Cerebral Palsy
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Brain Death and the Cervical Spinal Cord: a Confounding Factor for the Clinical Examination
Spinal Cord (2010) 48, 2–9 & 2010 International Spinal Cord Society All rights reserved 1362-4393/10 $32.00 www.nature.com/sc REVIEW Brain death and the cervical spinal cord: a confounding factor for the clinical examination AR Joffe, N Anton and J Blackwood Department of Pediatrics, Stollery Children’s Hospital, University of Alberta, Edmonton, Alberta, Canada Study design: This study is a systematic review. Objectives: Brain death (BD) is a clinical diagnosis, made by documenting absent brainstem functions, including unresponsive coma and apnea. Cervical spinal cord dysfunction would confound clinical diagnosis of BD. Our objective was to determine whether cervical spinal cord dysfunction is common in BD. Methods: A case of BD showing cervical cord compression on magnetic resonance imaging prompted a literature review from 1965 to 2008 for any reports of cervical spinal cord injury associated with brain herniation or BD. Results: A total of 12 cases of brain herniation in meningitis occurred shortly after a lumbar puncture with acute respiratory arrest and quadriplegia. In total, nine cases of acute brain herniation from various non-meningitis causes resulted in acute quadriplegia. The cases suggest that direct compression of the cervical spinal cord, or the anterior spinal arteries during cerebellar tonsillar herniation cause ischemic injury to the cord. No case series of brain herniation specifically mentioned spinal cord injury, but many survivors had severe disability including spastic limbs. Only two pathological series of BD examined the spinal cord; 56–100% of cases had upper cervical spinal cord damage, suggesting infarction from direct compression of the cord or its arterial blood supply. -
Lower Extremity Orthoses in Children with Spastic Quadriplegic Cerebral Palsy Implications for Nurses, Parents, and Caregivers
NOR200210.qxd 5/5/11 5:53 PM Page 155 Lower Extremity Orthoses in Children With Spastic Quadriplegic Cerebral Palsy Implications for Nurses, Parents, and Caregivers Kathleen Cervasio Understanding trends in the prevalence of children with cerebral prevalence for cerebral palsy in the United States is palsy is vital to evaluating and estimating supportive services for 2.4 per 1,000 children, an increase over previously re- children, families, and caregivers. The majority of children with ported data (Hirtz, Thurman, Gwinn-Hardy, Mohammad, cerebral palsy require lower extremity orthoses to stabilize their Chaudhuri, & Zalusky, 2007). Cerebral palsy is primar- muscles. The pediatric nurse needs a special body of knowledge ily a disorder of movement and posture originating in to accurately assess, apply, manage, teach, and evaluate the use the central nervous system with an incidence of 2.5 per 1,000 live births with spastic quadriplegia being the of lower extremity orthoses typically prescribed for this vulnera- common type of cerebral palsy (Blair & Watson, 2006). ble population. Inherent in caring for these children is the need This nonprogressive neurological disorder is defined as to teach the child, the family, and significant others the proper a variation in movement, coordination, posture, and application and care of the orthoses used in hospital and com- gait resulting from brain injury around birth (Blair & munity settings. Nursing literature review does not provide a Watson, 2006). Numerous associated comorbidities are basis for evidence in designing and teaching orthopaedic care usually present with cerebral palsy requiring various for children with orthoses. A protocol for orthoses management interventions. -
10Neurodevelopmental Effects of Childhood Exposure to Heavy
Neurodevelopmental E¤ects of Childhood Exposure to Heavy Metals: 10 Lessons from Pediatric Lead Poisoning Theodore I. Lidsky, Agnes T. Heaney, Jay S. Schneider, and John F. Rosen Increasing industrialization has led to increased exposure to neurotoxic metals. By far the most heavily studied of these metals is lead, a neurotoxin that is particularly dangerous to the developing nervous system of children. Awareness that lead poison- ing poses a special risk for children dates back over 100 years, and there has been increasing research on the developmental e¤ects of this poison over the past 60 years. Despite this research and growing public awareness of the dangers of lead to chil- dren, government regulation has lagged scientific knowledge; legislation has been in- e¤ectual in critical areas, and many new cases of poisoning occur each year. Lead, however, is not the only neurotoxic metal that presents a danger to children. Several other heavy metals, such as mercury and manganese, are also neurotoxic, have adverse e¤ects on the developing brain, and can be encountered by children. Al- though these other neurotoxic metals have not been as heavily studied as lead, there has been important research describing their e¤ects on the brain. The purpose of the present chapter is to review the neurotoxicology of lead poisoning as well as what is known concerning the neurtoxicology of mercury and manganese. The purpose of this review is to provide information that might be of some help in avoiding repeti- tion of the mistakes that were made in attempting to protect children from the dan- gers of lead poisoning. -
Management of Children with Cerebral Palsy
CEREBRAL PALSY MANAGEMENT OF CHILDREN WITH CEREBRAL PALSY The term cerebral palsy refers to motor impairments that result from an insult to the immature motor cortex and/or its motor pathways. I would like to dedicate this article to Dr Leila J Arens, whose passion for manag- ing children with cerbral palsy spans more than 4 decades, and has inspired so many of us to work in this field. The neurological lesion causing cerebral palsy (CP) is static, but the clinical con- dition changes over time. CP has a wide clinical spectrum with a number of aeti- ologies. It is not sufficient just to make the diagnosis of CP; the term needs further qualification by a more detailed description of the clinical condition. There are different types of CP, ranging in severity and associated with a variety of prob- lems. Doctors at primary and secondary level often feel inadequate, due to the complex and diverse nature of the CPs and their associated problems. There is also no magic cure, but rather intervention to allow the patient to reach maxi- BARBARA LAUGHTON mum function and potential and to prevent further complications. This article MB ChB, DCH (SA), FCPaed (SA) aims to give a logical and sound approach to the assessment and management Registered Developmental of the common problems and complications in patients with CP. These children Paediatrician may then be referred when a need for specialised intervention is identified, if Paediatric Neurology Clinic they are deteriorating on current management, or if there is doubt about the diagnosis or management plan. Tygerberg Children’s Hospital Cape Town ASSESSMENT OF CHILDREN WITH CEREBRAL PALSY Dr Laughton works as a consultant at Careful assessment of a patient with CP Tygerberg Children’s Hospital. -
Local Control of Human Umbilical Vessel Tone
LOCAL CONTROL OF HUMAN UMBILICAL VESSEL TONE. Thesis submitted to the University of London for the degree of Doctor of Philosophy in the Faculty of Science. by Anita-Jane Sexton B.Sc. (Hons.) Department of Anatomy and Developmental Biology University College London. ProQuest Number: 10017796 All rights reserved INFORMATION TO ALL USERS The quality of this reproduction is dependent upon the quality of the copy submitted. In the unlikely event that the author did not send a complete manuscript and there are missing pages, these will be noted. Also, if material had to be removed, a note will indicate the deletion. uest. ProQuest 10017796 Published by ProQuest LLC(2016). Copyright of the Dissertation is held by the Author. All rights reserved. This work is protected against unauthorized copying under Title 17, United States Code. Microform Edition © ProQuest LLC. ProQuest LLC 789 East Eisenhower Parkway P.O. Box 1346 Ann Arbor, Ml 48106-1346 ABSTRACT This thesis examines the influence of the vascular endothelium on the local control of human feto-placental vascular tone and how these influences may vary with gestation. Electron microscopic studies have revealed that human umbilical vessels are devoid of nerves throughout gestation. Structural changes in the arterial and venous wall could be detected throughout gestation. The presence of a subpopulation of endothelial cells containing nitric oxide synthase (NOS)-like immunoreactivity was identified only in late pregnancy. The umbilical artery and vein, from both early and late pregnancy, did not relax to acetylcholine (ACh), adenosine 5-triphosphate (ATP), substance P (SP) and calcium ionophore A23187, known to be endothelium-dependent vasodilators, but did relax to sodium nitroprusside (SNP). -
Child Neurology: Hereditary Spastic Paraplegia in Children S.T
RESIDENT & FELLOW SECTION Child Neurology: Section Editor Hereditary spastic paraplegia in children Mitchell S.V. Elkind, MD, MS S.T. de Bot, MD Because the medical literature on hereditary spastic clinical feature is progressive lower limb spasticity B.P.C. van de paraplegia (HSP) is dominated by descriptions of secondary to pyramidal tract dysfunction. HSP is Warrenburg, MD, adult case series, there is less emphasis on the genetic classified as pure if neurologic signs are limited to the PhD evaluation in suspected pediatric cases of HSP. The lower limbs (although urinary urgency and mild im- H.P.H. Kremer, differential diagnosis of progressive spastic paraplegia pairment of vibration perception in the distal lower MD, PhD strongly depends on the age at onset, as well as the ac- extremities may occur). In contrast, complicated M.A.A.P. Willemsen, companying clinical features, possible abnormalities on forms of HSP display additional neurologic and MRI abnormalities such as ataxia, more significant periph- MD, PhD MRI, and family history. In order to develop a rational eral neuropathy, mental retardation, or a thin corpus diagnostic strategy for pediatric HSP cases, we per- callosum. HSP may be inherited as an autosomal formed a literature search focusing on presenting signs Address correspondence and dominant, autosomal recessive, or X-linked disease. reprint requests to Dr. S.T. de and symptoms, age at onset, and genotype. We present Over 40 loci and nearly 20 genes have already been Bot, Radboud University a case of a young boy with a REEP1 (SPG31) mutation. Nijmegen Medical Centre, identified.1 Autosomal dominant transmission is ob- Department of Neurology, PO served in 70% to 80% of all cases and typically re- Box 9101, 6500 HB, Nijmegen, CASE REPORT A 4-year-old boy presented with 2 the Netherlands progressive walking difficulties from the time he sults in pure HSP. -
Accelerating Research. Empowering Families
RESEARCH STRATEGY AND MISSION We aggressively pursue research to identify treatments and a cure for Rett syndrome. New Mecp2 female mouse model developed AMO receives FDA Orphan Drug Designation 2018 + BEYOND With your support, we can Neuren begins plans for trofinetide Phase 3 continue to blaze a trail in Rett syndrome research and family 2017 14 clinics designated empowerment to transform lives. as Rett Syndrome Clinical Research First multi-site, Centers of Excellence multi-country clinical Join us in our mission: trial begins: sarizotan • Make a donation 2015 • Coordinate a Fundraiser Clinical trial for • Participate in an Event trofinetide begins • Advocate for Rett syndrome NIH funding of the NHS begins Visit www.rettsyndrome.org or Drug screening Scout program begins call 1.800.719.8214 2014 First multi-site clinical trial Rettsyndrome.org is a 501(c)3 organization in RTT begins: NNZ-2566 (trofinetide) Accelerating dedicated to accelerating research for treatments and a cure for Rett syndrome and related disorders, 2013 while providing family empowerment. As a Established stem Research. leading organization for Rett syndrome research, cell model for Rettsyndrome.org is committed to funding high- drug screening First clinical trial quality, peer-reviewed research grants and programs. in RTT supported by Rettsyndrome.org: IGF-1 Empowering Genetic manipulation 2010 and biochemical Families. intervention improve Rett-like symptoms in a mouse model 2007 4600 Devitt Drive Cincinnati, OH 45246-1104 ‘‘ (800) 818-7388 www.rettsyndrome.org I am very thankful that Rettsyndrome.org has taken such a strong leadership role /rettsyndrome /rettsyndrome /rettsyndromeorg with advancing research. Their progress to get trofinetide to market is very exciting as it could finally be an answer to relieving some of Jill’s daily struggles. -
Pathophysiology of Dysarthria in Cerebral Palsy
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.44.11.1013 on 1 November 1981. Downloaded from Journal of Neurology, Neurosurgery, and Psychiatry 1981 ;44:1013-1019 Pathophysiology of dysarthria in cerebral palsy PETER D NEILSON, NICHOLAS J O'DWYER From The Spastic Centre Research Unit, Department of Neurology, The Prince Henry Hospital and School of Medicine, University of New South Wales SUMMARY Electromyograms were recorded with hooked-wire electrodes from sixteen lip, tongue and jaw muscles in six normal and seven cerebral palsied adult subjects during a variety of speech and non-speech tasks. The recorded patterns of muscle activity fail to support a number of theories concerning the pathophysiology of dysarthria in cerebral palsy. There was no indication of weakness in individual articulator muscles. There was no evidence of uncontrolled sustained background activity or of abnormal tonic stretch reflex responses in lip or tongue muscles. Primitive or patho- logical reflexes could not be elicited by orofacial stimulation. No imbalance between positive and negative oral responses was observed. The view that random involuntary movement disrupts essentially normal voluntary control in athetosis was not supported. Each cerebral palsied subject displayed an idiosyncratic pattern of abnormal muscle activity which was reproduced across repeti- guest. Protected by copyright. tions of the same phrase, indicating a consistent defect in motor programming. There has been little experimental verification of disruption of the voluntary control of speech muscles existing theories concerning the pathophysiology of by random involuntary activity of the type associated dysarthria in cerebral palsy. The present study with athetosis.6 12 15 provides electromyographic (EMG) data in the light EMG studies of the speech musculature in cerebral of which these theories can be examined. -
Hereditary Spastic Paraparesis: a Review of New Developments
J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.69.2.150 on 1 August 2000. Downloaded from 150 J Neurol Neurosurg Psychiatry 2000;69:150–160 REVIEW Hereditary spastic paraparesis: a review of new developments CJ McDermott, K White, K Bushby, PJ Shaw Hereditary spastic paraparesis (HSP) or the reditary spastic paraparesis will no doubt Strümpell-Lorrain syndrome is the name given provide a more useful and relevant classifi- to a heterogeneous group of inherited disorders cation. in which the main clinical feature is progressive lower limb spasticity. Before the advent of Epidemiology molecular genetic studies into these disorders, The prevalence of HSP varies in diVerent several classifications had been proposed, studies. Such variation is probably due to a based on the mode of inheritance, the age of combination of diVering diagnostic criteria, onset of symptoms, and the presence or other- variable epidemiological methodology, and wise of additional clinical features. Families geographical factors. Some studies in which with autosomal dominant, autosomal recessive, similar criteria and methods were employed and X-linked inheritance have been described. found the prevalance of HSP/100 000 to be 2.7 in Molise Italy, 4.3 in Valle d’Aosta Italy, and 10–12 Historical aspects 2.0 in Portugal. These studies employed the In 1880 Strümpell published what is consid- diagnostic criteria suggested by Harding and ered to be the first clear description of HSP.He utilised all health institutions and various reported a family in which two brothers were health care professionals in ascertaining cases aVected by spastic paraplegia. The father was from the specific region. -
Outcomes Following Unilateral Selective Dorsal Rhizotomy In
Outcomes and Perioperative Considerations for Unilateral Selective Dorsal Rhizotomy in Children with Spastic Hemiplegia with Pre- and Postoperative Quantitative Gait Analysis Christine Hunt, D.O.1, Nicholas Wetjen, M.D.2, Kenton Kaufman, Ph.D.3, Krista Coleman Wood, P.T., Ph.D.3, Joline Brandenburg, M.D.1, Bradford Landry, D.O.1 1Department of Physical Medicine & Rehabilitation, 2Department of Neurologic Surgery, 3Department of Orthopedic Surgery Mayo Clinic, Rochester, MN Abstract Background & Objectives Methods Results: Postoperative Gait Analysis Discussion Background: Selective dorsal rhizotomy (SDR) is a Background Preoperative Baseline Characteristics Patient 1: Right SDR December 2013 • Pre-SDR, patients undergo an in-depth review of their medical procedure used to improve function, decrease pain and • Several human trials examining outcomes in SDR in children • Patient 1: 6 year old male, spastic right hemiplegia • 62.5% of sensory dorsal rootlets sectioned ( L2 to S1) history and imaging studies, consultation with a physiatrist, reduce spasticity in children and adults with cerebral palsy or neurosurgeon, and orthopedic surgeon, and evaluation with PT with spastic diplegia have been conducted, but there is a • GMFCS Level II • Normalized velocity and stride length stroke. Positive outcomes have been reported by numerous and OT. Testing includes QGA, MRI lumbar spine and brain, paucity of data describing outcomes following SDR for • 12 series of botulinum toxin • Improved hip and knee kinematics and kinetics authors but pediatric -
Cerebral Palsy the ABC's of CP
Cerebral Palsy The ABC’s of CP Toni Benton, M.D. Continuum of Care Project UNM HSC School of Medicine April 20, 2006 Cerebral Palsy Outline I. Definition II. Incidence, Epidemiology and Distribution III. Etiology IV. Types V. Medical Management VI. Psychosocial Issues VII. Aging Cerebral Palsy-Definition Cerebral palsy is a symptom complex, (not a disease) that has multiple etiologies. CP is a disorder of tone, posture or movement due to a lesion in the developing brain. Lesion results in paralysis, weakness, incoordination or abnormal movement Not contagious, no cure. It is static, but it symptoms may change with maturation Cerebral Palsy Brain damage Occurs during developmental period Motor dysfunction Not Curable Non-progressive (static) Any regression or deterioration of motor or intellectual skills should prompt a search for a degenerative disease Therapy can help improve function Cerebral Palsy There are 2 major types of CP, depending on location of lesions: Pyramidal (Spastic) Extrapyramidal There is overlap of both symptoms and anatomic lesions. The pyramidal system carries the signal for muscle contraction. The extrapyramidal system provides regulatory influences on that contraction. Cerebral Palsy Types of brain damage Bleeding Brain malformation Trauma to brain Lack of oxygen Infection Toxins Unknown Epidemiology The overall prevalence of cerebral palsy ranges from 1.5 to 2.5 per 1000 live births. The overall prevalence of CP has remained stable since the 1960’s. Speculations that the increased survival of the VLBW preemies would cause a rise in the prevalence of CP have proven wrong. Likewise the expected decrease in CP as a result of C-section and fetal monitoring has not happened. -
Effectiveness of Myofascial Release on Spasticity and Lower Extremity
hysical M f P ed l o ic a in n r e u & o R J International Journal of Kumar and Vaidya, Int J Phys Med Rehabil 2014, 3:1 l e a h n a DOI: 10.4172/2329-9096.1000253 o b i t i l a ISSN: 2329-9096i t a n r t i e o t n n I Physical Medicine & Rehabilitation Research Article Open Access Effectiveness of Myofascial Release on Spasticity and Lower Extremity Function in Diplegic Cerebral Palsy: Randomized Controlled Trial Chandan Kumar1* and Snehashri N Vaidya2 1Associate Professor, Smt Kashibai Navale College of Physiotherapy, Narhe, Pune, Maharashtra, India 2MGM’S School of Physiotherapy, Aurangabad, India Abstract Purpose: To find out the effectiveness of Myofascial Release in combination with conventional physiotherapy on spasticity of calf, hamstring and adductors of hip and on lower extremity function in spastic diplegic subjects. Methodology: 30 spastic diplegic subjects of age group 2-8 years were taken by random sampling method from MGM College and other private clinics in Aurangabad. 15 subjects were assigned in each group. Group A: Myofascial release and conventional PT treatment. Group B: conventional PT treatment. Both the groups received training for 4 weeks. Baseline and Post treatment measures of Modified Ashworth Scale (MAS), Modified Tardieu Scale (MTS) and Gross Motor Function Test (GMFM-88) were evaluated. Results: Mean difference of MAS and R2 value of MTS in group A was more than group B, for calf, hamstring and adductors, whereas GMFM showed nearly equal improvement in both groups. Conclusion: Overall, it can be concluded from our study that the MFR along with conventional treatment reduces spasticity in calf, hamstring and adductors of hip in spastic diplegic subjects.