOPMENT OF EMOTIONS From the age of 18 months to 3 years, children begin to develop autonomy and start separating from primary care-givers. At this stage, they also develop negativism and/or oppositionalism. When they cannot express their autonomy they become frustrated or angry. Some of them show their frustration and defiance with physical aggression or resistance.1 Caregivers who respond to toddler defiance with punitive anger run the risk of reinforcing the defiance and teaching the child that out-of-control emotions are a reasonable response to frustration. Anger, Hostility and Aggression Syndrome 301

DEFINITION The syndrome is a theoretical construct of co-occurrence and segregation of these 3 behaviorally observable entities, regardless of underlying cause and severity and duration of associated conditions,. This triad of signs is etiologically nonspecific and occurs in almost all psychiatric disorders. Anger is an emotional state or personality trait consisting of feelings of irritation, annoyance, fury and rage along with heightened activation of the autonomic nervous system and the endocrine system, tension in the skeletal musculature, antagonistic thought patterns and at the same time aggressive behaviors. Hostility is an internal or emotional feeling of enmity, anger, resentment, belligerence and disdain for other people. It may be expression of anger in verbal/nonverbal form. It is a multidimensional concept that includes an attitudinal (indirect hostility and suspicion), an emotional (anger, type A, irritability and resentment) and a behavioral (assault, verbal hostility and negativism) component. Aggression means a behavior with the intention of hurting someone, something or oneself. It is a domineering, forceful and/or assaultative verbal and/or nonverbal action towards a person as a motor component of anger, hostility and rage.

ETIOLOGY AND PREDISPOSING FACTORS Various factors which may be responsible for such type of behavior have been identified. Multiple risk factors have independent additive effects. Higher the number of risk factors, greater the likelihood of a child being aggressive. These include:

Genetic Such behavioral tendencies may have heritable or genetic predisposition. There are increased chances of criminality among monozygotic twins. In well-controlled studies, adopted children with antisocial biologic fathers presented later in life with more antisocial behaviors than did those with adoptive non-criminal parents. However, children with both biologic and adoptive antisocial fathers were the most antisocial in later life. 302 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Environmental The social context in which behaviors unfold may trigger, exacerbate or ameliorate those behaviors. Environmental factors like childhood rearing practices may also be responsible for hostile, aggressive behavior. Research into the relationship between the Type A behavior pattern and coronary heart disease suggests that the anger-hostility-aggression (AHA!) syndrome is directly related to total serum cholesterol and low-density lipoproteins.2

Familial Discord in the family, marital discord, chronic sexual abuse, drug or alcohol abuse in a parent, unemployment, criminality, aggression within the home and psychiatric disorder increases the risk of aggressive behavior. Temperament of the family members, caretakers and the individual has significant effect. It has been observed that criminality and violence runs in families.

Impact of Media Exposure to aggressive personalities on television or in plays stimulates aggressive behavior. Viewing media violence can contribute to promoting a view among children that violence is common and acceptable in everyday life.

Sex of the Child Aggressiveness is more common in boys as compared to girls. Chromosomal disorders with extra Y chromosome may be associated with aggressiveness.

Hormonal Status Male sex hormone is known to be associated with aggressive behavior. A relation between testosterone and aggression in human is yet inconclusive. It has been found that there is a tendency for testosterone levels to rise and hostility to drop (or vice versa) after repeatedly experiencing success (or failure).3 The levels of circulating serotonin are related to impulsivity and aggression. Increased serotonin functioning has been reported in prepubertal aggressive boys as compared to prepubertal non-aggressive boys. Anger, Hostility and Aggression Syndrome 303

Other Factors The impact of peers and teachers, expectations generated from school performance may also modify the behavior. Some other factors which may precipitate disruptive behaviors can be; Temporal lobe epilepsy, CNS (pre frontal lobe) injury/tumors or infections.

SPECTRUM OF SUCH BEHAVIOR There are multifaceted presentations of aggressive behavior. The challenge for clinician is to separate those behaviors that are normal developmental variation from those that represent an emotional disorder or are symptoms of a specific disease process. Psychologists and medical doctors feel that the AHA syndrome is associated with behavioral disorders and coronary heart disease for the angry person. The spectrum of anger and aggression may begin from simple breath-holding spells, temper tantrums etc. and may end in disruptive extremist conduct behavior.

Type A Behavior Typically, it comprises of achievement orientation, competitiveness, repressed hostile feelings, excessive impatience, over activity and a continuous sense of time urgency.

Types of Hostility The subclass of hostility may be; physical assault, indirect hostility, irritability, negativism, resentment, suspicion and verbal hostility.

Intentional Aggression Such type of behavior is to achieve an end, or primarily hostile, to inflict physical or psychologic pain.

Physical Aggression Physical aggression includes hitting, biting, pushing, shaking, pinching, scratching, burning, tying up, and the like. Violence is aggressive behavior where the actor/perpetrator uses his/her own body as an object to inflict injury or discomfort upon an individual. 304 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Verbal Aggression Verbal aggression means sarcasm, belittling, cursing, denigrating, scapegoating and saying other thoughtless, unkind cruel things to or about the person.

Symbolic Aggression Symbolic aggression is the use of angry or rude hand gestures or facial expressions.

Interpersonal Coercive Behavior Interpersonal coercive behavior is more common in girls and appears to be related to the development of more physical aggression in adolescents.

Aggression with ADHD In those children who have aggression associated with Attention Deficit Hyperactive Disorder have more chances of developing oppositional defiant disorders/conduct disorders.

Oppositional Defiant Disorder Oppositional defiant disorder is a negativistic, argumentative hostile behavior which may include loss of temper, disobeying or annoying other people, being vindictive and resentful etc.

Conduct Disorders Conduct disorders are those in which there are repetitive, persistent and violent disruption to the rights and property of other peoples. There may be aggression to peoples, destruction of property and serious violations of rules. They may result in antisocial personality disorders.

Extremist Activities/Adult Criminality Extremist activities/adult criminality is an antisocial personality behavior/ disorder in adulthood.

Passive-aggressive Behavior These children express hostility indirectly as procrastination, stubbornness or resistance. Parents often complain that such children do not hear them Anger, Hostility and Aggression Syndrome 305 and that they fail to respond to repeated requests. These children are fearful of direct expression of assertiveness, aggression and hostility.4 Angry or hostile individuals tend to engage in poor health practices like; avoiding physical exercises, eating high fat diets, drug abuse and other risk-taking behaviors.

ASSESSMENT AND DIAGNOSIS A detailed history of environmental factors or triggering events should be taken. Details of seriousness, nature and quality, motivation and attitude towards aggressive behavior should be sought. A detailed account of previous attempts at self-harming and suicidal attempt should also be obtained.5 Also try to find out the interest or strength of the child and other family members. Try to explore the past disciplinary evaluations, playground and classroom behavior and learning style and output in school. Parents and the child can be interviewed together as well as separately so that the detailed information can be gathered without hesitancy. A detailed family history including periods of anger outbursts, physical or verbal violence and specific encounters should be elicited. Information regarding impact of social forces, media including the duration of TV/ movie exposure should be asked for.

APPROACH TO THE CHILD Aggressive behavior is a major problem associated with significant morbidity and mortality in childhood. Before labeling any child as abnormal one must rule out that the toddler or adolescent is expressing his/her autonomy and quest for emotional independence in a healthy, affirmative way. It is important to assess causes and motives for childhood aggression. The approach can be: a. Individual therapy b. Family interventions c. Youth centered programs d. Community based programs e. Sports activities

Early mental health intervention should be undertaken so that the affected child may “grow out” of persistent aggressive behavior. 306 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Long-term behavior and psychotherapeutic intervention programs must be planned. Behavior modification techniques for adolescents in crisis comprise a number of therapeutic interventions designed to address specific behavioral problems and give the child tools to change those negative behaviors. The behavioral modification programs may include:6 a. Positive parental reinforcement with use of praise or rewards shall be practiced for an appropriate or healthy behavior. This may help by enhancing the self-esteem and conscience formation. b. Ignoring of undesirable negative behavior and distracting the mind of child from such behavior may be tried. c. Punishment and disciplinary measures may be needed in severe behavioral abnormality. A child may be put in “time-out” period after explaining the reasons for the same. This should be followed by “time- in” period when the child is welcomed back in the group. The punishment should be consistent, logical, proportionate and timely. There must be a strong message that unwanted behavior will not be tolerated. Effective family focused multisystemic intervention may be a good approach in such situation. Parent-child communication must be encouraged. Parents should be trained directly to promote pro-social behavior within the home and to place reasonable limits on unwanted, destructive behaviors. A parent’s ability to discipline and to teach is important for raising emotionally healthy children who can learn social rules. A parent should be role model for the expected behavior. There is need to teach self-regulation and proper alternatives to control abnormal behavior. Role-play, games, modeling may be used to teach children in terms of how to control their impulses. Relaxation exercises that the child learns to perform in order to remove a tension response may be used. Anger control/self-control techniques could also be advised. Improve the adolescent’s pro-social skills with regards to interpersonal issues, communication, conflict resolution, problem solving, negotiations etc. Recreational activities including sports participation and exercise may have positive effect on behavioral modification. Adolescent focused recreational activities that include sports participation and exercise may have positive effect in managing various mental health disorders while improving general well being. Anger, Hostility and Aggression Syndrome 307

Psychopharmacological Approach7 The indications for drug therapy are co-morbidity, failure of non- pharmacological interventions and severe symptoms. There is no specific drug therapy for the aggressive behavior. But many group of drugs have been tried. Polytherapy or multiple drug therapy should be avoided. Because aggression and oppositional behavior involves a wide spectrum of clinical syndromes, it is more relevant for the practitioner to identify target symptoms and then to use medications to alleviate these target symptoms. Psycho-stimulant drugs like, methylphenidate, dextroamphetamine and pemoline etc. act by enhancing sympathetic functions in the brain. They help in increasing social ability, improving mother-child and peer relationships. Antidepressants and anxiolytics (like buspirone) have been used in depression associated aggressive behavior. Alpha- adrenergic agonist like clonidine is found to be effective in patients with severe, resistant aggression. Similarly beta-adrenergic antagonist (beta- blockers) has found to be effective in organically induced aggression. Anticonvulsants like carbamazepine, valproic acid and newer drug like gabapentin have also been tried for the treatment of aggressive adolescents. Some miscellaneous drugs, which have been tried, are lithium, haloperidol, clozapine etc. The associated illnesses may be assessed and taken care off. Referral to a mental health specialist may be needed in complicated and advanced cases. Such cases may need collaboration with psychologists, social workers, educators and psychiatrists.

KEY LEARNING POINTS • The syndrome is a theoretical construct of co-occurrence and segregation of the triad of behaviorally observable entities i.e anger, hostility and aggression, regardless of underlying cause and severity and duration of associated conditions. • Etiologically nonspecific, it occurs in almost all psychiatric disorders. • Assessment and diagnosis involves a detailed history of environmental factors or triggering events should be taken. The approach can be: • Treatment approaches include individual therapy, family interventions, youth centered programs, community based programs, sports activities, etc. 308 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Indications for drug therapy (psychostimulants, antidepressants, anticonvulsants) are comorbidity, failure of non-pharmacological interventions and severe symptoms. • Associated illnesses should be assessed and taken care off. • Referral to a mental health specialist may be needed in complicated and advanced cases. • Collaboration with psychologists, social workers, educators and psychiatrists may be needed.

REFERENCES 1. Gupta P, Dua T. Developmental problems of Infant and young child. In Ghai OP, Gupta P, Paul VK (eds): Ghai’s Essential Pediatrics 6th edn. New Delhi; CBS 2004: 54-65. 2. Richards JC, Hof A, Alvarenga M. Serum lipids and their relationships with hostility and angry affect and behaviors in men. Health Psychol 2000;19:393-398. 3. Sluyter F, Keijser JN, Boomsma DI, van Doornen LJ, van den Oord EJ, Snieder H. Genetics of testosterone and the aggression-hostility-anger (AHA) syndrome: A study of middle-aged male twins. Twin Re 2000;3:266-276. 4. Boris NW Dalton R. Disruptive behavioral disorders. In Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics,17th edn. Philadelphia: Saunders 2004 :88-90. 5. Gupta V, Gupta N, Tyagi A.Violence and aggression. In: Gupte S (ed): Recent Advances in Pediatrics (Special Vol. 17: Adolescence). New Delhi: Jaypee Brothers 2006: 291-304. 6. Stein MT. Difficult behaviors Temper: Tantrums to Conduct Disorders. In Rudolph AM, Rudolph CD, Hostetter MK, Lister G, Siegel NJ (eds): Rudolph’s Pediatrics, 21st edn. New York: McGraw-Hill 2002;444-450. 7. Greydanus DE, Pratt HD, Patel DR, Sloane MA. The rebellious adolescent. Pediatr Clin North Am 1997;44:1457-1485. RAP Special Volume 19: Developmental and Behavioral Pediatrics 24 Behavior Analysis in Children

Ajay Gaur

INTRODUCTION Behavior, in the broadest sense, encompasses all activities concerning autonomic responses and integrated reactions to dynamic situations, the experiencing and expressing of emotions and the forming and maintaining of interpersonal relationship.1 The prevalence of behavior disorders in children with development disabilities approaches 50% and range from noncompliance and hyperactivity to self-injury.2 Applied behavior analyses often contribute substantially to the diagnosis and treatment of these problem behaviors. Applied behavior analysis is the branch of behavioral science that designs and experimentally analyzes practical procedures for producing adaptive change in socially significant behaviors. It has three principal defining characteristics:3 1. It focuses on human behavior, that is, what people do and say 2. It concerns itself with environmental influences on such behavior, in contrast to constitutional factors, intra psychic influences, or socio demographic variables 3. It uses within-subject experimental designs to determine which environmental interventions best result in behavior change.3,4 The fundamental assumption underlying applied behavior is a function of its past and current antecedents and consequences, including those related to biologic determinants and concomitants. Effective management hinges on the accurate identification of problem behaviors and their specific antecedents and consequences. Clinical evaluation of the children is both challenging and complex in that the Pediatrician must draw upon multiple sources and a range of techniques for eliciting information on various aspects of the child’s functioning. 310 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

CLINICAL ASSESSMENT OF THE CHILD The clinical assessment of the child for the behavior problem requires a comprehensive approach that evaluates the child’s developmental progress in various domains and positive adaptive capacities, as well as the presence of the pathognomonic symptoms of specific disorders. A developmental approach to the assessment of the child is essential because children differ from adults in certain key respects.5

Distinctive Features of Childhood Psychopathology First, behavior and psychiatric disturbances in children often consist of a lack of developmental progress in one or more domains, rather than the presence of specific symptoms that are pathognomonic of adult disorders.

Second, child’s developmental status may affect the clinical presentation of various syndromes.

Third, developmental brings expectable periods in which distressing emotions or impairing behavior may occur as part of a normal transition. In many cases clinical conditions represent severe forms of symptoms found in milder forms. Fears, tantrums, moodiness, or restlessness are relatively common in childhood and occur transiently at different stages. In many cases, children’s behavior is a greater cause of distress to others- parents, teachers, and peers- than to the children themselves. Concerned parents needing guidance on how to understand may seek assessment. To distinguish transient or normative difficulties from those that more clinically worrisome, the assessor must possess a knowledge of both normal and abnormal child development. This developmental frame of reference includes an understanding of what behaviors can be expected normally in children of different ages, the time frame within which various behaviors normally wax and wane and the natural history of behavior disturbances at different stages in development, including knowledge of the ages at which particular syndromes are more or less likely to present. Many children coming to clinical attention have difficulties that cannot be neatly subsumed under the rubric of a single diagnostic label. Thus, comorbidity is usually not the exception but the rule in childhood disorders.6 Behavior Analysis in Children 311

Distinctive Aspects of Child Assessment Context and the Need for Multiple Informants The child’s functioning and behavior well being are strongly related to the social context-the family, school and community settings-in which the child lives and develops. Thus, to evaluate the nature and severity of the child’s behavior status, information is needed about the child’s relationship to, and functioning in, these diverse settings. The social environment can be appraised from what parents say about their relationships with the child and from the child’s direct report as well as form other sources like playground; however direct observation of parent-child interactions in the office setting also provides much useful additional data. In some cases, a home visit may provide invaluable information. It is also important to gather information on the child’s functioning in school, a task that often requires speaking with the child’s teacher, in addition to reviewing school reports and educational assessments.7

Special type of Assessment During the evaluation the pediatrician will want to see the parents alone, the child alone, and the parents and child together. This decision is usually made with reference to the child’s age. When evaluating a young child the parents are often seen first, without the child. This allows the pediatrician to obtain a history and to discuss how to prepare the child for the evaluation. On the other hand, the adolescent should usually be included in the initial interview so that the youngster does not identify the pediatrician as an agent of the parents.

Parent Interview In the initial parents interview the pediatrician seeks to understand the parents view of the problems that have led to this assessment, including the nature of the child’s current difficulties; the explicit and implicit reasons for the timing of the assessment; and the impact of the child’s difficulties on the parents, individually and as a couple, as well as on the well-being and functioning of the family as a whole. A detailed history of the child’s development and a review of family medical and psychiatric disorders are obtained. Finally, the pediatrician seeks to understand how the family is functioning in their community and cultural setting. This includes a 312 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics careful appraisal of the strengths of both the child and the family, in addition to the history of the presenting symptoms and areas of impairment.8 In taking a history of the current problem, the pediatrician obtains a detailed picture of the parents’ current emotional and behavioral concerns about the child. Parents should be asked to describe specific instances of the child’s problematic behavior, frequency, intensity, duration, circumstances in which this behavior occurs, their responses, and the child’s responses to it. The extent to which the symptoms cause functional impairment is ascertained by asking about the level of the child’s distress, interference with social and academic activities, impact on ongoing development, and the effect of the child’s behavior or symptoms not reported by the parents but known to be commonly associated with the presenting problem or with disorders that the presenting problem may suggest. Thus, one goal of the initial parent interview is to develop a detailed picture of the presenting problems, their course, their effect on functioning and on the family, and what has been tried to help alleviate them. Throughout the history taking, the pediatrician strives to understand the meaning and function of the symptoms in relation to the factors in the child and environment that influence them.9 To understand the child’s presenting problems, developmental status, and family and social context, the pediatrician must learn about the child’s strengths, interests, talents, and areas of adequate or superior adjustment. Such information is essential for a full diagnostic understanding of the child and helps the pediatrician appraise the child’s overall adjustment and functioning. Moreover, in planning for treatment, factors that may help counterbalance or remediate the child’s vulnerabilities must be identified. Finally, the pediatrician’s appreciation and discussion of the child’s strengths and areas of good functioning provides support and reassurance at a time when the child’s problems and limitations have been the parent major focus of attention.

Developmental History The developmental history is a detailed accounting of the child’s development across physical, cognitive, linguistic, social, and emotional domains. One task of this part of the parent interview is to ascertain the timing and sequence of development events from pregnancy to the present. Parents Behavior Analysis in Children 313 vary in their ability to provide precise information on the timing of specific milestones; however, an adequate sense of the course of development can often be obtained by having the parents compare the patient’s progress in a particular domain with that of siblings. Asking parents about the timing of changes in the family, such as moves or other memorable life events may help provide a chronological framework for remembering features of the child’s development. Besides gathering descriptive information about the child’s development, this part of the interview often allows one to tap the emotional aspects of the parent-child relationship by querying the parent’s hopes, worries, expectations and life circumstance in relation to different developmental events. It is used to conceptualize the child’s past and current development, in the following domains.10

Cognitive and academic development In tracking cognitive development and academic success, ask about the child’s ability to separate from parents and to attend school regularly, interpersonal with peers and teachers, motivation to learn, ability to function independently, tolerance for frustration and delay of gratification, attitudes toward authority, ability to accept criticism, etc.

Physical development and medical history Assessment includes fine and gross motor development, toilet training and its lapses, eating behavior and altitudes, and sleep patterns. Precocious development or delay in physical growth or pubertal maturation should be noted. In this context, parents should be asked systematically about medication, illness, hospitalizations, operations, and episodes of loss of consciousness, seizures, head injuries, as well as the child’s reactions to these and their impact on subsequent health and activities. Inquiry about tics, difficulties with hearing or vision, lead exposure and other specific conditions may be suggested by the clinical presentation. When necessary, medical reports should be obtained and reviewed. Emotional development and temperament This domain of assessment includes the child’s present and past mood and capacity for affect regulation, anxieties, and ability to adapt to changes and situations that are new, challenging, or frustrating. Prevailing mood as perceived by the parents should be assessed, and if the child has shown depressive symptoms, including irritability, the pediatrician should ask specifically about worries, fears, and other manifestations of anxiety level of any distress and 314 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics impairment. Unusual fears, excessive shyness withdrawal, and obsessive or compulsive symptoms are also important.11 The child’s capacity to regulate aggression falls within this domain and should be reviewed. Ask about circumstance in which the child becomes angry or aggressive the mode of expressing anger (i.e. verbal, physical, or both), the impact on other, and the child’s reaction to and processing of such feeling and behavior. In this context the clinician seeks to evaluate how the child manages aggression, and whether the child is too aggressive, or too fearful of his or her own anger or that of others. Specific aggressive symptoms should also be sought.

Relationship with family and Peer The parent interview should include assessment of how the child relates to each family member and how the child fits into the overall family system. Ask about compliance with family rules and standards as well as consequences when the child does not comply (i.e. usual mode of discipline or limit setting) and the child’s response to such interventions. The pediatrician should gather information about how the child relates to peer, including the number of friends, preferences regarding age and gender of friends, and any major changes in peer group, the child’s satisfaction with these relationships; their relative stability; activities and interests shared with peers; and parent’s feelings about the child’s close peers or lack thereof. The parent’s perspective on the child’s social skills and deficits should be assessed, including their sense about any difficulties should be assessed, and including their sense about any difficulties the child has in this domain. For adolescents, this part of the history includes issues such as the capacity for intimate relationships, romantic interests, sexual activity, and concern over sexual orientation.

Interests, Hobbies, Talents and Avocations Although this line of inquiry is also pursued with the child, it is useful to obtain the parents perspectives on the child’s interests and activities and to assess the parents’ approval involvement and support for them. Indications of parent-child conflict should be probed and may provide useful information about the relationship. The presenting difficulties may have affected the child’s ability to focus on or engage in previously enjoyable areas of interest or activity such as sports or music. Behavior Analysis in Children 315

The quantity and type or TV programs, movies, and videos a child’s is permitted to watch provide information on both the child’s interests and the quality of parental limit setting.12

ASSESSMENT OF PARENTS The Pediatrician should assess the parents as individuals and as a couple, including strength, weakness and areas of conflict or difficulty. When taking the developmental history it is often natural to inquire about the parents’ feelings and involvement with the child’s at different stage. The parents’ individual views on the causes of the child’s difficulties and the extent to which they agree or disagree on this and other aspects of the child’s care and mediations. The parents’ education and occupation may bear on how they understand and respond to the evolution.

Family Medical and Psychiatric History A careful family medical and psychiatric history in needed to identify any medical or psychiatric disorders with potential genetic or environmental implications for the child. Among those to be routinely considered are psychiatric disorders, mood disorders, anxiety disorders and antisocial personality disorders as well as metabolic and neurological disorders. The impact of any family members’ symptoms, hospitalization or incarcerations should be probed, with particular reference to the child. While parent’s concerns usually play a major role in initiating the evaluation, parents may perceive the child’s problems differently and may disagree about their severity and the need for treatment. Thus, discrepancies should be noted and regarded as potentially useful data regarding what they reflect about the clinical situation. Reason for discrepant reporting involves differences in how the informants evaluate the child’s behavior. Observers may judge the same behavior by differing standards or expectations for many reasons. Mothers and fathers also differ in their experiences with their children, depending on the child’s age and gender, shared interest, parental availability and the respective temperaments and personalities’ availability and the respective temperaments and personalities of the parents and child. Although mothers have been reported to be more reliable informants than father, in practice it is wisest both for gathering the fullest information possible and for fostering an alliance with the parents, to involve both 316 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics parents from the outset. When appropriate other informants such as siblings, grandparents, or child caregivers, may be called on to contribute information, but only after the implications for the family system and the child’s specific relationships with household members are carefully thought out.13

CONDUCTING THE ASSESSMENT For the pediatricians conducting the assessment the issues is not which informant is “right” but how to understand the differences in viewpoint most effectively for the patient’s benefit. Thus, eliciting and synthesizing information from multiple informants is a critical aspect of the clinical evaluation of children and adolescents. During assessment it is important to note that the basic principles of counseling like nonverbal communication, open questions, your gesture, reflect back, empathy and use of non judge mental words should be practiced.

PREPARATION OF CHILD Children have many reactions to a first visit and assessor, and parents must anticipate their child’s possible reactions and prepare their own response. Children may feel anxious because they cannot envision what will transpire or because the evaluation may feel like a punishment. Some children fear that the assessment will reveal some profound or irreme- diable problem. Many younger children fear shots or having to get undressed for a physical examination. The child’s anxiety or defensiveness may be reduced if the purpose of the visit is put in supportive and friendly manner. The parents must encourage children to tell the pediatrician about their concerns.

INTERVIEW OF CHILD In the child interview the pediatrician directly assesses the child’s psychological functioning, development status across different domains, perceptions and experiences of the presenting problems. Thus, from the outset, the pediatrician should convey empathy and interest in understanding and helping the child. There has been debate regarding the reliability, validity, and clinical utility of various approaches to the child interview is an essential component of a comprehensive clinical child assessment. The child may be regarding important information, such as Behavior Analysis in Children 317 secrets about abuse, sexual or antisocial activities, suicide attempts, pathological patterns of eating or premonitory urges, obsessive or suicidal thoughts, or hallucinations may be apparent only to the child. Other symptoms may escape or mislead the untrained eye; detection of tics or a thought disorder may require direct clinical observation.14 There are many varieties of child interviews, depending on the nature of the presenting problem, the clinical setting, and the purpose of the assessment. Thus, the scope, focus, duration, setting and predominant techniques used will vary, depending on whether the assessment is done exclusively for behavior problem or it is a part of clinical assessment or the evaluation of a child who is academically underachieving, anxious, or manifesting tics. The clinical approaches the child interview with a developmentally based understanding of child and adolescent functioning and psychopathology. Within this framework the pediatrician expects to obtain information relevant to the diagnostic questions raised by the presenting problems, and so will systematically review the major domains of symptomatology. The order and manner of conducting the child interview vary with the child’s age and development status, the nature of the presenting problems, the setting and context. The constant elements in the interview process are the orienting framework chosen and the pediatrician’s clinical skill in engaging and eliciting relevant data from children of varying ages and capacities, with diverse problems and concerns. Some data may emerge spontaneously; some require questioning or other deliberate means of eliciting information.

Components of the Child Interview The components of the child interview are often conceptualized under two broad heading; history taking and the mental status examination. History taking involves inquiry into the significant areas of the child’s life and functioning, past and present, including the presenting problem. The mental status examination consists of an assessment and description of the child’s appearance and functioning as manifested in the interview. For the mental status examination, the following areas should be assessed. Physical appearance—including physical development, presence of minor congenital anomalies, style of hair and dress, cleanliness, and indicators of the quality of self-care or parental attentiveness (or its lack) to the child’s physical appearance and hygiene. 318 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Manner of relating to examiner and parents—including ease of separation, how confiding or guarded the child seems toward the pediatrician, eagerness to please or impress, flirtatious behavior, readiness to make excuses or minimize, passive withholding, or open defiance. The child’s behavior toward the examiner is compared with that observed or reported toward the parents or other adults. Affect—the child’s predominant mood and range of emotion during the interview and appropriateness of mood. Coping mechanisms—as manifested toward the assessor, expressed in age- appropriateness of the child’s dependent longing, aggressive feelings with respect to intensity, object and mode of expression; child’s control or modulation of such urges. Orientation to time, place, person—whether children are accurately aware of the date, where they are and who they are. Motor behavior includes activity level coordination, presence of unusual postures or motor patterns (tics, compulsions, or stereotypes, such as hand flapping or twirling). Quality of thinking and perception—including presence of hallucinations, delusions, thought disorder, flight of ideas, and adequacy of ideas, and adequacy of hearing and vision. Speech and language—including reading and writing. Articulation, inflection, pitch, rhythm and fluency of the child’s speech; richness, limitation or idiosyncratic aspects of vocabulary and syntax; delayed or deviant social use of language; and lack of accompanying nonverbal communicative behavior. When evidence of delays or problems in these realms exists, formal speech and language evaluation by a qualified specialist may be warranted. Overall intelligence and fund of knowledge—capacity for classification, abstraction and inference appropriate to age; counting, alphabet, days of months regarding writing computational abilities geographical and historical facts. Attention, concentration, impulsivity—level of attention of activity or topic of discussion, degree to child jumps from activity to need for physical limits; distractibility by outside noises. Memory—immediate short and long term memory (e.g. repeats three items and recall at 5 and 30 min.) Behavior Analysis in Children 319

Neuralgic functioning—fluctuating alertness, tremor, nystagmus, choreiform movements, abnormal gait, neurological signs, cerebral dominance (preferred hand, foot, eye, ear) testing for neurological signs can be incorporated in a game like fashion (e.g. ball throwing, repetitions of finger tapping, successive finger tapping, alternating pronation- supination of the hand are reliable components of the revised neurological examination for subtle signs [NESS]). Judgment and insight—especially concerning the presenting problem; this is most usefully judged after the child has had the opportunity to develop some rapport with the pediatrician, as the child’s initial impulse may be deny or minimize the parents’ presenting concerns. Preferred modes of communication—Is the child open to talking directly about the presenting problems and the significant aspects of his or her life or is the child more comfortable with indirect modes of expression such as play or drawing. Reliable detailed assessment of a child’s speech and language capacities, intelligence, academic attainment, attentional and executive functioning and memory requires standardization.15 Some simple tasks, such as those used in the Mini-Mental State Examination16 (as adapted for children by RA Ouvrier) can be incorporated in game like fashion into the interview of the child to provide a rough screen of higher mental functions such as orientation, attention, memory, language and constructional ability (Table 24.1). Table 24.1: Mini-mental scale

Orientation Score Points

1. What is the Year? — 1 Season? — 1 Date? — 1 Month? — 1 2. Where are we? Country — 1 State or territory — 1 Town or city — 1 Hospital or suburb — 1 Floor or address — 1 Registration 3. Name three objects, taking one second to say each. 3 Then ask the patient all three after you have said them. (Tree, clock, boat). Give one answers until patient learns all three. Contd... 320 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Contd... Orientation Score Points

Attention and Calculation 4. Serial sevens. Give one point for each correct answer. 5 Stop after five answers. 5. Spell WORLD backwards 5 Recall 6. Ask for names of three objects learned in 3 Q.3 Give one point for each correct answer Language 7. Point to a pencil and a watch. Have the patient name 2 them as you point 8. Have the child repeat “No ifs, ands or buts” 1 9. Have the child follow a three-stage command. 3 “Take a piece of paper in your right hand. Fold the paper in half. Put the paper on the floor” 10. Have the child read and obey the following 1 “CLOSE YOUR EYES.” (Write it in large letters). 11. Have the child write a sentence of his or her choice. 1 (The sentence should contain a subject and an object and should make sense. Ignore spelling errors when scoring). 12. Have a child copy the design printed below. 1 (Give one point if all sides and angles are preserved and if the intersecting sides form diamond shape). Total 35

Mental Status Examination Throughout the assessment the pediatrician observes and notes information relevant to the domain of the mental status examination. Some abnormalities or deficits may be evident through this process; however, other areas will requires direct examination, which can usually be incorporated in a playful way into the assessment. While intensive developmental, psychometric, language, and neurological assessment usually cannot be subsumed within the clinical examination and assessment, a brief semi structured evaluation requires only about 5 to 10 minutes and provides useful information. This generally involves drawing; copying a design; simple tests of orientation, recall, general knowledge and language (e.g. naming three objects; repeating a phrase such as “No ifs, and or buts” a three stage command such as “Take a piece of paper in your right hand, fold the paper in half, put the paper on the floor”; a Behavior Analysis in Children 321 writing sample [such as asking the child to write a sentence of their choice]; and a brief screen of neurological soft signs.17

Standardized Assessment Instruments for Children At present there are number of standardized interviews and rating scales that provide systematic methods for eliciting information about child psychiatric symptoms and for combing that information to yield categorical diagnoses or dimensional scores on statistically derived scales. There are a number of reasons why the development of measures to assess psychopathology in children is more challenging than the comparable task with adults. These challenges include (1) the need to incorporate an understanding of various aspects of development into the framing and wording of questions about symptoms and into the process of discriminating normative behavior from pathological behavior (2) the necessity of conducting the assessment with both child and parents (and at times with the teacher), since often some kinds of information are uniquely reported by one or the other informant; (3) the importance and difficulty of integrating concepts of social context and adaptive functioning into a symptom based assessment; (4) the lack of readily available gold standard with which to compare the results from assessment methods in order to modify and improve them. Despite or perhaps because of these challenges, extensive research effort has been directed toward diagnostic assessment in child psychiatry; this effort has yielded a number of measures for use in clinical and epidemiological research. Some of the methods described below have found appropriate adjunctive roles in certain clinical settings.18

Structured and Semistructured Interviews19 Available interviews for the assessment differ from each other in several respects. A fundamental one is the degree of structured that is built into the interview.20 Interviews range from highly structure instruments that specify the exact order and wording of all components to semi structured interviews that may be used but permit much latitude in the order and phrasing of question. The degree of structure reflects the intended use of the interview that will administer it, and the cost of using it in a study. On a continuum of structure, semi structured interviews resemble clinical interviews resemble clinical interviews more closely than do 322 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics structured interviews, but they nevertheless differ substantially in style and content from a true clinical assessment. Semi structured interviews are designed to be administered by interviewers with clinical training or with very intensive training in the assessment of symptoms covered by the measure. The flexible structure of the interview allows the clinically informed interviewer some freedom in the manner of inquiry and permits judgment about whether a reported behavior is to be considered as a symptom. Structured interviews, on the other hand provide highly specified protocols that are particularly useful for epidemiological studies in which large sample sizes require using many non-clinician interviewers. These studies investigate issues of prevalence of disorders, developmental patterns of psychopathology, psychosocial correlates, and risk and protective factors, topics that often require large samples to provide enough statistical power to examine study hypotheses. Because it is obviously unwise to have untrained interviewers make clinical distinctions and because using more highly trained interviewers becomes prohibitively expensive, considerable effort has been devoted to the development of highly structured techniques. In recent years, the bulk of this work has focused on writing and testing the Diagnostic Interview Schedule for Children (DISC), which has proceeded through several rounds of methodological testing in clinical and community samples.

Rating Scales A variety of checklist rating scales have been developed over recent years to assess symptomatology and various aspects of behavior and adaptive functioning. Different measures have been designed to be completed by parents, teachers, or children. These may focus on either a broad range of symptoms or a specific area. The development of each scale involves testing its reliability and validity and administering it to large and diverse samples to determine norms clinically relevant cutoffs. Probably the most widely used comprehensive symptom rating scale is the child behavior checklist developed and extensively tested by Thomas Achenbach and Craig Edelbrock. It is a 118-items inventory of behavior and the parent or primary caregiver fills out functioning that. The component behavior and competency scales have age gender group norms and clinically meaningful cutoffs. Companion forms exist for the teacher Behavior Analysis in Children 323 to fill out and for the youth (Youth Self Report). These measures provide a convenient way to screen for overall level of symptomatology, and when scored, they provide a profile of problem area and competencies. The child behavior checklist is stable over relatively brief intervals (i.e. good test retest reliability) but also reflects changes resulting from treatment. The child behavior checklist TRF and YSR have been widely used in epidemiological and clinical research and in many clinical settings. These measures can be a useful clinical screening tool and an efficient way for clinics to gather uniform information for characterizing the populations served and changes in those groups over time. The behavior assessment system for children, developed by Cecil Reynolds and Randy Kamphaus, is a relatively new multimethod assessment for evaluating aspects of behavior and personality in children ages 4 to 18 years. It has 5 components including parent, teacher and child self reports a structured developmental history and a system for systematic collection of observed classroom behavior. A distinctive feature of the BASC is its emphasis on positive aspects of the child’s adjustment in addition to rating symptoms and problem related to school functioning, such as difficulties in social or study skills. Norms have been established for children by age, gender and clinical, educational, research, and program evaluation contexts. The Conners’ Parent Rating Scale (CPRS) is a commonly used rating scale that collects systematic information from a parent on the child’s conduct, activity level, attention, impulsiveness and somatic complaints. The scale can be scored to reflect a child’s symptom level on six component subclasses, including a general index of hyperactivity. Norms have been determined for boys and girls ages 3 to 17 years. The Conners’ Teacher Rating Scale (CTRS) is a companion instrument completed by the child’s teacher that can be convenient means of collecting information on a child’s behavior in the classroom. It is often a useful adjunct in evaluating and monitoring children being treated with medication for attention-deficit/ hyperactivity disorder. The CPRS and CTRS are well known as the family of six instruments developed by Kieth Conners and colleagues, known as the Conners’ Rating Scale-Revised. (CRS-R) This family of instruments has scales of varying lengths for parent, teacher and adolescent informants. The child behavior checklist and BASC families or rating scales are example of assessment tools designed to collect information across a broad 324 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics range of symptoms and competencies. The CRS-R focuses specially on disruptive behavior and symptoms of attention- deficit/hyperactivity disorder. More narrowly focused symptom rating scales have been developed to permit the quantitative assessment of specific symptom realms. Such symptom scales may be useful in quantifying the presenting severity of a symptom to establish a baseline against which response to a therapeutic intervention, such as medication can be compared. A number of these scales are self-report measures. These have the advantages of providing an opportunity for the child to report on symptoms that might be difficult to conceptualize or verbalize. Sometimes parents or children (specially adolescents) are initially more willing to report an area of difficulty in the seemingly more anonymous context of a questionnaire than they are in a face-to-face interview. On the other hand, self-reports also carry the risk of the child misunderstanding some questions or feeling compelled to provide socially desirable responses rather than acknowledging behavior and feeling that the child believes would be met with disapproval. With these potential limitations in mind, child self-report measures can provide useful adjunctive information in clinical setting or research studies.21,22 Among the rating scales useful in clinical practice are a variety of scales for assessing the severity of depressive symptoms. One of the most widely used the children’s Depression Inventory, developed by Maria Kovacs, and is a self-report measure for assessing symptoms of depression in children aged 7 to 17 years. It was adapted from the well-known adult scale, the Beck Depression Inventory, and has seen considerable use in clinical and research setting.

Limitations of Structured Measures in Clinical Assessment Interviews and scales such as those described above can at times be useful adjuncts in clinical assessment, if thoughtfully chosen and administered with sensitivity to their meaning in the overall process of the evaluation. For example, they can help ensure a comprehensive review of symptoms or provide a standardized baseline measure of severity for later determination of intervention efficacy. They cannot, however, replace an individualized child behavior or psychiatric interview nor be relied upon as the sole basis for establishing diagnoses or planning treatment. Most of the existing instruments were developed for use in research studies where Behavior Analysis in Children 325 data must be gathered from all subjects in a specified, uniform manner. Eliciting information for a clinical child assessment, on the other hand, requires comprehensive, detailed, and flexible inquiry and an empathic rapport with the parent and child. These elements cannot be provided by standardized interview format alone. Furthermore, most standardized interview schedules are designed as symptom inventories and not the comprehensive assessment of feelings, personality style, coping mechanisms, situational context, and adaptive strength that the clinical interview affords. Such factors may be as crucial to the clinical assessment and treatment planning as the presence or absence of a given pathgnomonic symptom or categorical diagnosis.

PSYCHOLOGICAL AND NEUROPSYCHOLOGICAL ASSESSMENT Psychological assessment using tests of personality and mental functioning can be a valuable part of the child behavior evaluation. Psychological assessment provides a unique perspective on the child’s condition by generating information complementary to that obtained from history, observation, and interview. With history and observation, such psychological data may clarify relationships among various etiological factors or help direct the selection of an appropriate intervention. Although partly inferential, the clinical information derived from projective tests of personality is less influenced by certain reporting biases than self-report. Psychological testing also provides data that are more quantitative than the qualitative information provided by the child’s history, presenting complaints, and clinical presentation. The quantitative character of test findings is more important in the assessment of cognitive abilities than in personality assessment because the child’s relative strengths and weaknesses in mental processing can be estimated only grossly from history taking and clinical observation. Such clinical estimates are also, necessarily, less accurate. The ability to discriminate meaningful difference among various domains of functioning helps to clarify the particular pattern or syndrome evidenced by the child. The assessment of cognitive functioning also reveals cognitive impairments that may underlie or influence the psychological condition under investigation (e.g. whether poor social judgment in a child with conduct disorder also results from a nonverbal learning disability). Furthermore, the manner in which any cognitive impairments or strengths interact with 326 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics the child’s psychological disorder can be delineated. Findings from tests of personality also identify important dynamics that affect the child’s clinical condition.

Adjunctive Evaluations A general pediatric examination is a useful adjunct to the behavior evaluation of a child (Table 24.2). In addition to general review systems, the pediatrician should assess the child’s current and past physical growth (head circumference, height, weight, pubertal status). Screening of hearing and vision, with more detailed testing as indicated, is important because deficits in these areas can manifest themselves in learning language, and social delays or seeming inattention or oppositionality. For children with developmental difficulties, a careful history of prenatal risk factors is taken; the pediatric examination includes careful evaluation for major or minor congenital anomalies, dysmorphic features, dermatological abnormalities (hyperpigmentations, tumors, fibromas) or ophthalmologic finding (lens abnormalities, retinal pigmentation). History taking includes abnormal movements or absence spells suggesting seizures, abnormal postures, and tics. The physical examination notes difficulties in muscle tone and strength; asymmetrical, abnormal or primitive reflexes; tremor; and gait or postural disturbances.23

Table 24.2: Clues to developmental disability by the physical examination

Area Findings Implication

Growth Head circumference, Microcephaly correlates highly with cognitive Linear growth, deficits Macrocephaly may indicate central Somatic growth nervous system abnormalities (e.g. hydrocephalus) storage disorder. Small size may indicate genetic disorder or embryopathy (e.g. fetal alcohol syndrome), endocrine abnormality (e.g. thyroid) Large size may indicate genetic disorder (e.g. cerebral gigantism or Sotos syndrome) Congenital Organ defects Storage disorders (e.g. mucopolysaccharides); anomalies Major anomalies may have enlarge organs Major anomalies such Major anomalies as heart defects may be associated with developmental delays. Minor anomalies such as wide-set eyes, low hairline, or extra digits may suggest a genetic syndrome Contd... Behavior Analysis in Children 327

Contd... Area Findings Implication

Skin Hyperpigmentation Skin lesions may be seen in neurocutaneous syn- Tumors of fibromas dromes such as neurofibromatosis or tuberous sclerosis Eye findings Lens dislocation Abnormalities inside the eye may suggest Corneal clouding metabolic disorders or congenital infections Cataracts Retinal pigmentation

The pediatric history and examination, in turn, help to take decisions about what further medical consultations (e.g. neurological, orthopedic, audiometric, and genetic) and which diagnostic tests (e.g., neuroimaging, laboratory tests, and electroencephalogram) are likely to be informative.

Diagnostic Laboratory Tests The clinical utility and cost effectiveness of routine laboratory tests for children who present with behavior problems has not been thoroughly studies. Laboratory screening tests are of some use in behavior and psychiatric settings where patients are at high risk for medical illness, such as the emergency room, substance abuse treatment centers, acquired immune deficiency syndrome, new-onset depression. Similarly, routine screening laboratory measures are more likely to yield clinically significant information when clinical symptoms of physical illness are present. One can review of routine laboratory screenings (thyroid function tests, electroencephalograms [EEG], chest X-ray, chemistry panel, urinalysis, complete blood count, electrocardiogram, and rapid plasma regain). More-specialized diagnostic laboratory evaluations (EEG, computed tomography [CT] magnetic resonance imaging [MRI], or chromosomal analyses) also provide a relatively low yield of clinically useful information. More specialized neuroimaging technologies, such as positron emission tomography (PET), single photon emission computerized tomography (SPECT), functional MRI, and brain electrical activity mapping (BEAM) currently have no routine clinical or diagnostic utility in child and adolescent psychiatric populations.24

Communicating Findings and Recommendations Communicating the findings and recommendations that results from the assessment to the parents and child are and essential part of the evaluation 328 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics process and may require one or more sessions. The child’s age and level of comprehension, presenting the findings usually involves meeting with the child and parents, either separately or together. Several principles must be observed to maximize the likelihood that the parents and child will hear, understand, and experience the pediatrician’s findings and recommendations as helpful. First, the pediatrician must communicate a sense of the child as a whole person, with strengths and abilities as well as problems and vulnerabilities. This perspective conveys a sense or the pediatrician’s appreciation and empathic understanding of the child, reduces defensiveness, and helps mitigate the fear that there will be only and bad news. The findings and recommendation will be most useful to the family and likely to be implemented if the interpretive session is a dialogue rather than a lecture. The pediatrician must use language comprehensible to the parents and child, avoid jargon, and keep technical terms to minimum. When technical terms are necessary, they should be explained clearly so the parents and child understand them; terms that are merely descriptive to the pediatrician may hold frightening or negative commutations for the child or family. Allow ample opportunity for the parents and child to discuss the recommendation; this provides a chance to judge whether the pediatrician’s formulation made sense to the family, to address any differences of opinion, and to explore the feasibility and acceptability of the recommendations offered. Remaining areas of uncertainly or ambiguity about the assessment or recommendations should be clearly noted and discussed. If the assessment is a consultation requested by an agency, a school, or another clinician, the findings and recommendations should be communicated to the referring party in appropriate terms, after being discussed with the parents and child and with their consent. If further evaluation or treatment is indicated but is best done by someone else, the pediatrician should offer to assist in appropriate referral.

BEHAVIOR MANAGEMENT TECHNIQUES Applied behavior analysts contend that most behaviors problem can be managed effectively through simple environmental rearrangements based on the processes of positive reinforcement, negative reinforcement, and punishment. Table 24.3 depicts one conceptual model. Behavior Analysis in Children 329

Table 24.3: Fundamental processes of behavior management

Operation on the Environment ______Presentation of stimuli Withdrawal of stimuli

Increase in rate Positive Negative reinforcement reinforcement Effect on Behavior Decrease in rate Punishment Extinction or Punishment

Positive Reinforcement Positive reinforcement is a process that refers to the presentation of an object or event following a behavior that increases the rate or probability of that behavior recurring in the future.25 Reinforces can be social events, activities, manipulable objects, or edibles. Table 24.4 presents examples of common reinforcers, but the list of potential reinforcers is virtually limitless. Although positive reinforcement procedures are designed by applied behavior analysts only to increase prosocial behavior, in everyday life the process of positive reinforcement also may inadvertently increase inappropriate behavior. For example, a parent who attends to a demanding child may unintentionally increase the likelihood of “brat” behavior may engender positive reinforcement of such behavior by peers, thereby exacerbating the problem. As is commonly understood, children behave inappropriately at time in order to obtain positive reinforcement in the form of attention. Misdirection of positive reinforcement is not limited to attention; however, any occurrence of positive reinforcement can have negative side effects.26 Table 24.4: Types of reinforcers

Social reinforcers Manipulable reinforcers

Praise Small toys (e.g., dolls, trucks) Gesture of approval (e.g., smile, head nod, hug) Board games Increasing proximity to child Money Offering assistance when asked Tickets to preferred events Initiating conversation about interesting topic Certificates of accomplishment Asking for demonstration of a mastered skill Books, magazines Grooming aids (e.g. make up kits, combs) Crayons, paint sets Musical instruments Contd... 330 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Contd... Activity reinforcers Edible reinforcers

Extra or extended play period Dry cereals Going outside Raisins Viewing television Talking on telephone Carrots Listening to music Fruit Dining at restaurant Popcorn Solving a problem Cracker Distributing reinforcers Juice Caring for pets Playing ball Riding bikes

Extinction In situations in which the child exhibits annoying yet non-dangerous or nondestructive behavior, a procedure called planned ignoring has been shown to be effective in decreasing the rate of problem behaviors maintained by positive reinforcement, such as attention. Based on the process of extinction, planned ignoring requires the care provider to withhold positive reinforcement after the targeted inappropriate behavior occurs. In this way, the positive reinforcer that had previously followed the occurrence of the misbehavior is withheld. Although effective over time, the desired impact of planned ignoring is seldom immediate. In fact, planned ignoring frequently produces a transient increase in the frequency and intensity of the problem behavior before a subsequent reduction.27 This temporary setback is often termed as extinction burst. Another phenomenon that is likely to occur during extinction is spontaneous recovery.28 This is the reappearance of the undesirable behavior sometime after it has been extinguished. Essentially, the child periodically tests whether positive reinforcement is again available contingent on the problem behavior. If the care provider continues to ignore the behavior, a sustained decrease in the problem behavior usually occurs. As an example, consider the child who makes persistent inappropriate request. If, after denying the initial request or engaging the child in a discussion about the inappropriate nature of the request, the child is more likely to badger the parents later under similar circumstances. The granting of the repeated request or the negative attention that ensues following the Behavior Analysis in Children 331 child’s persistence may be sufficiently reinforcing to encourage the child to continue to behave in a pushy manner. If the parents consistently do not respond to the persistent requests subsequent to a clear denial, at first the child’s persistence will intensify along with other problem behavior. If the parents continue to ignore the behavior, however, the persistent requests will eventually subside.

Differential Reinforcement In most cases, a child with learning and behavior problems displays several inappropriate behavior problems that warrant ongoing positive reinforcement.29-31 Therefore, positive reinforcement and extinction procedures are often used concurrently in a tactic referred to as differential reinforcement. The key objective of this procedure is to reinforce “good” behavior while simultaneously withholding reinforcement for unwanted behavior. For example, parents use differential reinforcement when they offer praise to the child when he or she shares but ignore the child’s tantrums in refusing to share. Table 24.5 lists the types of differential reinforcement. Table 24.5: Types of differential reinforcements

Type Goal Example

DRO (Differential Absence of targeted Child with SIB earns privilege Reinforcement of problem behavior during of listening to music for Other behavior) treatment interval refraining from SIB for 10 minutes. DRA (Differential Increased occurrence of a Child acknowledged for Reinforcement of targeted appropriate checking accuracy of Appropriate behavior) behavior in absence of completed seatwork, in targeted aberrant absence of any behavior that behavior disrupts peers DRI (Differential Increased occurrence of Attention is given to child for Reinforcement of a targeted appropriate remaining seated instead of for Incompatible behavior that is being out of seal without behavior) incompatible with a permission targeted difficult behavior Child acknowledged with DRL (Differential Occurrence of problem privilege contingent on low Reinforcement of behavior at acceptably rates of talking back to adults Low rate behavior) low rates

An important consideration when using differential reinforcement is to ensure that someone else does not reinforce the problem behavior. As 332 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics long as peers encourage the problem behavior of a classmate, a teacher’s efforts to scold disruptive behavior while praising on-task behavior will probably are ineffective. Consistent implementation of differential reinforcement by numerous care providers across diverse settings is usually required for behavior change to take place and remain durable. When serving children with disabilities, differential reinforcement is preferred over extinction because the former more clearly indicates to the children, which behaviors are appropriate and which are not.

Shaping In many situations involving a child with a developmental disability, the desired behavior initially may not be in the child’s repertoire at all. Precludes any opportunity to reinforce the behavior differentially. The targeted appropriate behavior initially may have to be developed through a procedure known as shaping. Shaping involves reinforcing successive approximations of the goal behavior.32 Graduated prompting and guidance procedures are often used in shaping behavior. Much structure of the learning situation and direct action by the trainer is required in the beginning. As the skill is shaped, the amount of structure and degree of involvement maintained by the teacher is gradually faded away, requiring the student to independently demonstrate skill mastery.

Imitation and Instructional Training Two other tactics are also commonly used to teach new behaviors. One is termed imitation training and entails demonstrating a new behavior to the child. The other is known as instructional training and involves describing a new behavior to the child. Put simply, these procedures are more highly systematized methods of show and tell. During imitation training, the care provider demonstrates what the child is to do or learn, asks the child to imitate what was shown, and then provides differential reinforcement within a shaping paradigm. Imitation training is often conducted on behalf of young children or older children with limited communication skills. During instructional training, the care provider first describes what the child is to do and then asks the child to complete the indicated action. The child is then acknowledged for following instructions. Because generalized instruction-following, similar to functional communication skills, is often a keystone behavior in a child’s repertoire, behavior analysts Behavior Analysis in Children 333 often aim to teach even children with severe disabilities basic imitation and instructional skills.

Negative Reinforcement Negative reinforcement, like positive reinforcement, is a process whereby the frequency of a behavior is increased, as shown in Table 24.1. Often confused with punishment, negative reinforcement is said to occur when, as a consequence of a particular behavior, an aversive event is either avoided or is escaped, resulting in an increased frequency of that operant behavior in the future. Said differently, a behavior is reinforced negatively if its rate of occurrence increases because an aversive stimulus or situation is avoided or withdrawn.33 As with positive reinforcement, negative reinforcement processes may serve to increase undesirable as well as desirable behaviors. For example, parents may given in to their children’s demands, even when the demands are accompanied by inappropriate behavior such as tantrums and aggression. As a consequence, such problem behavior on the part of the child is more likely to recur in the future because it has yielded a desired outcome. Conversely, parents learn to threaten, yell, and spank when these responses result in the immediate suppression of disruptive behavior in the children. Thus, negative reinforcement has to be used constructively, or it can contribute significantly to the emergence of problem behavior on the part of the child or the child’s care provider.

Punishment In contrast to both positive and negative reinforcement, which increase a behavior, punishment is a process by which an action is taken following a behavior that results in a decrease in the rate or probability of that behavior occurring under similar circumstances in the future. Punishment is usually mediated through two formats: presentation and withdrawal. Presentation refers to the delivery of a punishment following the occurrence of a behavior. For instance, a parent may punish a child for touching a hot stove by spanking or scolding. Punishment by withdrawal refers to the removal of positive reinforcement. Here the parents may withdraw from a child the privilege of a favorite television show contingent on incomplete homework of remove a preferred toy because of a tantrum. A teacher may withhold 334 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics recess from an aggressive student or restrict the entire class’ access to a special activity because of its failure to follow a classroom rule. Applied behavior analysts tend to avoid the use of punishment because (1) there are many reinforcement-based procedures at that are typically effective, (2) punishment can result in negative side effects,34 and (3) punishment procedures are sometimes misused.35 Nevertheless, the application of punishment occurs in everyday life and, given that positive reinforcement is not always effective, it may sometimes be necessary in clinical practice as well. When punishment procedures are used, they are typically implemented together with positive reinforcement procedures, thereby providing differential consequences for appropriate versus inappropriate behavior (i.e., right and wrong). Punishment should be introduced only after positive reinforcement has been shown to be insufficient. Perhaps the most ubiquitous form of punishment by contingent stimulation in everyday life is a negative verbal statement. Adults often issue warnings or reprimands following a child’s misbehavior. Such negative statements have inconsistent effects, especially when presented to children with developmental disabilities. Sometimes such remarks suppress misbehavior immediately; at other time they serve as positive rein forcers by way of negative attention. The manner in which verbal disapprovals are stated may partially determine their degree of effectiveness. Private reprimands delivered softly may be more effective than loud reprimands voiced in front of others.36

Time-out The other approach to punishment is contingent withdrawal and includes time-out and response cost. Time-out is removal form time-in (access to positive reinforcement).37,38 During time-out the child is not permitted to engage in preferred activities. A variety of time-out procedures have been shown to be effective especially with children aged 2 to 8 years. These include brief chair or corner time-out, brief isolation, partial removal, from preferred activities marked by continent observation of ongoing events from the sideline, and passively precluding the child’s opportunity to receive reinforcement for problem behavior.39 For time-out to be effective, all positive reinforces that support the problem behavior must be removed. Time-out makes a difference only if the child has been removed from a time in; it does not work if the child Behavior Analysis in Children 335 has simply been removed from an aversive situation. For example, a mistaken use of time-out occurs when teachers remove children from classrooms because of misbehavior assuming that such disciplinary dismissals constitute time-out. Suspensions may actually result in increase in disruptive behavior if the child is motivated to escape from or avoid an aversive academic assignment. If this is the case, what is considered to be time-out for problem behavior is actually negative reinforcement of this behavior. Time-out, similar to other disciplinary tactics, should be administered as consistently as possible. Time-out is more effective if it is consistently applied contingent on only one to three problem behaviors at any given point of time. Once the targeted problem behavior abates, new misbehaviors can be selected for treatment.

Response Cost Response cost entails the loss of a positive reinforcer or the imposition of a penalty when the child misbehaves. With children, response cost usually involves removal of a privilege or part of an allowance. The precise association of cost with each type of infraction is determined in advance and applied immediately and consistently. Response cost is often administered in conjunction with positive reinforcement through systematic protocols such as token economies or point systems. According to these protocols, positive reinforcement is dispensed for specified appropriate behavior and taken away for specified problem behavior. In order for such differential consequences to be effective, children with developmental disabilities often must have an opportunity to sample available reinforcers and fines, so they can better discriminate the contingencies in effect (i.e., the relationships between what they do and what happens as a result). Small fines are often effective, unless the patient has a prior history of being fined heavily. Although response cost, similar to other punishment procedures, may engender avoidance, aggression, or resentment on the part of the patient, it is usually easy to implement and often produces rapid reductions in problem behavior.

Overcorrection Another punishment procedure that involves a penalty for inappropriate behavior is over correction. This intervention consists of two components, restitution and positive practice. Restitution pertains to correcting any 336 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics damage caused by the misbehavior. Through positive practice, the child is required to exhibit an acceptable alternative behavior. Over correction, in conjunction with other treatments, has been used successfully to teach independent skills, such as toileting. For over correction to be effective, both components must be related directly to the problem behavior. Also, the procedure needs to be applied immediately after the problem behavior, without pause or fanfare, while child’s preferred activities are withheld until the procedure is completed.

Guidelines for the Punishment When administering a protocol that involves punishment, it is important to adhere to the following guidelines: 1. Ensure that the child does not escape from a warranted punishment. Punishment is not effective if the child is permitted to ignore a verbal reprimand or play with a preferred toy during time-out. 2. Punishment is more likely to work if it is first applied at full strength, rather than increased gradually. This guideline does not justify overuse of punishment, however, such as extended time-outs or excessively large fines. 3. If punishment has been shown to be necessary for a given behavior, one should deliver the identified consequence each time the behavior occurs. 4. When punishment is indicated, one should apply it immediately. This better enables the child to understand what behavior is being punished. 5. Any reinforcement that supports the problem behavior should be eliminated. Rather than using coercive techniques such as threats, scolds, nags, or lectures that may inadvertently reinforce the unwanted behavior, one should simply state the contingency and enforce it. 6. One must be careful that the delivery of punishment is not associated with the delivery of reinforcement. For example, providing punishment and then apologizing later for it sends a confusing message and may lead to increased misbehavior. 7. Extensive of prolonged use of punishment should be avoided. If used a lot, punishment loses its effectiveness. 8. Punishment should be provided early in the child’s approach toward unacceptable behavior. Most severe behaviors have readily identifiable and predictable precursors, which if interrupted may prevent these Behavior Analysis in Children 337

behaviors. For instance, punishing a child when he or she reaches for a lighter is preferable to punishing a child after a fire has been set.

Record Keeping and Trouble Shooting Assessment proceeds throughout all phases of treatment. Optimally, the pediatrician teaches the parents, and sometimes the child, to record their observation of the child’s target behavior as recommended interventions are practiced at home.40 Contact with the parents can be sustained by way of telephone contacts or in consultation chamber. When the parents have implemented the recommended procedures over a 1 to 3 week interval, they are expected to share records. These records are reviewed in conjunction with the parents’ verbal reports. When indicated, the assessor revises recommended procedures or provides additional instruction.

OUTCOME OF BEHAVIOR MANAGEMENT Effective management based on the principles and procedures of applied behavior analysis often results in (1) acquisition of prosocial skills and (2) concomitant reductions in problem behaviors. The key beneficiaries are the child, parents, and teacher. Although the impetus for referral often arises from the occurrence of excessive misbehavior by the child, the focus of behavior analysis typically shifts to teaching the child, parents, and teacher’s new skills. Usually, the child misbehaves in order to get what he or she wants or to avoid or escape from what he or she does not want to do. The behavior analyst teaches the child how to achieve these aims through acceptable behavior. In so doing, the child learns how to behave more adoptively. Concurrently, the child’s parents and teachers learn how to guide the child to behave more appropriately in order to facilitate the child’s progress. These adult care providers also learn how to prevent occurrences of child misbehavior and how to discipline the child in a consistent, supportive manner if and when problem behavior arises. When the practitioner of behavior analysis carefully identifies and analyzes the conditions under which the child misbehaves and skillfully designs a contingency management program to replace the child’s inappropriate behavior with more adaptive alternatives, behavior analysis is successful. 338 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

KEY LEARNING POINTS 1. As pediatricians are serving children with developmental disabilities, it is important to study and practice applied behavior analysis. 2. Within a behavior analytic framework, the pediatrician views the child’s aberrant performance as being a constellation of behavioral excesses and skill deficits that serve a functional purpose, rather than as being a symptom of some underlying illness or intrapsychic mechanism. 3. The pediatrician can counsel the child, parents, and teachers to rearrange their environment, with an emphasis on how they can behave differently toward one another in specific situations, so as to alter the target behavior. 4. When the child’s problem behaviors are relatively severe or intractable, the pediatrician can refer the patient and family to a behavior specialist while continuing to support efforts to improve the child’s overall health and adjustments.

REFERENCES 1. Pressler RM. Behavior problems in children with epilepsy. In: David TJ (ed): Recent Advances in Pediatrics–23. London: Royal Society of Medicine Press 2006:171-183. 2. Maston JL, Mulick J. Handbook of mental retardation. Elmsford, NY: Pergamon Press 1983 3. Miller LK. Principles of Everybody Behavior Analysis, 2nd edn. Monterry, CA: Brooks Cole Publishing Co 1980. 4. Kazdin AE. Single-case Research Designs: Methods for Clinical and Applied Settings. New York: Oxford University Press 1982. 5. American Academy of Child and Adolescent Psychiatry. Practice parameters for the psychiatric assessment of children and adolescents. J Am Acad Child Adolesc Psychiatry 1995; 34:1386. 6. American Academy of Child and Adolescent Psychiatry. Practice parameters for the psychiatric assessment of infants and toddlers. J Am Acad Child Adolesc Psychiatry 1997; 36(suppl): 21S. 7. American Academy of Child and Adolescent Psychiatry. Practice parameters for child custody evaluation. J Am Acad Child Adolesc Psychiatry 1997; 36(suppl): 57S. 8. Angold A, Messer SC, Stang lD, Farmer EM, Costello EJ, Burns BJ. Perceived parental burden and service use for child and adolescent psychiatric disorders. Am J Public Health. 1998; 88:75. 9. Cox AD. Interviews with parents, In: Rutter M, Taylor E, Hersov L (ed): Child and Adolescent Psychiatry: Modern Approaches, 3rd edn. Oxford: Blackwell Scientific 1994. Behavior Analysis in Children 339

10. Levy SE. Pediatric evaluation of the child development delay. Child Adolesc Psychiatr Clin North Am, 1996; 5:809. 11. Chess S, Thomas A. Temperament. In: Lewis M (ed): Child and Adolescent Psychiatry, 2nd edn. Baltimore: Williams and Wilkins 1996. 12. Lewis M. Psychiatry assessment of infants, children, and adolescents. In: Lewis M (ed): Child and Adolescent Psychiatry, 2nd edn. Baltimore: Williams and Wilkins 1996. 13. Greenspan SI, Greenspan NT. The Clinical Interview of the Child, 2nd edn. Wahington DC: American Psychiatric Press 1991. 14. Angold A. Clinical interviewing with children and adolescent. In: Mutter M, Taylor , Hersov L (eds) Child and Adolescent Psychiatry: Modern Approaches Oxford: Blackwell Scientific 1994. 15. Costello EJ, Angold A. Scales to assess child and adolescent depression: Checklists, screens, and tests. J Am Acad Child Adolesc Psychiatr 1999; 38:121. 16. Ouvrier RA, Goldsmith RF, Ouvrier S, Willians IC. The value of the Mini-Mental State Examination in Childhood. A preliminary study. J Child Neuro, 1993; 8:145. 17. Racusin GR, Moss NE. Psychological assessment of children and adolescents. In: Lewis M (ed): Child and Adolescent Psychiatry, 2nd edn. Baltimore: William and Wilkins 1996. 18. Bird HR, Kestenbaum CJ. A semistructured approach to clinical assessment. In: Kestenbaum CJ, Williams DT (eds): Handbook of Clinical Assessment of Children and Adolescent. New York: New York University Press 1988. 19. Herjanic B, Reich W. Development of a structured psychiatric interview for children: An agreement between child and parent on individual symptoms. J Abnorm Child Psychol 1982; 10:307. 20. Hodges K. Structured interviews for assessing children. J Child Psychol Psychiatry 1993; 34:49. 21. Sparrow SS, Balla DA, Cicchetti DV. The Vineland Adaptive Behavior Scales: Interview Editions. Circle Pines MN: American Guidance Service 1984. 22. Sparrow SS, Carter AS, Racusin G, Morris R. Comprehensive psychological assessment throughout the lifespan: A development approach. In: Manual of Developmental Psychopathology, Vol 1 D Cicchetti, DJ Cohen (Eds): Wiley, New York, 1995 23. Kestenbaum CJ. The clinical interviews of child. In: Weiner JM (ed): Textbook of child and Adolesc Psychiatry (2nd edn). Washington DC: American Psychiatric Press, 1991. 24. Zametkin AJ, Ernst M, Silver R. Laboratory and diagnostic testing in child and adolescent psychiatry: A review of the past 10 years. J Am Acad Child Adolsc Psychiatr 1998; 37:4645. 25. Achenbach T, Edelbrock C. Manual for the Child Behavior Checklist and Revised Child Behavior Profile. Burlington: University of Vermont Press 1983. 26. Balsam PD, Bondy AS. The negative side effects of reward. J Appl Behav Anal 1983;16: 283-296. 27. White GD, Neilson G, Johnson SM. Timeout duration and the suppression of deviant behavior in children. J Appl Behav Anal 1972;5:111-120. 28. Skinner BF. Science and Human Behavior. New York, Macmillan, 1953. 29. Kazdin AE. Symptom substitution, generalization, and response covariation: Implications for psychotherapy outcome. Psychol Bull 1982; 91: 349-365. 30. Kazdin AE. Psychiatric diagnosis, dimensions of dysfunction, and child behavior therapy. Behaviorl Ther 1983;14:73-99. 31. Kazdin AE. Selection of target behaviors: The relationship of the treatment focus to clinical dysfunction. Behavior Assesst 1985;7: 33-47. 340 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

32. Sulzer-Azaroff B, Mayer GR. Applying behavior-analysis procedures with children and youth. New York: Holt, Rinehart and Winston, 1977. 33. Iwata BA. Negative reinforcement in applied behavior analysis: An emerging technology. J Appl Behav Anal 1987;20: 361-378. 34. Matson JL, Dilorenzo TM. Punishment and its alternatives: A new perspective for behavior modification. New York: Springer-Verlag, 1984. 35. Kazdin AE. Behavior modification in applied settings. Homewood, IL: Orsey Press 1984. 36. O’Leary KD, Kaufman KF, KassR, et al. The effects of loud and soft reprimands on the behavior of disruptive students. Exceptional Children 1970; 37: 45-155. 37. Christophersen ER. Incorporating behavioral pediatrics into primary care. Pediatr Clin North Am 1982;29: 261-296. 38. Forehand R, McMohan RJ. Helping the noncompliant child: A clinician’s guide to parent training. New York: Guilford Press, 1981. 39. Foxx RM, Shapiro ST. The time-out ribbon: A nonexclusionary timeout procedure. J Appl Behav Anal 1978;11: 125-136. 40. Finney JW, Weist MD. Behavioral assessment of children and adolescents. Pediatr Clin North Am 1992;39: 369-378. RAP Special Volume 19: Developmental and Behavioral Pediatrics

25 Play Therapy

B Vishnu Bhat, Rathi Sharmila R

INTRODUCTION Play is the child’s natural medium of self expression.1 It is an opportunity which is given to the child to play out his feelings or problems just as in certain types of adult therapy where an individual ‘talks out’ his difficulties. Play is the natural way that children understand their world, express thoughts and feelings, develop social skills and learn self mastery.2 It is a medium for expressing feelings, exploring relationships, describing experiences and disclosing wishes. Play therapy can be a beautiful and effective way to help children and works for children as counseling or psychotherapy does for adults. Child centered play therapy helps children work through emotional, psychosocial and behavioral difficulties and helps to address family problems. In child centered play therapy, a relationship develops between the child and the therapist. The therapist enters the child’s world finds the needs of the child and helps the child.

PLAY THERAPY RESEARCH Play therapy is a well thought out, philosophically conceived, developmentally based, and research-supported approach to help children cope with and overcome the problems they experience in the process of living their lives. Play therapy has been demonstrated to be an effective therapeutic approach for a variety of children’s problems as shown in Table 25.1. 342 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 25.1: Conditions where play therapy is useful

Learning Psychosocial Psychiatry Health related

• Learning • Abuse and • Mentally • Burn victims disabilities neglect challenged • Chronic illness • Reading • Aggression and children • Hospitalization difficulties acting out • Dissociation and • Traumatization • Self-concept • Attachment schizophrenia • Withdrawn and self-esteem difficulties • Emotionally children • Social adjustment • Selective mutism disturbed problems • Deaf and physically children • Speech challenged children • Autism difficulties • Enuresis and encopresis problems • Fear and anxiety • Grief

RATIONALE FOR PLAY THERAPY Because children’s language development lags behind their cognitive development, they communicate their awareness of what is happening in their world through their play. In play therapy, toys are viewed as the child’s words and play as the child’s language—a language of activity. Play therapy is to children is what counseling or psychotherapy is to adults. In play therapy the symbolic function of play is used to express their inner world.3 All emotionally significant experiences can be expressed comfortably and safely through the symbolic representation the toys provide. The use of toys enables children to transfer anxieties, fears, fantasies, and guilt. In the process, children are safe from their own feelings and reactions because play enables children to distance themselves from traumatic events and experiences. Therefore, children are not overwhelmed by their own actions because the act takes place in fantasy. By acting out through play a frightening or traumatic experience or situation symbolically, and perhaps changing or reversing the outcome in the play activity, children move toward an inner resolution, and then they are better able to cope with or adjust to problems.4 In a relationship characterized by understanding and acceptance, the play process also allows children to consider new possibilities not possible in reality, thus greatly expanding the—expression of self. In the safety of Play Therapy 343 the play therapy experience, children explore the unfamiliar and develop a knowing that is both experiential-feeling and cognitive. It can then be said that through the process of play therapy, the unfamiliar becomes familiar, and children express outwardly through play what has taken place inwardly. A major function of play in play therapy is the changing of what may be unmanageable in reality to manageable situations through symbolic representation, which provides children opportunities for learning to cope.

DIFFERENT FORMS OF PLAY THERAPY5 1. Non-directive play therapy 2. Directive play therapy 3. Individual play therapy 4. Group play therapy 5. Child parent relationship therapy (CPRT).

Non-directive Play Therapy This is the conventional play therapy which is being used. Therapist does not intervene and the child is allowed to explore all the possibilities by himself. The therapist just observes and interprets.

Directive Play Therapy So far most of the play therapy has focused on non-directive techniques, which allow a child to develop his own resources for self control. They don’t work well with children like of attention deficit hyperactivity disorder (ADHD). Directive play therapy is a form of play therapy where in structured play is used to help children overcome characteristics of attention deficit hyperactivity disorder (ADHD) and self-defeating attitudes and behaviors, as well as heal from traumas such as sexual abuse and neglect. These techniques help children recover much more quickly than other treatment methods, because form of play therapy includes parents in the play therapy sessions and incorporates their help in the treatment.

Individual Play Therapy It can help children between 2-8 years old experiencing psychosocial, emotional, behavioral and/or developmental difficulties. It is also offered 344 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics for children 9-11 years old, where there has been early or recent trauma in the child’s life and autism.

Group Play Therapy In group play therapy generally small groups are used and play therapy sessions with 2-3 children are usually arranged and used for some sibling groups. It is used for social skills development, self esteem, self-expression and to assist with psychosocial and adjustment difficulties.

Child Parent Relationship Therapy (CPRT) In this form of play therapy individual or group sessions are conducted and parents/careers are counseled. They are taught how to support the emotional, psychosocial and developmental growth of the child and to help strengthen and nurture the parent/child relationship.

THE PROCESS OF PLAY THERAPY Given the opportunity, children will play out their feelings and needs in a manner or process of—expression that is similar to that for adults. Although the dynamics of—expression and the vehicle for communication are different for children, the expressions (fear, satisfaction, anger, happiness, frustration, contentment) are similar to those of adults. Children may have considerable difficulty in trying to tell what they feel or how their experiences have affected them. If permitted, however, in the presence of a caring, sensitive, and empathetic adult, they will reveal inner feelings through the toys and materials they choose what they do with and to the materials, and the stories they act out.

ROLE OF THERAPIST In play therapy the child and the therapist forms a dyad. The play therapy process can be viewed as a relationship between the therapist and the child in which the child utilizes play to explore his or her personal world and also to make contact with the therapist in a way that is safe for the child. Play therapy provides an opportunity for children to live out, during play, experiences and associated feelings. This process allows the therapist to experience, in a personal and interactive way, the inner dimensions of the child’s world. This therapeutic relationship is what provides dynamic Play Therapy 345 growth and healing for the child. The role of therapist is very important for a successful outcome in play therapy. The therapist is desired to have the following qualities. • Must develop a warm and friendly relationship with the child. • Accepts the child as she or he is. • Establishes a feeling of permission in the relationship so that the child feels free to express his or her feelings completely. • Is alert to recognize the feelings the child is expressing and reflects these feelings back in such a manner that the child gains insight into his/her behavior. • Maintains a deep respect for the child’s ability to solve his/her problems and gives the child the opportunity to do so. The responsibility to make choices and to institute change as the child’s. • Does not attempt to direct the child’s actions or conversations in any manner. The child leads the way, the therapist follows. • Does not hurry the therapy along. It is a gradual process and must be recognized as such by the therapist. • Only establishes those limitations necessary to anchor the therapy to the world of reality and to make the child aware of his/her responsibility in the relationship. Because the child’s world is a world of action and activity, play therapy provides the therapist with an opportunity to enter the child’s world. The child is not restricted to discussing what happened; rather, the child lives out at the moment of play the past experience and associated feelings. If the reason for the child was referred to the therapist is aggressive behavior, the medium of play gives the therapist an opportunity to experience the aggression and also to help the child learn self-control by responding with appropriate therapeutic limit-setting procedures. In play therapy, whatever the reason for referral, the therapist has the opportunity to experience and actively deal with that problem in the immediacy of the child’s experiencing.

PLAY THERAPY METHOD Different methods and different ideas are being used in play therapy. Most commonly used ones are art, story telling, creative visualization, dance, drama therapy, music, sand tray, puppets and mask. 346 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

TOYS AND MATERIALS6 Although desirable, a fully equipped playroom is not essential for children to express themselves. What is important is that children have ready access to play materials selected for the purpose of encouraging—expression. Therefore toys and materials which encourage children’s—expression or exploration of their needs, feelings, and experiences, should be selected. Play therapy is not used as a way to pass the time or to get ready to do something else. The purpose is not to engage the child’s hands while trying to elicit some verbal—expression from the child’s mouth. Consequently, careful attention should be given to selecting play materials that aid in the following: Exploration of real life experiences—expression of a wide range of feelings, testing of limits, expressive and exploratory play, exploration and—expression without verbalization.

SETTING LIMITS IN PLAY THERAPY4,5 Limits in play therapy have both therapeutic and practical benefits in that they preserve the therapeutic relationship, facilitate the child’s opportunities to learn self-responsibility and self-control, among many other dimensions, and provide the child and the therapist with a feeling of emotional security and physical safety. This feeling of emotional security enables a child to explore and express inner emotional dimensions that perhaps have remained hidden in other relationships. Play therapy is not a completely permissive relationship because children do not feel safe, valued, or accepted in a relationship without boundaries. The play therapy relationship has minimal limits. Messiness is accepted, exploration is encouraged, neatness or doing something in a prescribed way is not required, and persistent patience is the guiding principle. The child’s desire to break the limit is always of greater importance than actually breaking a limit. Because play therapy is a learning experience for children, limits are not set until they are needed. The child cannot learn self-control until an opportunity to exercise self-control arises. Limits are worded in a way that allows the child to bring him or herself under control. The objective is to respond in such a way that the child is allowed to say “No” to self. Play Therapy 347

FUTURE TRENDS IN PLAY THERAPY The field of play therapy is growing and is now represented by the association for Play Therapy, an international professional organization. Increasing numbers of elementary school counselors and therapists in private practice and agencies are incorporating play therapy into their work with children. There is a trend in family therapy to address social and emotional values of developmental as well as family group session issues. In filial therapy, parents are trained to use play therapy procedures with their children. This method is well researched and has proven to be effective in ameliorating children’s problems through enhancing the parent-child relationship. KEY LEARNING POINTS • Play therapy is based on developmental principles. • It provides, through play, developmentally appropriate means of— expression and communication for children. • Skill in using play therapy is an essential tool for mental health professionals who work with children. • Therapeutic play allows children the opportunity to express themselves fully and at their own pace with the assurance that they will be understood and accepted.

REFERENCES 1. Jeroma L, Singh Ph D. Cognitive and affective implication of imaginative play in childhood. In: Melvin Lewis (ed): Child and Adolescent Psychiatry a Comprehensive Textbook, 2nd edn. Pennsylvania: Williams and Wilkins Company, 1996; 210-211. 2. Henry P, Capapillo MD. Use of play in psychodynamic psychotherapy. In: Melvin Lewis (ed): Child and Adolescent Psychiatry in Child and Adolescent Psychiatry a Comprehensive Textbook, 2nd edn. Pennsylvania: Williams and Wilkins, 1996; 809- 815. 3. Sadock BJ, Sadock VA. Kaplan and Sadock’s Synopsis of Psychiatry. Behavioral Sciences/Clinical Psychiatry, 9th edn. Philadelphia: Lippincott Williams and Wilkins, 2003;215-216. 4. Kelvin John O’connor. Sue Ammen Play therapy—Treatment and Planning Interventions. The Ecosystemic Model and Workbook. San Deigo: Academic Press, 1997:1-13. 5. www.ptuk.com (PTUK)—official web site for play therapy in UK. Play therapy United Kingdom, Principles and Forms of Play Therapy. RAP Special Volume 19: Developmental and Behavioral Pediatrics

Developmental Aspects 26 of Self-esteem

Suraj Gupte, WE Wilson, Lilly Wilson

INTRODUCTION Also termed “self-concept” and “self-worth”, it is defined as “estimation or view of self, generally thought to influence feelings and behaviors”. Child’s (especially adolescent’s) personal successes, expectations and appraisals of the views others hold towards him are thought to influence his personal appraisal.1-3 According to one school of thought, self-esteem is the “difference between what one actually thinks and feels about oneself (“perceived self”) and one’s ideal-self”. The greater the difference, the lower is the self-esteem. The fundamental ingredient of self-esteem includes the respect and caring that we show towards our friends, neighbors and others. This concept is best advanced in the definition offered by the California Task Force to Promote Self Esteem and Personal and Social Responsibility. The Task Force defines self-esteem not only in terms of appreciating one’s own worth and importance but also having the character to be accountable for oneself and to act responsibly towards others. Self-esteem is a “drive” that guides and motivates our actions. In turn, it is influenced by the outcome of our actions. High self-esteem is accompanied by hope and confrontation of challenges. A reciprocal process is invariably in operation. Low self-esteem is associated with despair and avoidance of challenges. Insecurity and doubt are central to the latter. Developmental Aspects of Self-esteem 349

SELF-ESTEEM AT DIFFERENT PERIODS OF CHILDHOOD AND ADOLESCENCE

Middle Childhood According to Needlman,1 as the child approaches adolescence, his self- consciousness begins to increase. The focus is more on external characteristics which, later (towards latter adolescence) shifts to introspection. It is absolutely normal for an early teenager to scrutinize his appearance and to feel that he is being stared by everybody.

Middle Adolescence This period is known for experimentation with different personae, changing styles of dress, group of friends and interests from month to month. Inner turmoil is common. Influence of perr groups is diminished.

Late Adolescent Introspection is typical of this phase. The teenagers tends to be idealistic but, frequently, intolerant of opposing views.

FACTORS INFLUENCING SELF-ESTEEM2-4

Inborn Temperament Children are arbitrarily classified into 3 categories depending on their inherent temperament: 1. The easy child: more active, hyperactive to sensory input, more responsive to interactions with others, easily satisfied and content. 2. The slow-to-warm child: less active, less sensitive to sensory input, less responsive to interactions with others, not easily satisfied and content. 3. The difficult child: Inactive, refractory to sensory inputs, extremely cautious to interactions with others, unhappy, insatiable and discontented. Yet, it is well established that many children do demonstrate attributes from at least two of the categories.

Environments Environmental influences, especially how parents react to child’s temperament, have an immense bearing on child’s self-esteem. 350 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Achievement-oriented parents are likely to blame the child for being difficult, lazy and without motivation. They fail to appreciate that, though the child is leaving no stone unturned to succeed but his efforts are being nullified or, at least, diluted because of developmental problems. Parent’s reaction further lowers child’s self-esteem. This, in turn, further elicits reactions from environment. This child-environment interaction points to the need for adults to recognize, respect and accept the unique makeup of each and every child. Nevertheless, remember, it only means that the parent (yes, teacher too) should see the child for what he is rather than what he should be. Unless and until this acceptance comes his way, child cannot be viewed for his actual strength and limitations, nor does he receive emotional support to try new ways of behaving.

ATTRIBUTION THEORY OF SELF-ESTEEM According to this theory, children with high esteem take credit for their achievements, viewing their success as a result of their own efforts, resources and abilities. They feel a sense of control over developments transpiring in their lives. Such a child, in case of a failure, responds by a positive reaction in some such words: “I am sure capable of doing better. Let me put up greater efforts and show my worth next time.” In contrast, children with low self-esteem are inclined to ascribe their achievements to a sheer chance or luck. They hardly think of having played a role in achieving success. This weakens their confidence in accomplishing success again and may, actually end up with failure. His response to failure is like this: “The paper setter is lousy, setting all that is unimportant.” The theory is helpful in designing strategy for strengthening (reinforcing) self-esteem of the child by creating an environment in schools and homes so that children assume increasing sense of ownership and responsibility for the events transpiring in their lives. Creating an environment that reinforces the belief in children that mistakes often form the very basis for learning, that mistakes are not only accepted but expected.

MANIFESTATIONS Low self-esteem is manifested by several self-defeating coping behaviors. Developmental Aspects of Self-esteem 351

Quitting This refers to biding good-bye when the child is frustrated, cannot master a task or win a game. He may offer a lame excuse like “What a boring task!” A noteworthy point is that he has already started off with the task but retracts when he finds the things not going his way. An example is a child who enters a sports competition and withdraws half way offering an excuse that the judgement is likely to be partial. Avoiding This is in a way similar to quitting but the child refuses to undertake the task at all. He may have a mind to take part in a declamation contest but at the eleventh hour does not go ahead apprehending to cut a sorry figure. Cheating Being certain of losing , the child may begin to change rules of the game from time to time. Clowning and Regressing The child, lacking confidence or wanting to camouflage his failure in a given situation, may attempt to attract attention by acting out in a clownish or silly manner. Controlling Since the child with low self-esteem has only a limited control over his own life and thus feels helpless, he may take up the role of commanding others. Aggressiveness and Bullying Coping may be in the form of striking out at others – belittling or scapegoating others. Passive Aggressiveness This way of exerting control consists in promising to meet certain responsibilities which are, of course, not discharged out of forgetfulness. Denying This coping behavior of the child with low self-esteem consists in denying their weaknesses , limitations and vulnerabilities. Rationalizing The child does not believe he can succeed and offers excuses for perceived difficulties and failure, often blaming others for unsatisfactory outcome. Impulsiveness The child quickly finishes a challenging task or burdensome chore.

REMEDIAL INTERVENTIONS Responsibility and Contribution It is important to provide opportunity to the child for taking responsibility, especially contribution to school, home or neighborhood. Availability of 352 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics contributory activities and responsibilities drives home the vital message that he is a capable individual having something of value to offer. Such a message is bound to enhance child’s self-esteem and motivation. Brooks mentions the following examples of this strategy: 1. An elementary school child who was turned off to school and whose self-perceived island of competence was taking care of his pet dog. Consequently, he was recruited as the “pet monitor” of the school, a position that involved taking care of various pets at school, authoring a brief manual about pet care that was bound and placed in the school library (typically this boy did not like to write, but with the encouragement of his teacher he wrote the pet manual because he felt he something of value to offer), and lecturing to all classrooms about the care of pets. 2. A hyperactive high school student who was asked to help by becoming the “attendance monitor” of the school, a job that required walking through the halls during homeroom time (he almost never made it to homeroom anyway) to check if thee was a teacher present in each classroom and then to report back to the principal (this activity utilized the boy’s high activity level in a positive way and helped him to become more settled and successful at school. 3. A sixth grade girl whose low-esteem was masked by her oppositional behavior and her need to control situations. Because she enjoyed interacting with young children, her self-esteem was enhanced by asking her to tutor first and second grades in school and do some baby- sitting in the neighborhood (these kinds of responsibilities were also in accord with her need to be in control, but this need was now being expressed in a more constructive fashion. 4. Classrooms that rely on operative learning so that students gain experience working together and helping each other.

Choices, Decisions and Problem Solving The child should be provided opportunities to make choices and decisions and to solve problems that influence his life, of course, in keeping with the child’s developmental level and interests. Examples cited by Brooks include 1. A first-grade perfectionistic child with learning disabilities who refused to do any writing because he could not write his letters perfectly. He Developmental Aspects of Self-esteem 353

responded positively when the teacher offered him the option of choosing one ““vacation day”” each week when he could substitute a nonwriting activity during writing time. 2. Students who were given a choice by class vote of what day to take a test. 3. A junior high school girl who often engaged in struggles with her parents about bedtime and was permitted to select one evening each week when she could stay up 30 minutes later. 4. A group of students who did research about and decided on charity for which they would raise money through abake sale. 5. A pediatrician permitted an anxious child to choose whether to have his eyes or ears examined first.

Encouragement and Positive Feedback Self-esteem is indeed nurtured when child receives appreciation, encouragement, and positive feedback. Even a small encouraging gesture leads to a positive effect.3-6 Brook2 cites the following examples 1. A teacher writing a brief note on a child’s paper praising the child’s efforts. 2. A pediatrician sending a postcard to a child (as long as this is a true feeling). 3. A parent finding a “special time” each week to spend with each child in the family individually. This time might be for 15 to 30 minutes each evening or for a longer period once a week (e.g., for dinner outside the home). A 5-year-old patient believed that his father was disappointed and angry with him (an accurate assessment). Their relationship improved noticeably when the father scheduled a “private time” once a week with his son, which involved going before school to a donut shop for breakfast. 4. A recognition assembly in school in which a variety of student achievements and contributions were noted.

Self-Discipline A secure and comfortable sense of self-discipline is important for developing high self-esteem. Self-discipline is most effective when laden without undue pressure or feeling of guilt. The goal must be to teach and educate the child. In no case, it should aim at ridiculing him or punishing him ruthlessly. Examples, again borrowed from Brook are 354 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

1. A teacher who at the beginning of the year asks for students what rules they think should exist in the classroom, why rules are necessary, and what should the consequences be if someone forgets to abide by a rule. Skillfully involving students in the creation of both rules and consequences does not lead to chaos but rather to increased student understanding of the importance of limits and guidelines and greater motivation to follow the rules that they themselves have helped to establish. 2. The hyperactive, young adolescent referred to earlier whose behavioral problems at school were lessened when asked to be the “attendance monitor” (an illustration of preventing a discipline problem from emerging by providing a concrete activity to reinforce self-esteem). 3. The assistant principal of a middle school who asked students to write a brief essay while serving detention (they were given the choice of over 30 topics). The topics were all relevant to situations in students’ lives so that in writing about a topic the students were prompted to reflect upon their behavior. 4. Parents of a preschool child who reported that they were having difficulty getting their child to sleep. They found themselves yelling at him, which served only to make matters more tense. The child started having terrible nightmares and was frightened of going to bed. Greater understanding and empathy on the part of the parents and the use of a nightlight as well as placing a photo of the parents net to the child’s bed (interestingly, the child’s idea) greatly lessened his anxiety and misbehavior.

Condoning Mistakes and Failures The development of high esteemed is closely related to condoning mistakes and failures. Remember, mistakes should be regarded as experiences to learn from rather than feel defeated by.3-5 Brooks2 gives the following examples 1. Parents who do not overreact to mistakes their children make, who do not discourage their children by saying, “I told you it wouldn’t work!” or “If you would only use your brains!” Instead, assisting children to see what can be learned from the mistakes is a much more productive approach. Developmental Aspects of Self-esteem 355

2. A teacher who during the first or second day of the new school year asks students, “Who feels they will probably make a mistake in class this year or not understand something the first time?” and before anyone can respond the teacher raises her own hand. The teacher can then ask the class why they thought she asked this question and can use their responses as a launching pad to discuss how fears of making mistakes and being humiliated interfere with offering opinions and answers and learning. To acknowledge openly the fear of failure renders it less powerful and less destructive. 3. A teacher who on the first day of class placed an empty jar on her desk and distributed small rocks to her students. She told them that whenever a student “caught” her making a mistake the student was to place one of the rocks in the jar, and when the jar was filled she would provide the class with a treat. What a powerful message about feeling comfortable with making mistakes. 4. Adults who share some of their own experiences of growing up and what they personally learned from failing. 5. Adults who emphasize what children are able to do and what they know rather than what they cannot do and what they do not know. For instance, the seemingly simple practice of teachers scoring tests by adding points for correct answers rather than subtracting points (often in red ink) for incorrect answers would focus on positive. Such a shift in focus might help some children face what they do not know with increased confidence.

KEY LEARNING POINTS 1. Self-esteem is “one’s personal evaluation or view of self, generally thought to influence feelings and behaviors”. 2. According to the self-attribution theory, children with high esteem take credit for their achievements, viewing their success as a result of their own efforts, resources and abilities. They feel a sense of control over developments transpiring in their lives. 2. Factors influencing self-esteem include inborn temperament and environments. 3. Manifestations include quitting, avoiding, cheating, clowning/ regressing, controlling, rationalizing, aggressiveness, denying, impulsiveness 356 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

4. Remedial measures include • It is important to provide opportunity to the child for taking responsibility, especially contribution to school, home or neighborhood • The child should be provided opportunities to make choices and decisions and to solve problems that influence his life, of course, in keeping with the child’s developmental level and interests • Self-esteem is indeed nurtured when child receives appreciation, encouragement, and positive feedback • A secure and comfortable sense of self-discipline is important for developing high self-esteem • Condoning mistakes and failures

REFERENCES 1. Needlman RD. Growth and development. In: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, 17th edn. Philadelphia: Saunders 2004: 23-66. 2. Brooks RB. Self-esteem during the school years. Pediatr Clin North Am 1992;39:537-549. 3. Bednar RL, Wells MG, Peterson SR. Self-esteem. Paradoxes and Innovations in Clinical Theory and Practice. Washington, DC: American Psychological Association, 1989. 4. Wilson WE, Henry J. Self-esteem in children and teenagers. In: The Teenager, 3rd edn. London: Smithsons 2006:234-248. 5. Wilson WE. Underestimated difficulties of adolescents. Chr Psychol 2002;7:24- 29. 6. Gupte S. Self-esteem and the teenager. Eur Bull Dev Pediatr 2004;11:78-83. RAP Special Volume 19: Developmental and Behavioral Pediatrics 27 Child Abuse and Neglect

Devendra Mishra, Kiran Aggarwal

INTRODUCTION Child abuse in all its forms is far more prevalent in India than our society has been willing to acknowledge. This is because the focus has been more on basic survival, needs of health, nutrition, shelter and education; consequently there has been very little attention paid to the abuse of children and its impact. Further, traditional factors such as the sanctity of the family, prerogative of the adult caregivers, and the view of children as parental property have denied intervention and inspection by society. The abuse thus remains underreported. Even among the health services provider, there is only a superficial understanding of the definition compounded by a paucity of knowledge, and hardly any awareness regarding the extent and severity of abuse among children in India. There is no legislation on child abuse in India and no provision of mandatory reporting. Therefore, when it comes to dealing with a case, a pediatrician is supposed to act on his/her own. However, with the increasing awareness about child abuse, there has now been a steady increase in the number of reported cases.

DEFINITION World Health Organization (WHO)1 defines child abuse or maltreatment as “all forms of physical and/or emotional ill-treatment, sexual abuse, neglect or negligent treatment or commercial or other exploitation, resulting in actual or potential harm to the child’s health, survival, development or dignity in the context of a relationship of responsibility, trust or power.” Child abuse is a universal problem that occurs across all racial, and socioeconomic groups. The 358 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Fig. 27.1: The tip of the iceberg of child abuse* implications of the physical, developmental, social, economic and psychological effects of child abuse are significant, both in the short-term and the long-term. Definitions of what constitutes child abuse and neglect will vary between countries and even communities. However, a number of studies suggest that persons across cultures share common working definitions of abuse.1 Number of cases of CAN that come to our notice represent only tip of the iceberg (Fig. 27.1).

TYPES CAN may be of four major types: • Physical abuse • Emotional abuse • Neglect and negligent treatment • Sexual abuse.

Physical Abuse Physical abuse of a child is that which results in actual or potential physical harm from an interaction or lack of an interaction, which is reasonably within the control of a parent or person in a position of responsibility, power or trust. There may be single or repeated incidents.1 Child Abuse and Neglect 359

Emotional Abuse The failure of a caregiver to provide a developmentally appropriate and supportive environment or caregivers action that can have an adverse effect on the emotional health of the child; restrictions of movement, belittling, denigrating, scapegoating, threatening, scaring, discriminating, ridiculing or other nonphysical forms of hostile or rejecting treatment are examples of emotional abuse.1

Neglect and Negligent Treatment Neglect is the failure to provide for the development of the child in all spheres: health, education, emotional development, nutrition, shelter, and safe living conditions, in the context of resources reasonably available to the family or caretakers and causes or has a high probability of causing harm to the child’s health or physical, mental, spiritual, moral or social development. This includes the failure to properly supervise and protect children from harm as much as is feasible.1

Sexual Abuse Child sexual abuse is the involvement of a child in sexual activity that he or she does not fully comprehend, is unable to give informed consent to, or for which the child is not developmentally prepared and cannot give consent, or that violate the laws or social taboos of society. Child sexual abuse is evidenced by this activity between a child and an adult or another child who by age or development is in a relationship of responsibility, trust or power, the activity being intended to gratify or satisfy the needs of the other person. This may include but is not limited to: • The inducement or coercion of a child to engage in any unlawful sexual activity • The exploitative use of child in prostitution or other unlawful sexual practices • The exploitative use of children in pornographic performances and materials.1

PREVALENCE The problem of child abuse continues to grow in alarming proportions in Indian society. Child abuse reporting has not been made mandatory in 360 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

India unlike in countries like USA and UK. Besides, Indian nationals, including health care providers, have been found to show a general lack of sensitivity to the issue of child abuse.2 Professionals in India, like in many other countries, continue to be ignorant of the extent of the problem. In our recent questionnaire-based study in India, more than half of the pediatricians were found to be ignorant of the extent of the problem of CAN, and more than 75% considered their clinical skills to be inadequate to handle a case of child abuse. Even in countries with a history of awareness regarding this problem, clinical skills in the recognition of CAN among medical professionals are far from adequate. This may also be because hardly any time is devoted to child abuse in the medical colleges (only a few hours in medical schools even in prosperous USA).3 Understandably, therefore, the rates of abuse and neglect are just estimates based on small cross-sectional studies and views of experts in the field.

Physical Abuse Rates The literature on medical research of child abuse in India is limited. According to WHO, the highest homicide (fatal abuse) rates for children under age 4 are noted in the Eastern Mediterranean low/middle income groups, with India being the next highest region at 10.1 and 13.6 per 100,000 children for boys and girls, respectively.4 The majority of child deaths in developing countries are not investigated or routinely autopsied so it is difficult to know the precise number of fatalities from child abuse in any given country. There is general agreement that fatalities from child abuse are far more frequent than official estimates from vital records in every country where studies of infant deaths have been undertaken.5 Where there are good data, abusive head trauma is the most common cause of death from child abuse followed by blunt abdominal trauma and suffocation.5-7 Mehta8 studied abandoned children brought by the police to pediatric ward. Eighty three percent of these babies had been abandoned before first week of life and majority were females. Another study reported that 45% out of 72 children of alcoholics had physical injuries in contrast to 23.4% of 81 control children.9 Hunter et al11 studied the type and severity of discipline practices in rural India. 42% of 500 mothers admitted having Child Abuse and Neglect 361 used severe physical discipline on children. A study of urban educated middle-class professionals indicated that, in the previous year, 56.9% had engaged in ‘normal’ corporal punishment, 41.9% in ‘abusive’ forms of discipline, and 2.9% in ‘extreme’ forms of violence with their children.12 The widespread use of children in sectarian violence (allegedly in Nepal, Sri Lanka, many African countries etc.), use of children as jockeys in life- threatening sports in Arabian countries, widespread use of child domestic labor are all forms of child abuse being practiced by the society as a whole and need to be labeled as such, and wiped out.

Sexual Abuse Rates A variety of methods are used for estimating prevalence rates for sexual abuse, and the results vary depending on the definition of sexual abuse and the method used to collect the information. Some studies have been conducted among children, others have asked adolescents/ adults about their childhood, and still others have queried parents about their knowledge regarding such incidents with their children. A mean lifetime prevalence rate of childhood sexual victimization of women as girls is 19% and men as boys is 7%.13 In India traditional practices like giving over girls as “Devdasi” have been considered as a form of sexual abuse. A study conducted in 1990 by the Central Social Welfare Board in six metropolitans cities in India reported that 40% of the population of commercial sex workers entering the trade is below eighteen years of age. There is an estimated four-lakh child prostitutes in the country, as per data released by the National Institute of Public Cooperation and Child Development. The National Commission for women conducted a multicentric study14 of sexual abuse practices in the country using qualitative methods. They reported the prevalence of child sexual abuse in various parts of the country under various guises, e.g. domestic laborers, “Devdasi” system in Karnataka and similar systems in Andhra Pradesh and Tamil Nadu, prostitution with community sanction of children of many tribal groups, a flourishing sex trade in young girls in Delhi and Mumbai, etc. Some researchers have documented cases of sexually transmitted diseases among children below 14 years15 that may be considered as signs of sexual abuse. A recent study, which assessed the magnitude of physical abuse, sexual abuse and behavioral problems of the inmates of an observation home in 362 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Delhi gives a realistic idea of the magnitude of the problem.16 Of the 189 boys (6-16 years age) studied, 76.7% reported physical abuse. On clinical examination, among the physically abused children physical signs were seen in 49.7% and behavioral signs in 22.8%. The most common perpetrators of physical abuse were father (55.2%) followed by policemen (29.7%). Physical abuse was found to be significantly associated with domestic violence, substance use in family, stepparent, substance use by child, running away from home and working status. Sexual abuse was reported by 38.1% of children, with physical signs in 23.8% and behavioral signs in 16.3% of the sexually abused children. The most common perpetrators of sexual abuse were strangers. Sexual abuse was found to be significantly associated with domestic violence, solvent/ inhalant use, and working status.

Emotional Abuse and Neglect Rates Cultural issues strongly influence parental choice of techniques to make a child behave. Others may regard these as employing psychological or emotional harm or threats. Understanding and developing prevention and intervention for psychological or emotional abuse in different sociocultural paradigms, is a major challenge for researchers in the field. As definitions of neglect also vary widely, it is difficult to estimate the global/country- specific dimensions of the problem.

PHYSICAL ABUSE10 The term, physical abuse, refers to physical harm to the child at the hands of a caregiver. It may encompass a single incident or repeated incidents. Defining injuries as abusive requires a detailed history and a complete detailed physical examination, followed by consideration of other possible explanations for the findings (Table 27.1). History provided by the caregiver and the child is the most important diagnostic element in evaluation of child abuse. An important diagnostic feature of child abuse is a marked discrepancy between clinical findings and historical data as supplied by the parents. Victims of serious physical abuse are often too young or too ill to provide a history of their assault. Older victims may be too scared or intimidated to do so. Historical features having a high specificity and positive predictive value for diagnosing child abuse include a lack of Child Abuse and Neglect 363

Table 27.1: Differential diagnosis of injuries associated with child abuse10

• Bruises i. Accidental injury ii. Dermatologic disorders—Mongolian spots, erythema multiforme, phyto- photodermatitis iii. Hematologic disorders—ITP, leukemia, hemophilia, DIC iv. Cultural practices v. Genetic diseases—Ehlers-Danlos, familial dysautonomia (congenital indifference to pain) vi. Vasculitis—Henoch-Schonlein purpura

• Burns i. Accidental burns ii. Infections—SSSS, impetigo iii. Dermatologic—SJ syndrome, fixed-drug eruption, epidermolysis bullosa, severe diaper dermatitis iv. Cultural practices

• Fractures i. Accidental injury ii. Birth trauma iii. Metabolic bone disease, e.g. osteogenesis imperfecta, rickets, etc. iv. Infections—Congenital syphilis, osteomyelitis

• Head trauma i. Accidental head injury ii. Hematological disorders, e.g. vitamin K deficiency, hemophilia, etc. iii. Intracranial vascular abnormalities iv. Infections v. Metabolic diseases, e.g. glutaric aciduria type II history of trauma, history of low impact trauma in patients with persistent neurological deficits, changing histories and trauma blamed on home resuscitative efforts. In order to improve the identification of child abuse, it is important to consider inflicted injury on the differential diagnoses of all pediatric injuries. Some historical and physical examination features may offer clue to the diagnosis of inflicted injury (Table 27.2). Abused children typically present with inaccurate histories. Verbal children should be asked about the cause of identified injuries and physical findings should be discussed with family members, which may give them an opportunity to explain injuries. 364 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 27.2: Historical and physical findings suggestive of child abuse#

History • Inconsistent with injuries • Incompatible with child’s development • Changing with time/respondent • Contradictory history • Delay in seeking treatment • Denial of trauma despite significant injuries Examination • Bruises in different stages of healing • Bruises on the face, lips, mouth, back, bottom, thighs, or genitals • Bruises/burns in the shape of household articles, e.g. belt buckle, iron, etc. • Bruises after a child has been absent from school • Black eye, broken bones • Cigarette burns (especially on hand, feet, back, or bottom) • Burns that look like a child’s body part was held under hot water • Complaints of pain or itching in genital or anal areas • Unexplained burns • Child has difficulty walking or sitting • Torn or bloody underwear • Pain/discomfort with urination or has to go bathroom often • Sexually transmitted diseases/swollen, red, or itchy genital area • Always has a stomach ache or vomiting • Failure to thrive

#This is neither an all-inclusive or exhaustive list, nor are these pathognomonic

General appearance can provide clues to the child’s overall care and well-being. Approximately 50% of children intentionally injured have injuries to the head and neck. These injuries include ecchymoses, abrasions and oral injuries. Tears of the frenulum are highly suspicious in children who are not yet ambulatory. External injuries of the head and neck may be a sign of more serious internal injuries. A bruised pinna is a warning sign that more serious ear trauma, such as perforation, hemotympanum or ossicle injury may have occurred. Ophthalmologic examination is a necessary component to evaluate for retinal hemorrhage as well as other orbital trauma. Internal chest injuries are uncommon but well documented in the child abuse literature. Blunt trauma to the abdomen is a well- recognized but relatively infrequent manifestation of abuse (< 1% of identified cases of abuse). Inflicted abdominal injury is the second most common form of fatal inflicted injury. Presentation to medical care may range from severe signs and symptoms to nonspecific or no symptoms. Asymptomatic injuries may be discovered by routine screening of liver function tests and pancreatic enzymes or abdominal CT scans. A high Child Abuse and Neglect 365 index of suspicion for abdominal injuries should be kept in children presenting with other inflicted injuries. Examination of the skeletal system is an important component of the evaluation, especially for infants and toddlers. The American Academy of Pediatrics (AAP) recommends that all children less than 2 years of age with suspected abuse have a skeletal survey. Around 24% of fractures in children less than 3 years of age were abusive and among children less than 12 months of age, the abusive rate was 39%. Skeletal surveys generally are not recommended for children older than five years of age as they rarely have occult fractures. In children between 2 and 5 years of age, skeletal surveys should be done in the presence of a strong suspicion of abuse. Some skeletal injuries have a higher specificity for intentional trauma. Injuries with a moderate to high specificity for child abuse include posterior rib fractures (especially if bilateral or multiple), metaphyseal fractures of the long bones, and scapular, digit and sternum fractures. Fractures of different ages and multiple bilateral fractures also are considered to be of high or moderate specificity for abuse. Evidence suggests that a follow-up skeletal survey approximately 2 weeks after the initial study increases the diagnostic yield and, should be considered if abuse is strongly suspected. Radionuclide bone scan can play a complimentary role in infants and toddlers to the skeletal survey in maximizing detection of injuries. Inflicted neurotrauma (iNT), also known as. “shaken baby syndrome”, is the leading cause of morbidity and mortality in physically abused children. The child’s symptoms may vary from mild lethargy, vomiting or irritability to apnea, loss of consciousness and/or seizures. Seizures are common in such children and are reported in up to 79% of severely injured victims. Many cases are missed by physicians as shown in one study where 31% of patients with inflicted head injury had seen a physician with symptoms of head injury an average of 2.8 times prior to identification of the abuse. Features seen on physical examination may include irritability, lethargy, soft tissue swelling of the scalp, full fontanelle, opisthotonic posturing and/or coma. The hallmark feature is subdural hemorrhage, which may lie over one or both cerebral convexities but often is found in the posterior interhemispheric fissure. CT scan is the first line modality in evaluating possible head injury. Injuries associated with inflicted neurotrauma include retinal hemorrhages and various non-cranial injuries. Approximately 80% of 366 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics children with inflicted neurotrauma have retinal hemorrhages when examined by an ophthalmologist. The hemorrhages may be unilateral/ bilateral and may involve the preretinal, intraretinal or subretinal layers. Severe hemorrhagic retinopathy that includes multiple hemorrhages in various retinal layers extending to the periphery of the retina is considered pathognomonic of iNT. Dilated, indirect ophthalmoscopy is preferred in the evaluation of suspected head injury to identify and document the extent of retinal involvement. All victims with iNT require a skeletal survey to evaluate for other injuries, as extracranial abnormalities are detected in 30-70% of abused children with head injuries. Skeletal injuries classically associated with iNT include rib and metaphyseal fractures. Skin is the organ system with the highest number of inflicted injuries, which can be burns, bruises, lacerations and abrasions. The agents of burns include scalding objects, flames and hot solids. The scald burn with hot tap water is the most frequent type of inflicted burn and the history must be corroborated carefully with observed pattern of injury, burn depth, and wound appearance. The Immersion burn is a pathognomonic injury with involvement of the buttocks, posterior thighs, and feet with relative spring of the inguinal area. These burns characteristically have uniform depth, an unvaried appearance, and distinct wound borders. Bruises are a universal finding in ambulatory children but are also among the most common injuries identified in abused children. However, bruises in an unusual distribution or location are a cause for concern. The distribution of normal bruises varies by age and motor development. In general, bruises to the extremities and over other bony prominences are common in normal children, and bruises centrally located (buttocks, chest and abdomen) are less common. Estimating the age of a bruise is not recommended. In an effort to differentiate abusive skin injuries from corporal punishment, the AAP recommends that bruises that are non-accidental and last on the skin for more than 24 hours should be considered abusive. There are multiple medical illnesses that cause bruising and can be mistaken for abuse (Table 27.1). Identification of physical abuse is a skill which each pediatrician should have, and it is just an extension of the routine clinical skills tempered with the relevant knowledge and guided by a high index of suspicion. Child Abuse and Neglect 367

SEXUAL ABUSE Understanding Sexual Abuse It is important to appreciate that sexual abuse can happen to children of all ages. It can happen to both girls and boys and to both able and disabled children. Further, it can happen with contact or without contact of the bodies. Sexual abuse can occur both within and outside the family. Victims of child sexual abuse come from all social, ethnic and economic groups. Children who are isolated from others with few friends and little contact with siblings are at greater risk. Therefore, children who are abandoned or who live on the streets (street children in general) with/without adult caregivers are the most vulnerable. Contrary to popular belief, the abuser is not always the dirty old man in the park or the mentally unstable man walking the street. In fact, in 90% of the cases, the perpetrator is either related or known to the victim.17 Sexual abuse can occur once or even repeatedly. Sexually abusive acts have been classified into two major categories:17 • Contact sexual abuse • Noncontact sexual abuse.

Contact Sexual Abuse • Touching, fondling or oral contact with breasts or genitals • Insertion of fingers or objects into vulva or anus • Masturbation by adult of him/herself in the presence of child, or by child of adult • Intercourse: vaginal, oral or anal whether actual or attempted in any degree • Rape—the attempted/achieved penile penetration of the vagina • Other genital contact • Prostitution: Any of the above abuse that includes the exchange of money, gifts or favors and applies to both boys and girls.

Non-contact Sexual Abuse • Exhibitionism • Pornography of many kinds: photographing sexual acts or anatomy • Showing pornographic photographs, films, videos 368 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Erotic talk, telling children titillating or sexually explicit stories • Other sexual exploitation. Sadistic activities • Burning a child’s buttocks/genital area. Sexual abuse in all forms is damaging to the child. Often, the child is silenced with physical threat to himself or to his loved ones, or the child is told that the perpetrator will get into trouble (especially if the perpetrator is an important family figure to the child) if the ‘secret’ is revealed. Or else, the child is silenced with promises of tangible rewards or with actual rewards (sweets, money, foreign trips, etc.). Children find it difficult to break the silence, especially in cases of incest, enforced secrecy and a child’s fear of destroying the privacy and security of the family are powerful obstacles to disclosure. The children who disclose abuse feel guilt, fear, confusion and anxiety and need to be provided with support and effective assistance. No particular way is available to detect children that may have been abused sexually. But the key is to consider the possibility of abuse in the presence of suggestive signs (Tables 27.2 and 27.3). There are also very obvious physical signs such as pregnancy or sexually transmitted diseases found vaginally, anally or orally. Table 27.3: Behavioral signs of child abuse

• Low self esteem • Sexually acting out • Does not trust others • Aggressive, angry • Disruptive • May do illegal activities • Self-destructive • Talks about suicide • Has trouble with schoolwork • Often rubs or scratches the genitals • Fearful of new people or activities • Sleeping or eating problems • Worry, anxiety, sadness, depression • Talks about having a secret • Nightmares • Acting much younger than age • Drug or alcohol abuse • Self-injury • Avoids going home after school • Daydreams • Is scared when other children cry • Is fearful of certain adults • Often thinks she deserves to be punished • Unusual habits (sucking, biting, rocking, etc.) • Knowledge about sex is beyond age-appropriate • Infants who lie very still while looking all around • Is always looking for affection, tries to please • Withdrawn, doesn’t make friends, doesn’t like affection or being close to others

Unfortunately, there are many myths and misconceptions in the society17 we live in that prevent us from identifying the signs of symptoms of sexual abuse, and in providing children with the necessary legal, medical and psychological interventions (Table 27.4). Child Abuse and Neglect 369

Table 27.4: Commonly prevalent myths about child sexual abuse*

• Children are rarely abused • Child sexual abuse occurs mostly in poor, illiterate families (It occurs in all strata of society) • CSA generally occur on playgrounds or other public places and by servants, drivers or strangers (Mostly children are sexually abused inside their homes and the abuser is either an acquaintance, relative or family friend) • Often precocious children provoke abuse by their seductive behavior • If there happens to be a case of CSA, the offender is generally mentally ill • Usually the mother is aware of the fact that her child is sexually abused • Reporting of child sexual abuse causes more harm than good (If child sexual abuse is not reported, the same abuser may harm other children) • Child sexual abuse is usually a one time violent act that involves intercourse • Most children will forget the abuse if the adults don’t remind them • Child sexual abuse is a result of western influences/media • Child sexual abuse has always existed in society • Girls are sexually abused, not boys (Both girls and boys are sexually abused) • Children should not know about sexual abuse, we should protect their innocence (Children need to be empowered to protect themselves) • Child marriage protects children from sexual abuse (It is itself another form of child abuse) • Only the police and NGO’s should solve the problem of child sexual abuse and exploitation • Infants are never sexually abused • Children cannot tell the difference between sexual abuse and simple affection.

* Modified from reference 17.

Child Sexual Abuse and Internet Internet has become the fountainhead of all sort of crimes. Sexual abuse is no exception. There is an urgent need to protect children in the cyber space. However, India lacks effective laws to combat such cyber crimes against children. The strategy in the war against pornography on the net has to be three fold viz., • Tightening up the law • Giving more teeth to the enforcement machinery • Educating the parents in ways in which they can protect their children. The most effective preventive and easy step is educating the parents and teachers in monitoring the cyber habits of children (Table 27.5).

EMOTIONAL ABUSE Emotional abuse often goes unnoticed. Yet, it does much damage to the development of the child. This may include rejecting a child by refusing to acknowledge, believe, hear or support the child; separating a child from 370 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 27.5: Suggestions that can be given to parents and teachers to prevent CSA through the net

Home computer be installed in a common room easily accessible to everyone in family rather than in children’s bedroom Children to be instructed: • Not to chat with strangers in the chat rooms • Not to give their identity particulars to strangers in the chat rooms • Not to respond to telephone calls from individuals met online. • Reject suggestions of face to face meetings with strangers met on the net • Not to upload his/her photograph on the net. • Parents to keep a track of children’s emails and telephone calls received by them specially the strangers • Parents should be vigilant about behavioral changes in their children especially when the child stays isolated from other family members or tries to speak secretly on phone. • Install a filter to prevent the visits to porn sites others and from normal social experiences that result in extreme loneliness; terrorizing a child by intimidating, causing a state or instance of extreme fear, violent dead or fright; corrupting a child by encouraging mal-social, destructive, antisocial behavior; failure to provide sensitive interaction for the child and being emotionally unavailable; to have excessive and inappropriate expectations of children (these expectations and demands adultify the child and negatively effect the development), etc.

Emotional maltreatment affect negatively on the: • Development of social skills (ability to make friends, ability to communicate well, courage to be assertive and to manage impulsive behavior) • Development of self confidence • Emotional development and functioning • Capacity for healthy interpersonal relationships • Capacity to dream and move towards personal fulfillment • Capacity for intimacy • Ability to cope with life’s stresses. The effects of domestic violence in the family deserve much attention. Children who observe violence against their mothers, fathers, and siblings, or are victims of violence themselves are being emotionally maltreated. These children observe and hear threats of assault or actual assaults done to their loved ones; they see bruises and other injuries; and, they live in an environment of fear, tension, intimidation and anger. They also experience Child Abuse and Neglect 371 the absence of consistent positive family functioning, learn negative behaviors and unhealthy emotional expressions. They learn that it is acceptable to resolve problems through violence; they learn that the victims of violence are to tolerate it and accept the violent behavior of others and, sometimes even blame themselves for it. They become a part of the vicious cycle of violence, either as a perpetrator of violence themselves or as a victim of it. However, if such a child were provided with adequate assistance, he would be able to overcome the negative effects of family violence.

CHILD NEGLECT18 More children suffer from neglect than from physical and sexual abuse combined. In spite of this, neglect has received significantly less attention than physical and sexual abuse by practitioners, researchers, and the media. This may be because of a lack of awareness about neglect being a form of child maltreatment. Further, neglect in itself is difficult to identify, as it often is an act of omission, the absence of an action. However, neglec- ting children’s needs can be just as harmful as physically injuring them.

Definition of Neglect What is neglect? It is not an easy question to answer, even though broadly: neglect is the failure of a child’s primary caretaker to provide adequate food, clothing, shelter, supervision, and medical care. However, neglect in itself is a complex problem that is difficult to define, identify, and treat. But there are three issues that need consideration: • First, how do we define ‘adequate’ in various socioeconomic and cultural settings? • Secondly, if the caretaker is simply unable to provide for the child’s needs, would it constitute neglect or must the caretaker ‘willfully’ deprive the child? That is, should the caretaker’s intent to harm be a consideration? • Thirdly, do we label neglect only after the child has suffered harm, or even if the child is potentially at harm? In essence, should there be evidence of harm, or does neglect include endangerment of a child’s health or welfare? Most researchers consider some behaviors and conditions as ‘neglectful’: • Inadequate nutrition, clothing, or hygiene • Inadequate medical, dental, or mental health care 372 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Unsafe environments • Inadequate supervision, including use of inadequate caretakers • Abandonment or expulsion from the home.

Characteristics of Neglected Children and their Families Some behaviors, it has been suggested, should present a ‘chronic pattern’ to be considered neglectful, e.g. inadequate hygiene, lack of supervision, etc. This suggests that rather than focusing on individual incidents, one should look for an accumulation of incidents that may together constitute neglect. Other studies have linked poverty to an increased risk of child neglect. A number of factors associated with poverty may explain this association; unemployment, single parenthood, housing problems, limited access to healthcare, household crowding, and exposure to environmental hazards. Neglect may be distinguished from poverty in that culpable neglect is thought to occur only when there are resources available to the family not offered to the child. The relationship between domestic violence and child maltreatment has lately been receiving increasing attention in the western countries. In one study, domestic violence had occurred in the home in 35% of a sample of child neglect cases,19 and frequently domestic violence is included as ‘exposure to injurious environment’ in the definition of neglect. Drug affected newborns (newborns suffering from narcotics withdrawal symptoms due to maternal drug addiction) are now increasingly being included in the definition of neglect in some countries. Various studies have shown strong association between neglect and poverty, parental substance abuse, parental impulsivity, parental low self- esteem, and a lack of social support for the family. Studies from the US have found no difference between the rates of neglect between the two sexes although the status of the girl child in the Indian setup is definitely worse than her male counterpart.

Consequences of Neglect The consequences of neglect include problems with attachment, low self- esteem, increased dependency and anger, impaired cognitive development and academic achievement, and a risk for delinquent behaviors.18 It has been shown that the effects of neglect become more severe as children get older, a phenomenon referred to as ‘cumulative malignant effects’ of neglect.18 Child Abuse and Neglect 373

There are some ‘protective factors’ that promote resilience among neglected children. Protective factors include individual characteristics such as intelligence, creativity, initiative, humor and independence, or external factors such as access to good health, care and a family’s social support system, including alternative caregivers. Death is the most severe, irrecoverable consequence of neglect (fatal neglect) and in the US, neglect accounts for 45% of all childhood maltreatment fatalities.18 A majority of children dying due to neglect in the US, die as a result of a single life-threatening event, rather than from chronic neglect. 20 Similar information from India is yet not available.

Interventions Neglect is a complicated issue and poses significant challenges to treatment providers. Understanding neglect requires an awareness of related social problems such as poverty, substance abuse, and domestic violence. Interventions to treat children and families affected by neglect require thorough assessment and customized treatment. Various interventions can be designed to address the issue and have been detailed elsewhere.21 In the Indian situation, the primary need however is to recognize the entity as a form of child abuse and to create awareness and target societal conditions responsible for the situation.

RISK AND PROTECTIVE FACTORS FOR CAN 22 In the most widely adopted explanatory model or theory, “ecological model”, 23 there are four contributory components, viz. • Child • Family • Community • Society. Though children are not responsible for the abuse inflicted on them, certain child characteristics have been found to increase the risk for maltreatment, e.g. children with disabilities, age (younger children are more likely to be neglected whereas the risk of sexual abuse increases with age), sex (females are more likely to suffer sexual abuse), etc.3,11 In India, physical abuse rates have been reported to peak between 6-11 years of age.11 Important family characteristics are also linked with maltreatment, e.g. substance abuse in family, history of child abuse in parents 374 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

(intergenerational transmission),24 domestic violence, etc.22 In India, mothers self-report more use of physical discipline of children, as compared to their partners.11 Factors like poverty, poor social support network, violence, unemployment etc. are related to the community and are also linked with child maltreatment.22 Certain protective factors within children and families can reduce risks, build capacity and foster resilience. Initial studies brought out some of these factors where resilience in maltreated children was related to personal characteristics that included a child’s ability to recognize danger and adapt, distance oneself from intense feelings, create relationships that are crucial for support, and project oneself into a time and place in the future in which the perpetrator is no longer present.25 Many more protective factors have been elucidated and are detailed in Table 27.6.

Table 27.6: Common risk and protective factors for child abuse and neglect#*.

Risk factors Protective factors

CHILD FACTORS • Premature birth, birth anomalies, • Good health, history of adequate low birth weight, exposure to development toxins in utero • Above-average intelligence • Temperament: difficult or slow • Hobbies and interests, Good peer to warm up relationships • Physical/cognitive/emotional • Personality factors disability, chronic or serious illness, • Easy temperament, positive disposition Childhood trauma • Active coping style, positive self-esteem • Antisocial peer group • Good social skills, internal locus of control • Age, child aggression, behavior • Balance between help seeking and problems, attention deficits autonomy PARENTAL /FAMILY FACTORS • Personality factors, • Secure attachment; positive and external locus of control warm parent-child relationship • Poor impulse control, • Supportive family environment depression/anxiety • Household rules/structure; parental • Low tolerance for frustration monitoring of child • Feelings of insecurity, lack of trust • Extended family support and involvement, • Insecure attachment with own including caregiving help parents • Stable relationship with parents • Childhood history of abuse • Parents have a model of competence and • High parental conflict, domestic good coping skills violence • Family expectations of prosocial behavior Contd... Child Abuse and Neglect 375

Contd... Risk factors Protective factors

• Family structure—single parent • High parental education with lack of support, high number of children in household • Social isolation, lack of support • Parental psychopathology, substance abuse • Separation/divorce, especially high conflict divorce • Age, high general stress level • Poor parent-child interaction, negative attitudes and attributions about child’s behavior, inaccurate knowledge and expectations about child development SOCIAL/ENVIRONMENTAL FACTORS • Low socioeconomic status, • Mid to high socioeconomic status stressful life events • Access to health care and social services • Lack of access to medical care, • Consistent parental employment health insurance, adequate child • Adequate housing care, and social services • Family religious faith participation • Parental unemployment; • Good schools homelessness • Supportive adults outside of family who • Social isolation/lack of social serve as role models/mentors to child support • Exposure to racism/discrimination • Poor schools, Dangerous/violent neighborhood • Exposure to environmental toxins • Community violence * This is neither an all-inclusive or exhaustive list nor do these factors imply causality. #Modified from ref.22

SUBSTANCE ABUSE AND CAN Children of substance abusing parents are more likely to experience abuse- physical, sexual, or emotional-or neglect than children in non-substance abusing households.26, 27 In the US, between one-third and two-thirds of child maltreatment cases involve substance abuse and it has been reported to be one of the two major problems exhibited by families in which maltreatment was suspected. Parents who abuse substances are less likely to be able to function effectively in a parental role. This can be due to: • Impairments (physical and/or mental) while under the influence of alcohol or other drugs 376 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Expenditure of often limited household resources on purchasing alcohol or other drugs • Time spent seeking out drugs • Time spent using alcohol or other drugs • The basic needs of children, including nutrition, supervision, and nurturing, often go unmet due to parental substance abuse, resulting in neglect. • Additionally, families in which one or both parents abuse substances, and particularly families with an addicted parent, often experience a number of other problems including mental illness, unemployment, high levels of stress, and impaired family functioning, all of which can put children at risk for abuse.

LONG-TERM CONSEQUENCES OF CAN28 CAN may have consequences for children, families, and society that last lifetimes, if not generations. The effects vary depending on the circumstances of the abuse or neglect, personal characteristics of the child, and the child’s environment.

A. Physical Health Consequences The immediate physical effects may be minor like bruises/cuts, etc. or severe (broken bones, hemorrhage, or even death). Some of the known long-term effects are:

Shaken Baby Syndrome The immediate effects of shaking a baby can include vomiting, concussion, respiratory distress, seizures, and death. Long-term consequences can include blindness, learning disabilities, mental retardation, cerebral palsy, or paralysis.29

Impaired Brain Development Child abuse and neglect have been shown, in some cases, to cause important regions of the brain to fail to form properly, resulting in impaired physical, mental, and emotional development.30 In other cases, the stress of chronic abuse causes a ‘hyperarousal’ response by certain areas of the brain, which may result in hyperactivity, sleep disturbances, and anxiety, Child Abuse and Neglect 377 as well as increased vulnerability to post-traumatic stress disorder, attention deficit/hyperactivity disorder, conduct disorder, and learning and memory difficulties.31

Poor Physical Health Studies have demonstrated a relationship between various forms of household dysfunction (including child abuse) and long-term health problems such as STD, heart disease, cancer, chronic lung disease, skeletal fractures, and liver disease.32,33

Psychological Consequences The immediate emotional effects are—isolation, fear, and an inability to trust, and these can lead to life-long consequences including low self- esteem, depression, and relationship difficulties. Some of the well- recognized effects are:

Poor Mental and Emotional Health In one study, many of the young adults who had been abused as children have been shown to suffer from depression, anxiety, eating disorders, and suicide attempts.34 Other psychological and emotional conditions associated with abuse and neglect include panic disorder, dissociative disorders, ADHD, PTSD, and reactive attachment disorder.28

Cognitive Difficulties Children placed in out-of-home care due to abuse or neglect have been reported to score lower than the general population on measures of cognitive capacity, language development, and academic achievement.

Social Difficulties Children who suffer CAN by their caretakers often do not form secure attachments to them. These early attachment difficulties can lead to later difficulties in relationships with other adults as well as peers.28

Behavioral Consequences Child abuse and neglect appear to make the following more likely in some victims of CAN: 378 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Difficulties During Adolescence Such children are 25% more likely to experience problems such as delinquency, teen pregnancy, low academic achievement, drug use, and mental health problems.28

Juvenile Delinquency and Adult Criminality A history of CAN has been reportedly increases the likelihood of arrest as a juvenile by 59%, adult criminal behavior by 28%, and violent crime by 30%.28

Alcohol and Other Drug Abuse Abused and neglected children are more likely to smoke cigarettes, abuse alcohol, or take illicit drugs.

Abusive Behavior Abusive parents often have experienced abuse during their own childhood. Approximately one-third of abuse and neglected children estimated to eventually victimize their own children.

Societal Consequences CAN has a fallout on the society as a whole. The society pays a price in terms of both direct and indirect costs. Direct costs include those associated with maintaining a system to identify, respond to and treat abused children and their families, and has been estimated to be USD 24 billion per year in the US. Indirect costs (long-term economic consequences) have been estimated to be more than USD 69 billion per year.

Factors Affecting the Consequences of CAN 28 Outcomes of CAN in individual cases vary widely and are affected by many factors including: • The child’s age and developmental status at the time of the event • The type of abuse • Frequency, duration, and severity of abuse • Relationship between the victim and the abuser • Presence or absence of ‘protective factors.’ Child Abuse and Neglect 379

Research has shown that, given similar conditions, some children experience long-term consequences of CAN while others emerge relatively unscathed. The ability to cope following a negative experience is sometimes referred to as ‘resilience’. A number of ‘protective factors’ (Table 27.6) may contribute to the resilience and include individual characteristics like optimism, self-esteem, intelligence, creativity, humour, and independence. These may also include family or social environment, such as child’s access to social support, especially a caring adult in the child’s life. Community factors like neighbourhood stability and access to health care, is also a protective factor.35

Post-traumatic Stress Disorder (PTSD)36 It is now well established that stressful events occurring in the course of routine activities can affect children, both emotionally and physically. But the reaction to such stress is usually transient. However, undergoing a catastrophic event may lead to the development of ongoing difficulties known as post-traumatic stress disorder (PTSD). Such an event may a situation where someone’s life has been threatened, or severe injury has occurred, e.g. natural disasters, etc. Children who are victims or witnesses of physical or sexual abuse are frequently found to be suffering from this disorder. The risk of developing PTSD depends on the proximity of the child to the event, his relationship to the victim, seriousness of the trauma, and whether it is a one-off event or repeated. Immediately after the trauma, children may show agitated or confused behavior and/or fear, helplessness, anger, horror or denial. Children with PTSD avoid situations or places that remind them of the trauma and may become less responsive emotionally, depressed and withdrawn. The symptoms of untreated PTSD may last from several months to many years. Early intervention is essential in the form of support from parents, schools and peers. Emphasis should be placed upon establishing a feeling of safety. Individual/group/family psychotherapy, behavioral modification techniques, cognitive therapy and medications may be helpful. With appropriate management and support, youngsters with PTSD can learn to cope with the memories of the trauma and go on to lead healthy and productive lives. 380 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

MANAGEMENT (Table 27.7) Various frameworks for characterizing preventive efforts have been developed by researchers in the field.4,37 One of these is based on the developmental/ecological model (vide supra) of CAN and suggests that single- faceted interventions that focus on single risk factors are unlikely to have much impact on prevention, unless combined with other interventions addressing associated risk/protective factors. Complementing this is a public health model that organizes prevention in to primary, secondary and tertiary efforts. ‘Children’s rights’ as a framework for prevention has gained momentum recently, especially after the UN Convention on the Rights of the Child.

Table 27.7: Selected guidelines for physicians for managing child abuse

1. Obtain specialized training in identifying child abuse 2. Maintain linkage to an experienced multidisciplinary team If not possible or available, then consult with other professionals 3. The child is the physician’s primary concern, and it is his responsibility to do all he or she can to protect the child from further harm 4. If hospitalization is required, a prompt evaluation of the child’s physical, emotional and developmental problems should be done. 5. If child abuse is suspected, the physician should inform the parents, maintaining objectivity and avoiding accusatory or judgmental statements. 6. The physician should record the findings in the medical chart during the evaluation process 7. Patient confidentiality must be abrogated in cases of child abuse and an official report made. The first duty of a doctor is to protect his/her patient if child abuse is suspected. 8. Physicians should support the enactment of legislation by the government, that will effectively identify and protect abused children 9. In detecting a child with suspected abuse, the immediate actions to be taken by the physicians include: a. Reporting all suspected cases to appropriate authorities; b. Hospitalizing any abused child needing protection during the initial evaluation period; and c. Informing the parents of the diagnosis and the report sent

Most preventive programs have focused on secondary/tertiary efforts with at-risk families, children, or perpetrators of CAN and very few well- designed primary preventive efforts have been undertaken. Some of the more common approaches have been: • Public awareness campaigns • Family support approaches. Child Abuse and Neglect 381

a. Universal approaches targeting broad population of parents b. Targeted programs c. Home-visitation to new parents • School-based approaches • Prevention in medical settings. However, a comprehensive approach to the prevention of CAN would require many coordinated efforts across different sectors of the society. Ultimately, the most effective approaches will address the root causes of maltreatment by addressing issues of poverty, housing, employment, schools, healthcare and other community and neighborhood systems that build financial, human and social capital.

Interviewing Victims of Child Abuse Although the onus for conducting the interview is on trained personnel (social worker, child welfare officer, etc.), all pediatricians should at least know the basic skills. Inconsistencies, misinterpretations and distortions in a child’s statement are usually due to a lack of interviewer skills. It is a particular challenge to the personnel interviewing child victims due to the private nature and the stigma of the incident as well as the complex relationship sometimes with the alleged offender. Four factors to be considered are: 1. Being sensitive to the child’s level of development: The questions should be phrased to relate to the child’s language maturation, memory, knowledge, reasoning and emotional maturity. 2. Flexibility: Do not stick to rigid protocols of questioning. It would be useful to follow the child’s relation of the account. 3. Objectivity: The interviewer should take an objective neutral stand. Tone of voice, facial expressions, suggestive or accusatory phrasing of questions may lead the witness to give a biased statement. 4. Being emphatic: The interviewer must take steps to overcome anxiety and fear in children, which is often inevitable. Understanding nature of comments would allay anxiety and fear in the child, so that s/he could focus more on remembering the actual event accurately. The content of the language and the words used must be within the capabilities of understanding for that age, culture and other factors applicable to an individual child. Spontaneous information from children may be limited, and open-ended questions are useful in getting accurate 382 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics information. By beginning an interview with a broad, open-minded question you give the child the opportunity to narrate an independent spontaneous disclosure. If the narration is brief, you may help by asking a focused question. Children would be anxious and scared at the time of interview. Those with PTSD would not want to be reminded of the traumatic event and may take a long time to answer questions. Most children who have been sexually abused would have been told by the offender not too disclose the incident and may also have also been told not to trust authority like the police. It is often difficult for a child to talk about what would be considered as ‘secret’ or ‘shameful’ acts. Therefore, it usually takes a long time to interview a child (much longer than a clinical history). The interviewer has to be very patient and would have to have several sessions.

What to do when you find that a child has been abused? A. When a child reports abuse, or possible abuse i. Listen to the child calmly. ii. Immediately after reporting, the child needs to feel comforted and protected. iii. Make sure that the child is not in physical discomfort (pain, bleeding, bruises, etc.). If so, attend to alleviate the pain immediately. iv. Tell the child that he or she did the right thing by coming to you and reporting the abuse. More importantly, tell the child that you believe him/her. v. Do not ask too many questions. Too many questions can confuse him, and convey the message that you do not believe the child. vi. Contact the appropriate authorities about the abuse. vii. After all the legal proceedings are complete the incident might be over for the adults, but it can very well continue for the child. There may be behavioral changes in the child and a therapist to support the child to get through the incident may be needed. viii. Make all the necessary arrangements to protect the child from further abuse. B. The medical evaluation of a child who has been physically abused should consist38 • Obtaining a history of injury and physical examination of the patient Child Abuse and Neglect 383

• A trauma X-ray survey; a bleeding disorders screen; color photographs • Physical examination of siblings • Mobilize the child abuse management team (if available) • An official written medical report • A behavioral screening • A developmental screening of infant and preschool age children. C. The doctor’s role in the assessment and management of sexually abused children is to: • Take a full medical history without details about the CSA • Do a complete physical examination of the child • Collect medicolegal evidence if abuse is recent (< 72 hours) • Do further investigations • Explain the findings to parent(s) and the child • Mobilize the child abuse management team (if available) • Write detailed notes and necessary reports • Ensure the safety of the child • Give treatment and prophylaxis as clinically indicated • Arrange referral(s) and follow-up appointment. In the absence of mandatory reporting in India if the family of the child does not cooperate/agree, reporting is not possible in every case but we can act in the best interest of the child.

SOME RECOMMENDATIONS To develop a comprehensive strategy for addressing the issue of child abuse, we need to develop strategies to address this issue. The intricacies involved mean that it should be addressed different levels at same time and different administrative machineries need to come together to take necessary actions and initiatives to deal with it. Some steps can be: • Uniform age should be defined for male and female child in all laws as 18 years in view of implementing Convention of Rights of the Child. • Child marriage is the leading cause of child sexual abuse and needs to be addressed by amending the appropriate laws and ensuring their implementation. • Special court for speedy disposals of child sexual abuse cases. 384 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Incorporation of child sexual abuse/offence in subsection 376, including incest, sexual abuse of boys and non-penetrative sexual abuse. • The investigative procedures of child abuse should be made child friendly and avoid re-victimization of the child • Grievance box to be placed in all educational/residential institutions for children. • Any external agencies/volunteers seeking to conduct programs in schools should have a No Objection Certificate (NOC) from the concerned authority • Each educational/residential institution should have a Counselor/ Child Psychologist/ Social Worker. • All school inspection formats should have questions regarding child abuse. • Life Skills Education should be implemented in all educational institutions. • Intake proforma for pediatric patients at all hospitals should include parameters regarding abuse. • Special emphasis to be given to protection of mentally/physically challenged children.

KEY LEARNING POINTS • WHO defines CAN as “all forms of physical and/or emotional ill- treatment, sexual abuse, neglect or negligent treatment or commercial or other exploitation, resulting in actual or potential harm to the child’s health, survival, development or dignity in the context of a relationship of responsibility, trust or power.” • Different types of CAN are:physical abuse, emotional abuse, neglect and negligent treatment and sexual abuse. • Child neglect is far more common than child abuse. • “Children’s rights” as a framework for prevention has gained momentum recently, especially after the UN Convention on the Rights of the Child. • CAN may have consequences for children, families, and society that last lifetimes, if not generations • A comprehensive approach to the prevention of CAN would require many coordinated efforts across different sectors of the society. Child Abuse and Neglect 385

• Most effective approaches should address the root causes of maltreatment by addressing issues of poverty, housing, employment, schools, healthcare and other community and neighborhood systems that build financial, human and social capital. • Recommendation for prevention of CAN include: - Uniform age should be defined for male and female child in all laws as 18 years in view of implementing Convention of Rights of the Child. - Child marriage is the leading cause of child sexual abuse and needs to be addressed by amending the appropriate laws and ensuring their implementation. - Special court for speedy disposals of child sexual abuse cases. - Incorporation of child sexual abuse/offence in sub-section 376, including incest, sexual abuse of boys and non-penetrative sexual abuse. - The investigative procedures of child abuse should be made child friendly and avoid re-victimization of the child. - Grievance box to be placed in all educational/residential institutions for children. - Any external agencies/volunteers seeking to conduct programs in schools should have a No Objection Certificate (NOC) from the concerned authority - Each educational/residential institution should have a Counselor/Child Psychologist/ Social Worker. - All school inspection formats should have questions regarding child abuse. - Life Skills Education should be implemented in all educational institutions.

REFERENCES 1. World Health Organization. Report on the Consultation on Child Abuse Prevention. Geneva: WHO 1999. 2. Segal UA: Child abuse in India: an empirical report on perceptions. Child Abuse Negl 1992; 16: 887-908 3. Runyan D, Eckenrode J. International perspectives on the epidemiology of child neglect and abuse. Annales Nestlé 2004; 62: 1-12. 4. Krug E, Dalhberg LL, Mercy J, et al. World Report on Violence and Health. Geneva: WHO 2002. 5. Meadow R. Unnatural sudden infant death. Arch Dis Child 1999; 80: 7-14. 386 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

6. Kirschner R, Wilson H. Pathology of fatal child abuse. In: Reece R, Ludwig S (eds) Child Abuse: Medical Diagnosis and Management, 2nd edn. Philadelphia: Lippincott, Williams and Wilkins 2001: PP. 7. Alexander R, Levitt C, Smith W. Abusive health trauma. In: Reece R, Ludwig S (eds) Child Abuse: Medical Diagnosis and Management, 2nd edn. Philadelphia: Lippincott, Williams and Wilkins 2001: PP. 8. Mehta MN. Physical abuse of abandoned children in India. Child Abuse Negl 1982; 6: 171-175. 9. Rao KN, Begum S, Venkataramana V, Gangadharappa N. Nutritional neglect and physical abuse in children of alcoholics. Indian J Pediatr 2001; 68: 843-845 10. Christian CW, Cox M. A primer on the diagnosis of child abuse and neglect. Annales Nestlé 2004; 62: 13-24. 11. Hunter WM, Jain D, Sadowski LS, Sanhueza AI. Risk factors for severe child discipline practices in rural India. J Pediatr Psychol 2000;Sep; 25; 435-447. 12. Segal UA. Child abuse by middle class professionals in India. Child Abuse Negl 1995; 19: 217-231. 13. Finkelhor D. The international epidemiology of child sexual abuse. Child Abuse Negl 1994; 18: 409-417. 14. National Commission for Women. The Velvet Blouse: Sexual Exploitation of Children. New Delhi: Government of India, 1997. 15. Pandhi RK, Khama K, Sekhri R. STD in children. Indian Pediatr 1995; 32: 27-30 16. Pagare D. A study of Physical and Sexual Abuse and Behavioural Problems amongst Boys in a Child Observation Home in Delhi. Thesis: Doctor of Medicine (Community Medicine), Delhi: University of Delhi 2003. 17. The Forum against Child Sexual Exploitation. Child sexual exploitation and its Complex Shades-A compilation. FACSE: Mumbai: 2003. 18. National Clearinghouse on Child Abuse and Neglect Information. Acts of omission: An overview of child neglect. Available online at URL: http:// www.nccanch.acf.hhs.gov 19. Shepard M, Raschick M. How child welfare workers assess and intervene around issues of domestic violence. Child Maltreatment 1999; 4: 148-156. 20. Margolin L. Fatal child neglect. Child Welfare 1990; 69: 309-319. 21. Gaudin J. Child Neglect: A Guide for Intervention. Washington: Department of Health and Human Services 1993. 22. National Clearinghouse on Child Abuse and Neglect Information. Risk and protective factors for child abuse and neglect. Available online at URL: http:// www.nccanch.acf.hhs.gov 23. Runyan D, Wattam C, Ikeda R, et al. Child abuse and neglect by parents and other caregivers. In: Krug EDL, Mercy J, Zwi A, Lozano R (eds) World Report on Violence and Health. Geneva: WHO 2002. 24. Clarke J, Stein M, Sobata M, Marisi M, Hanna L. Victims as victimizers. Physical aggression by persons with a history of childhood abuse. Arch Internal Med 1999; 159: 1920-1924 25. Mrazek P, Mrazek D. Resilience in child maltreatment victims: A conceptual exploration. Child Abuse Negl 1987; 11: 357-366. 26. Dube SR, Anda RF, Felitti VJ, et al. Growing up with parental alcohol abuse: Exposure to childhood abuse, neglect, and household dysfunction. Child Abuse Negl 2001; 25: 1627-1640. 27. Kelleher K, Chaffin M, Hollenberg J, Fischer E. Alcohol and drug disorders among physically abusive and neglectful parents in a community-based sample. Am J Publ Hlth 1994; 84: 1586-1590. Child Abuse and Neglect 387

28. National Clearinghouse on Child Abuse and Neglect Information. Long-term consequences of child abuse and neglect. Available online at URL: http:// www.nccanch.acf.hhs.gov 29. Conway EE. Nonaccidental head injury in infants: The shaken baby syndrome revisited. Pediatr Annals 1998; 27: 677-690. 30. Perry BD. Childhood experiences and the expression of genetic potential: What childhood neglect tells us about nurture. Brain Mind 2002; 3: 79-100. 31. Dallam SJ. The long-term medical consequences of childhood maltreatment. In: Franey, Geffner R, Falconer R (eds) The Cost of Child Maltreatment: Who Pays? We All Do. San Diego: Family violence and Sexual assault Institute Year. 32. Felliti VJ, Anda RF, Nordenberg D, et al. Relationship of childhood abuse and household dysfunction to many of the leading causes of death in adults: The adverse childhood experiences study. Am J Prevent Med 1998; 14: 245-258. 33. Hillis SD, Anda RF, Felliti VJ, et al. Adverse childhood experiences and sexually transmitted diseases in men and women: A retrospective study. Pediatrics 2000; 106: 238-242. 34. Silverman AB, Reinherz HZ, Giaconia RM. The long-term sequelae of child and adolescent abuse: A longitudinal community study. Child Abuse Negl 1996; 20: 709-723. 35. Thomlison B. Risk and protective factors in child maltreatment. In: Fraser MW (ed) Risk and Resilience in Childhood: An Ecological Perspective. Washington: NASW Press 1997. 36. Anonymous. Resource Book. First National Conference on Child Abuse for Multidisciplinary Professionals (COCAMP ) 2004. Chennai: Indian Council for Child Welfare, Tamil Nadu and Sri Ramchandra Medical College and Research Institute 2004. 37. Eckenrode J, Runyan DK. The prevention of child abuse and neglect. Annales Nestlé 2004; 62: 32- 42. 38. Schaaf HS. Forensic medicine. Part I. Child abuse: management of physical abuse. SADJ 2004; 59:379-380. RAP Special Volume 19: Developmental and Behavioral Pediatrics

28 Childhood Injury Control

PK Bhatnagar, Suraj Gupte

INTRODUCTION Childhood injuries rank among the problems in which morbidity and mortality continue to be high. Prevention of childhood injuries based on developmental factors is emerging as an important area.1,2 There is a growing appreciation now that not only primary prevention (preventing the very occurrence of injury) but also secondary and tertiary prevention play considerable role in reducing morbidity and mortality from injuries.3 The old term “accidental prevention” now stands replaced by “injury control”.1,2 The argument favoring this change is that most injuries occur under predictable circumstances to developmentally high-risk children and can be prevented. On the other hand, the term “accident” denotes an even occurring without predictability or pattern.

ACCIDENT VULNERABILITY Evidence is available that impulsive children are more vulnerable to accidents and injuries.4,5 Toddlers and preschoolers who are impulsive and tend to seek out new and unfamiliar activities overestimate their physical abilities as six year olds. More injuries requiring medical attention compared with children who do not have these traits are experienced by them. According to a longitudinal study, young children’s temperaments offer important clues to their later risk of being injured unintentionally.5 Childhood injuries are among the few child health problems where annual mortality rates have not significantly decreased over the past several decades, As public health workers have sought to develop programs to Childhood Injury Control 389 prevent childhood injuries, researchers have begun to explore the psychological factors that may play a role. Researchers, evaluated 59 children when they were 33 months old, 46 months old and six years old. The children completed a standard battery of tests designed to measure their “inhibitory control” — whether they were impulsive or deliberate in responding to new situations - and their level of “extraversion” - whether they sought out and responded to novel situations enthusiastically. At six years old, the researchers tested the children’s skill at estimating their own physical abilities, for example, having them predict whether they could reach a toy off of a wooden block beyond their grasp. Mothers also provided ratings of their impulsivity and extraversion and a history of the children’s injuries. Children who scored high on extraversion and low on inhibitory control as preschoolers and toddlers tended to overestimate their physical abilities at age six. They also experienced more injuries requiring medical attention. The picture was reversed for those who scored low on extraversion and high on inhibitory control; they tended to underestimate their physical abilities and have few injuries. They suggest that injury prevention programs should target children with vulnerable temperaments, including those who tend to overestimate their physical abilities and those whose impulsiveness and desire for novel situations leaves them at higher risk of injury. Certain age groups have more accidents than others and certain children are more vulnerable to accidents than others as do certain situations predispose children to accidental injuries (Table 28.1). A noteworthy point is that the cause and type of injury is dictated by the child’s level of development at the time of accident. For example, the crib-bound neonate is relatively safe from accidents. However, an infant or a toddler, who is able to move around, is at a definite risk since he lacks the knowledge and experience to protect himself. Likewise, an active preschool child may wander into street without regard for traffic, tumble off a tricycle when riding too fast or fall into a body of water and drown himself. An older school-going child, despite his knowledge concerning accident prevention, may place himself in danger thoughtlessly. Not infrequently, seeking peer approval, he may accept a dangerous challenge on a dare. 390 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

Table 28.1: List of children prone to accidental injuries

• Children aged 2 to 3 years and 5 to 6 years have the greatest incidence of accidental injuries of all children below puberty (under 10 years). • The investigative curious child of either sex has more accidents than the lethargic, uninterested child. • Boys tend to have more accidents than girls because they are more daring. • Accidents tend to occur — whenever the young child is unsupervised as when the parents are under stress, — when the parents are busy caring for another family member who is ill, — when the mother is pregnant — when the parents are too tired or just not as intent on protecting the child from danger, or — when one parent mistakenly believes the other is watching the child • Accidents increase when the child is hungry and tired, especially during the afternoon when the parent is busy preparing the evening meal. • The child who is in unfamiliar surroundings or who is being cared for by an unfamiliar person or one too young to assume this responsibility tends to have an increased number of accidents. • Only children and the oldest child in a family have fewer accidents than others because of their close supervision and because of their having been taught how to avoid dangerous situations.

Teenagers are known for high rate of cycle and motor vehicle crashes because of inexperience and developmental immaturity.

RISK FACTORS

Age Accidents are natural when a child is exposed to tasks that are beyond his developmental level. For instance, poisoning becomes a risk in a toddler whose exploratory behavior is not in keeping with his lack of judgement to understand that medication can be poisonous.

Sex Boys decidedly have much higher rates of accidental injuries than girls. It seems that more than the developmental and behavioral differences, it is the variation in exposure to risk that is important.

Socioeconomic Status Lower the income, more the childhood accidents and morbidity and mortality from them. Also important are poverty-related factors such as teenage mothers, single-parent families, family stress, multiple providers, and multiple siblings. Childhood Injury Control 391

Environments Poor environment often mean poor housing, poor living conditions, bad roads, etc. All these factors (eminently correctable through interventions) contribute to injuries to children.

Race Racial disparities are known. For instance, African-Americans have very high rates of injuries compared to the whites. The rate of fire and burn deaths is 5.4 per 100,000 in black preschool children compared to just 1.5 per 100,000 in whites—a clear 3 to 4 fold increase. However, it is felt that these disparities are not because of race per se but secondary to poverty and educational level of parents and existence of hazardous environments.

ACCIDENT PREVENTION AT HOME3,5 The various hazards along with their prevention include:

Articles like matches, firearms, knives, nail file sand other manicuring implements: Lock out of reach.

Swallowable, like broken toy parts, pins, needles, thumbtacks, jewelry, hairpins, bulletin board magnets, erasers, small toys such as jacks: Store safely.

Dangerous Substances and Poisons Aspirin and other medications in pill or liquid form: Store in child proof containers. Detergents, floorwax, insecticides, household cleaners, paints, glue, pesticides, kerosene, gasoline: Safety packaging; locking out of reach.

Situations like • Stoves, fireplace, barbecue pit and other heat sources: Remove child from area or observe carefully • Venetian blinds: Cut open the loop cords that can strangle a child • Handles of cooking utensils: Turn handles away from edge of the stove • Unplugged electrical outlets: Plug them with blanks or cover them with heavy tape • Playpen or crib: Slats must be no more than 2.5 in a part mattress should fit snugly in crib 392 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Major appliances: Unplug when not in use • Plastic wrappings: Destroy.

Falls • Stairs: Securely gate top and bottom • Windows: Securely bar, close or screen • Electrical cords: Place out of the way so that child cannot trip • Bathtubs: Install adhesive rubber appliques on bottom of tub.

PARENT EDUCATION ON ACCIDENT PREVENTION Undoubtedly, accident prevention needs to be taught to the parents right from the time the mother conceives and the baby starts growing within her uterus. What is important is that it should be in a simple digestible form rather than in a form that overburdens the parents with much-too- much information. Medical profession can educate parents individually about prevention of accidents as also participate in and provide leadership for groups promoting home safety and accident prevention in the communities. A noteworthy point is that parents should be taught to consider children’s activities on the basis of various levels of their growth and development and the hazards into which their interests can lead them. Thereafter, protection and education needed by each sex and age group are adapted to the prevention of accidents to which they are most liable.

CHILD EDUCATION ON ACCIDENT PREVENTION No less important is education of children on accident prevention. In case of infants and toddlers, teaching accident prevention revolves round making them accustomed to the safety measures employed in their care. They learn by observing their mother’s actions what behavior displeased her, such as behavior that may lead to their harm. Once the infant moves from toddler into the preschool age, simple explanations do. There should be nothing wrong in inculcating a limited degree of fear of being hurt. This forms the foundation of caution. As the child enters school age and later adolescence, accident hazards increase though health hazards lessen. This is high time the child is taught accident prevention in the classroom as also on the playground. Childhood Injury Control 393

In case an accident indeed occurs, parents (yes, teachers and older children) should be prepared to provide at least the minimal first-aid.

KEY LEARNING POINTS • The old term “accidental prevention” now stands replaced by the “injury control”. • Prevention of childhood injuries based on developmental factors is emerging as an important area. Not only primary prevention (preventing the very occurrence of injury) but also secondary and tertiary prevention play considerable role in reducing morbidity and mortality form. • Impulsive children are more vulnerable to accidents and injuries. More injuries requiring medical attention compared with children who do not have these traits are experienced by them. • Cause and type of injury is dictated by the child’s level of development at the time of accident. • Risk factors are related to age, sex, socioeconomic status, environments, race. • Accident prevention should be taught to parents right from the time the time the mother conceives and the baby is growing within her uterus. • Child education on accidental prevention is equally important. • In case an accident indeed occurs, facilities for at least the minimal first-aid should be available at home and in school.

REFERENCES 1. Rivara FP, Grossman D. Injury control. In: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, 17th edn. Philadelphia: Saunders 2004: 256-267. 2. American Academy of Pediatrics, Committee on Injury Prevention and Poison Prevention. Injury Prevention and Control for Children and Youth, 3rd edn. Elk Grove Village, IL: American Academy of Pediatrics 1997. 3. American Academy of Pediatrics, Committee on Injury Prevention and Poison Prevention. Children in pickup trucks. Pediatrics 2000;106:857-859. 4. Singh T, Gupte S. Community pediatrics. In: Gupte S (ed): The Short Textbook of Pediatrics, 10th edn. New Delhi: Jaypee Brothers 2004. 5. Woodworth C. Injury Control. London: Smithsons, 2003. RAP Special Volume 19: Developmental and Behavioral Pediatrics

29 Adoption

Suraj Gupte, Ansuya A Reddy, PSN Reddy

INTRODUCTION The term, adoption, is employed to an act of affiliation by virtue of which a child is removed from the biological parents and is placed in the adoptive family.1-3 The adoptive parents become responsible for his care and welfare. It is a social, emotional and legal process aimed at creating a family for children when the birth or biological family is unable or unwilling to carry out responsible parenting. In terms of law, it is transfer of rights and responsibilities concerning the child from biological or birth parents to the adoptive parents irrevocably.

Types of adoption are 3-6 • Intrafamily adoption • Agency adoption • Single parent adoption • Old child adoption • “Special needs” child adoption • Intercountry or international adoption The term, surrogancy, denotes an arrangement wherein a lady agrees to be artificially inseminated with the sperms of a man who is not her husband and then undergoes pregnancy and delivers the baby to him at birth to bring up as his own child. According to one school of thought, surrogancy fails to meet the best interests of the child.6 According to this view, it is, in fact, self-serving for the adults involved in the contract. Adoption 395

WHY ADOPTION The most common reason for adoption is a “viable alternative means for attaining parenthood by a childless couple”. Other reasons could be an eagerness of a couple to provide home to a homeless child, an incurable genetic disorder in the couple’s biologic child, a desire for a child of the other sex, advanced age of the mother/parents. The parents are expected to adopt a child because they simply love to take his care and not because they see a support in him for their old age. The majority of the children available for adoption come from young unwed mothers who fail to keep such children with them because of the stigma attached to out-of-wedlock issues. Remaining reasons for giving the child away for adoption include desertion by one parent, death of a parent, birth of an unwanted child (usually a girl or a handicapped child). All said and done, adoption is also an instrument to ensure protection of child’s rights (Table 29.1)4 Table 29.1: Child rights protected by adoption

• Right to life survival and development • Right to name and responsibility • Right of child interest as a primary consideration • Right to be specially protected from physical harm and neglect • Right to universal brotherhood • Right to social security through education, employment and earning • Right for special attention by the disable child

SOURCE OF ADOPTION: RELATIVE OR AGENCY? Most often, adoption is restricted to the couple’s relatives. Nevertheless, this is, by no means, the recommended means of adoption. Neither, taking resort to private adoptions through hospitals and medical practitioners without completing the legal formalities is in appropriate. Such an adoption can never guarantee confidentiality. Today, moreover, biological parents can any time contest the adoptive parents’ right to continue with the custody of the child. Remember, the best way to adopt a child is through the reputed adoption agencies, children’s homes and other institutions that have children for adoption. These agencies make available to the adopting couple the requisite details abut the exact procedure for adoption. The agencies make sure that the adopted child is smoothly placed with the adopting parents. 396 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

ADOPTION LAWS In India. The Hindu Adoption and Maintenance Act 1956 governs adoption among the majority community. In case of minority communities whose personal laws fail to permit adoption, and in intercountry adoption, the Guardian and Work Act 1890 becomes applicable. Here, the parents can only be guardians to the adopted child. The adopted child does not automatically get the status of a biological child. The notable features of the adoption law are summarized in Table 29.2. The adoption laws have been criticized for some glaring deficiencies which leave a room for violation of the laws by various quarters including the Apex Court. For instance, a married Hindu woman is not entitled to adopt a child notwithstanding a consent from her husband. Secondly, an adult orphan cannot be adopted because he has no guardian. Thirdly, an adopted child has got to break all relations with biological parents, He can never return to them even when he opts for this course as he grows up. Table 29.2: Highlights of the adoption law in India

A. In Respect of the Child • A prospective child for adoption must not have completed 15 years of age. • An adopted child cannot be readopted by another parent even though the latter is child’s natural (biologic) parent. • A married child cannot be adopted. B. In Respect of the Guardians Adopting Parents • A Hindu cannot adopt more than one male or a female child. • The adopting parent must be a major (has completed age of 18 years) and of sound mind. • It is obligatory for a married Hindu male to obtain consent of the wife for adopting a child. • A married Hindu woman cannot adopt even with the consent of her husband. C. In Respect of Parents who Give in Adoption • The father cannot give the child in adoption without the consent of the mother. • The mother of an illegitimate child is entitled to give the child for adoption. D. In Respect of the Guardians • The guardian is entitled to give the child in adoption under special circumstances such as when the parentage is not known, e.g. abandoned children in hospital, nursing home or refugee camp.

ADOPTION AND THE PEDIATRICIAN The role of the pediatrician, both before and after adoption, remains important. His role extends beyond evaluating the health and developmental status of the child. In fact, he should be the source of key guidance and support at all stages of adoption:6 Adoption 397

• He must encourage adoption from an approved agency only. • He should appropriately assess the psychosocial settings of the adopting couple. • He should provide adequate safeguard to the adopting couple by providing correct information about the health status. • He should make available to the family benefit of his advice for the emotional problems of the adopted child as a consequence of overindulgence of parents. • No child should be rejected for adoption on the basis of an incorrect diagnosis of mental subnormality or spasticity. • Though interests of the child are the primary consideration, interests of parents must never be ignored. • The adopting parents should be prepared to take some risk.

ASSESSMENT OF FITNESS FOR ADOPTION2-5 Assessment for appropriateness for adoption a difficult task best dealt with by an expert, has got to be based on the following three parameters • History • Developmental examination • Investigations • Interpretation

History It is important to obtain a good history with special reference to real parents, pregnancy, duration of gestation, delivery, condition of the neonate at birth and later, weight at birth, etc. In addition, enquiries should be directed at family history of diseases like HIV/AIDS, psychosis, and degenerative disorders of the nervous system. Information should also be obtained about illnesses during pregnancy (preeclampsia, APH) and other risk factors such as extreme prematurity, SFD, etc. Remember, history of mental subnormality or epilepsy in a parent is not a contraindication for adoption.

Developmental Examination The earliest age at which an infant can be assessed for development is 6 weeks. This is true for a full-term baby, certainly not for a baby born 6 weeks preterm. In case of doubts about the development at 6 weeks, it is 398 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics advisable to reassess the infant at 6 months when one can confidently assess the gross motor development (Table 21.2)

Table 29.3: Salient features of gross motor development at 6 months

• He is able to reach out for objects and grasp them without being put into the hand • Maturity of the grasp can be assessed. • He can transfer objects from one hand to the other. • He can chew. • He begins to imitate cough or other noise. • He takes interest in surroundings. • He immediately turns head to sound

In case of doubt about development at 6 months, the infant needs to be reassessed at 10 months (Table 29.3). Table 29.3: Salient features of gross motor development at 10 months

• He is able to stand holding on to a chair or bed. • He may well even walk holding on to some furniture. • He may creep • He makes index finger approach to objects and finger thumb apposition. • He waves “bye bye”. • He plays patcake • Helps the mother to dress him by holding his arm for a shirt or foot for a shoe.

Investigations The following investigations should be carried out • Complete blood count (CBC) • Urinalysis • Stool examination • Tuberculin (Mantoux) test • Chest X-ray • TORCH Special investigations are indicated in case of suspicion of • Hemolytic anemia • Hypothyroidism • Chromosomal anomalies • Inborn errors of metabolism (PKU, galactasemia, aminoaciduria) Adoption 399

Interpretation While interpreting the results for fitness, it is important to bear in mind the background situation. The child could have suffered physical, emotional or nutritional setback in the recent past. In case of uncertain interpretation if the child is normal or abnormal, the adopting parents must be told the truth. If you are finally sure that the child is mentally backward, you must assess the extent of backwardness and state the position to parents. It is not quite unusual for some foster parents to adopt even mentally subnormal child. It is advisable to provide genetic advice as and when warranted to the foster parents.

POSSIBLE POST ADOPTION CRISIS SITUATIONS4

Sudden Change Crisis Failure to adjustment may lead to anxiety reactions, rejection or aggression

Behavior Crisis Too much of pampering and overprotection or rejection may cause behavior problems in the child, e.g. stubbornness, pouting, temper tantrums.

Communication and Identity Crisis This is particularly seen in intercountry adoption in which complexion and language differences play important role.

Assimilation Crisis This again is a problem occurring in intercountry adoption. Young adolescents and adults face discrimination in marriage and job procurement. KEY LEARNING POINTS • Adoption is an act of affiliation by virtue of which a child is removed from the biological parents and is placed in the adoptive family that becomes responsible for his care and welfare. It is a social, emotional and legal process aimed at creating a family for children when the birth or biological family is unable or unwilling to carry out responsible parenting. 400 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

• Adoption should always be in keeping with law of the country. • Pediatrician must ensure that adoption is made through a dependable agency. • Assessment of the child for fitness should be based on history, physical examination, certain investigations and interpretation. • Parents must tell the child about his adoption rather than keeping it a secret. • A post adoption follow-up is important to determine occurrence of crisis situations and for timely action.

REFERENCES 1. Gupte S. Child adoption. In: The Short Textbook of Pediatrics, 10th edn. New Delhi: Jaypee Brothers, 2001. 2. Simms MD. Adoption. In: Behrman RE, Kliegman RM, Jenson HB (eds): Nelson Textbook of Pediatrics, 17th edn. Philadelphia: Saunders 2000. 3. Gupte S. Child adoption. In: Speaking of Child Care: Everything You Wanted to Know, 2nd edn. New Delhi: Sterling, 1991. 4. Potdar RD. Adoption and care of orphans. In: Parthsarthy A (ed): IAP Textbook of Pediatrics, 3rd edn. New Delhi: Jaypee Brothers 2006: 190-192. 5. Illingworth RS. Assessment of suitability for adoption. In: The Development of the Infant and Young Child, 9th edn. Edinburgh: Churchill Livingstone, 1987. 6. Gaur A. Adoption. In: Gupte S (ed): Recent Advances in Pediatrics, Vol 16. New Delhi: Jaypee Brothers 2006: 453-461. Index

A symptom 64 hyperactivity-impulsivity 65 Adoption 394 inattention 66 adoption and the pediatrician 397 psychopharmacologic management 79 adoption laws 396 alpha2 agonists 89 assessment of fitness 397 amphetamine products 87 developmental examination 398 atomoxetine 93 history 397 bupropion 93 interpretation 399 clonidine 89 investigations 398 future ADHD medications 94 possible postadoption crisis situations guanfacine 90 399 methylphenidate 82 source 395 MPH preparations 86 types 394 MPH side effects 83 why adoption 395 pemoline 89 Aggression 272, 301 prevalence of stimulant abuse 88 Aminoaciduria 125 stimulant medication 80 Angelman’s syndrome 125 tricyclic antidepressants 91 Anger 301 Autism and related disorders 115 Anorexia nervosa 44, 237 definition of autism 118 differential diagnosis 45 differential diagnosis 122 laboratory anomalies 45 emotional and autonomic regulations treatment 46 116 nutritional 46 language development 116 pharmacotherapy 47 motor and adaptive behavior 115 psychotherapy 47 neurobiological insights into autism Anxiety 179, 279 119 Arithmetic skills 182 evidence for an organic cause 119 Asperger’s syndrome 124 genetic factor 119 Attention deficit hyperactivity disorder magnetic resonance spectroscopy 60, 79, 181 122 diagnosis 60 magneto encephalography 122 educational issues 62 neurochemical 121 management 67 neuroimaging studies 120 behavior therapy 68 neuroplasticity 117 biofeedback 70 personal and social behavior 116 cognitive-behavioral therapy 69 psychosocial assessment 126 contingency management 68 assessment of development 126 functional analysis 68 Baroda scale 127 psychological 75 ordinal scale 127 psychopharmacological 75 test of assessment of intelligence psychosocial treatments 70 127 psychotherapy 67 Weschler’s intelligence scale 128 school based interventions 73 Weschler’s primary and preschool self-management 69 scale of intelligence 127 402 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

B Bulimia nervosa 47 Behavior problem 222, 309 differential diagnosis 49 assessment and therapy 242 treatment 49 classification 223 C etiopathogenesis 222 assessment of parents 315 Child abuse 357 clinical assessment 310 definition 357 childhood psychopathology 310 long-term consequences 376 distinctive aspects 311 management 380 conducting the assessment 316 prevalence 359 interview of child 316 emotional abuse and neglect rates components of the child interview 362 317 physical abuse rates 360 limitations of structured measures sexual abuse rates 361 324 risk and protective factors 373 mental status examination 320 types 358 rating scales 322 emotional abuse 359 standardized assessment 321 neglect and negligent treatment 359 structured and semistructured physical abuse 358 interviews 321 sexual abuse 359 management techniques 328 Child abuse and neglect 237 differential reinforcement 331 Child neglect 371 extinction 330 characteristics 372 guidelines for the punishment 336 consequences 372 imitation and instructional training definition 371 332 interventions 373 negative reinforcement 333 Childhood disintegrative disorder 99, 124 overcorrection 335 assessment positive reinforcement 329 behavioral 107 punishment 333 developmentally based 107 record keeping and trouble diagnostic 108 shooting 337 investigations 107 response cost 335 clinical features 101 shaping 332 course and prognosis 109 time-out 334 development of diagnostic concept outcome of behavior management 337 100 preparation of child 316 differential diagnosis 104 psychological and neuropsychological epidemiology 101 assessment 325 etiology 105 adjunctive evaluations 326 genetics 106 communicating findings and medical conditions 107 recommendations 327 neurochemistry 105 diagnostic laboratory tests 327 neurological aspects 106 Breath-holding spells 228 neuropathology 105 clinical features 229 Indian data 109 diagnosis 229 interventions etiology 228 behavioral interventions 108 prognosis 230 enhancing language and treatment 229 communication 108 Index 403

family interventions D psychopharmacology 109 Depression 52 rehabilitation 108 clinical features 54 Childhood injuries 388 comorbidity 55 accident prevention 391 etiology 53 accident vulnerability 388 treatment 56 child education on accident prevention Developmental disability 1 392 historical aspects 2 parent education on accident Developmental screening 4, 5 prevention 392 harms and limitations 6 risk factors 390 prescreening developmental test 7 Childhood lead poisoning 137 Dissociative disorders 31, 37 clinical manifestations 139 Dyslexia 210 exposures 137 clinical manifestations 211 management 146 diagnosis 211 pathophysiology 139 etiology 210 primary prevention management 212 screening 144 toxicology 140 E behavior 142 Emotional abuse 369 dental effects 144 emotional maltreatment 370 early cognitive development 142 Encopresis 235 hematological effects 143 Enuresis 232 intellectual functioning 141 clinical features 233 learning and memory 140 diagnosis 234 neurodevelopment 142 etiology 233 neurological effects 140 treatment 234 renal effects 144 Epileptic encephalopathy 126 Conduct disorder 271, 274 Eye movement desensitization and assessment 284 reprocessing 207 classification 273 clinical features 273 F comorbidity 278 epidemiology 277 Fragile X syndrome 123 etiology 279 H biological factors 279 functional/individual factors 281 Headache 158 neuroanatomy and etiology 158 neuropsychology 280 evaluation 160 neurotransmitters and migraine headaches 37, 162 neurochemicals 280 atypical and rare forms 164 psychosocial factors 282 migraine management 164 Indian data 291 migraine with aura 163 treatment 286 migraine without aura 163 cognitive behavioral treatment 288 tension headaches 166 multisystemic therapy 286 Hostile behavior or conduct disorders 300 parent management training 287 approach to the child 305 pharmacological treatment 290 assessment and diagnosis 305 social skill training 289 development of emotions 300 404 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

etiology and predisposing factors 301 Mood disorders 279 environmental 302 Motor paralysis 37 familial 302 genetic 301 N hormonal status 302 Neurocutaneous syndromes 125 impact of media 302 Normal development 10 sex of the child 302 feeding/dressing 18 spectrum of such behavior 303 bladder control 19 Hostility 301 bowel control 19 Hypomelenosis of Ito 124 handedness 19 Hysteria 240 opening of mouth 18 common presentations 240 speech 18 management 241 general understanding 16 Hysteria 31 displeasure and pleasure 17 governing principles 10 I locomotion 11 Infantile neuronal ceroid lipofuscinosis additional forms 13 123 prone position 12 sitting position 12 L standing and walking position 13 Lactic acidosis 125 supine position 11 Landau-Kleffner syndrome 126 ventral suspension 11 manipulation M hand and finger skills 14 Mathematics disabilities 215 use of ears 16 management 219 use of eyes 15 changing attitudes of the family and O the teachers 220 evaluation 219 Obsessive-compulsive disorder 131 intervention strategy 219 comorbidity 133 manifestations 215 incidence and prevalence 132 accumulations 218 psychological impact 132 affinities 218 treatment 133 applications 218 behavior therapy 133 apprehensions 218 pharmacotherapy 134 approaches 218 Oppositional defiant disorder 273 concepts 217 details 216 P estimations 217 Pain syndromes 33 facts 215 Periodic syndrome 239 images 217 Phenylketonuria 125 logical processes 217 Physical abuse 362 manipulations 216 bruises 366 patterns 216 general appearance 364 procedures 216 inflicted neurotrauma 365 sentences 216 skeletal system 365 words 216 PICA 224 Memory 182 clinical features 227 Metabolic disorders 125 diagnosis 227 Index 405

etiologic hypotheses 226 family 266 etiology 225 individual 265 management 227 normal changes 259 risks 227 predisposing circumstances 260 types 225 the role of 266 Play therapy 341 government 268 different forms 343 media 268 child parent relationship therapy parents 267 344 pediatrician 266 directive 343 voluntary organizations 267 group 344 various acts/behavior 261 individual 343 accident proneness 264 non-directive 343 childhood obesity 265 method 345 cyber sex 262 process 344 delinquent acts 263 rationale 342 drug abuse 261 role of therapist 344 live-in-relationship 263 setting limits 346 sexual activities 262 toys and materials 346 street children 263 Post-traumatic stress disorder 196 suicidal tendencies 264 clinical features 201 super brat syndrome 263 comorbidity 199 terrorism 264 course and prognosis 204 violent behavior 264 diagnosis 199 epidemiology 196 S biological factors 198 School phobia 236 psychological factors 198 Seizures 150 social factors 198 classification management 205 juvenile absence epilepsy 151 pharmacotherapy 205 juvenile myoclonic seizure 151 psychotherapy 206 rolandic epilepsy 151 Post-traumatic stress disorder 379 issues in adolescents Pseudoseizure 32, 37 contraception 152 Psychotropic drugs in children 23 driving 154 antidepressants 26 effects of antiseizure drugs 152 stimulant medication for ADHD 24 pregnancy 153 lessons for India 25 psychological aspects 152 US scenario 24 teratogenicity 153 timeline of the genesis of SSRI debate vocational guidance 154 27 Self-esteem 348 use of SSRI 27 attribution theory 350 different periods 349 R factors influencing 349 Reading and writing skills 182 manifestations 350 Recurrent abdominal pain 33, 37 remedial interventions 351 Rett’s syndrome 99, 122 choices, decisions and problem Rhythmic headbanging/rocking 231 solving 352 Risk-taking behavior 259 condoning mistakes and failures impact of high risk behaviors 265 354 community and nation 266 encouragement 353 406 RAP Spl. Volume 19: Developmental and Behavioral Pediatrics

positive feedback 353 sexual function 185 responsibility and contribution 351 sexual maturity 184 self-discipline 353 social skills and social isolation 176 Sexual abuse 367 transition to adulthood 183 abuse and internet 369 Stuttering (stammering) 232 contact 367 Substance abuse 236, 278, 375 non-contact 367 Suicidal ideation 178 Sexual education 187 Suicidal tendencies 238 Sibling rivalry 238 Surrogacy 394 Social skills training 70 Somatization 30 T classification 31 Teeth grinding (bruxism) 230 clinical characteristics 31 Temper tantrums 232 diagnostic categories 31 Thumbsucking and nailbiting 230 etiology 35 TIC disorders 244 maintaining factors 36 assessment of child 251 precipitating factors 35 clinical features 248 predisposing factors 35 comorbidity 251 management complications 251 evidence base 36 etiology principles of treatment 38 genetic factors 245 patient-physician interaction 39 neuroanatomical theories 246 prevalence 32 neurochemistry 246 psychiatric illness in children 34 neuroimaging 247 specific syndromes 32 other theories 247 Somatoform disorders 32, 279 treatment 253 Spina bifida 170 pharmacotherapy 253 anxiety 179 psychosocial management 255 cognitive function 180 TICS 231 depression and suicidal ideation 178 Trauma focused cognitive behavioral fertility, pregnancy, and complications therapy 206 186 Tuberous sclerosis 124 life skills and family function 173 neuropsychological evaluations 183 V parental issues 174 Visuo-and fine-motor skills 183 physician attitude 172 W self-concept 175 sexual attitudes 184 West syndrome 126 sexual education 187 William syndrome 125