CHALLENGING CASE: DEVELOPMENTAL DELAYS AND REGRESSIONS

Selective Mutism*

CASE Dr. Martin T. Stein Peter’s parents made an appointment with his pe- is an acquired disorder of inter- diatrician because of their increasing concern about personal communication in which a child does not his refusal to speak. After approximately 2 weeks speak in one or more environments where commu- following the start of a new preschool, Peter, 4 years nication typically occurs. These children most often 10 months old, refused to speak to other children or refuse to speak in school and to adults outside the the teacher. During the first week of school, he found home. Some children with selective mutism will not it difficult to separate from his mother or father. He speak to any child; others will use speech with only would cry and cling to them while asking to go a few other children. Parents report normal speech home. This difficult separation experience gradually within the home with at least one parent and some- subsided by the beginning of the third week and times with siblings. The onset of selective mutism is corresponded with the onset of mute behavior. At usually in the preschool or early school age period of home, he spoke only to his mother, with a clear development; often, it is associated with the start of speech pattern and full sentences. He limited his school. responses to his father or two older siblings with Although this disorder is uncommon (1% among body gestures. He appeared to hear well and under- children seen in mental health centers; 0.1% in the stand verbal directions. general population), parents of these children are Peter’s parents described him as a shy child who understandably alarmed when the behavior persists. eventually makes friends and plays interactively. His Normal language production at home and persistent play, both by himself and with others, is filled with mutism outside the home may be baffling. imaginary activities. There have not been any disrup- The etiology and management of selective mutism tive behaviors at home or with friends. Peter’s ges- have undergone a significant change during the past tation and birth were uneventful. Motor milestones three decades. Clinicians from a variety of disciplines were on time. At 18 months, he spoke only five have discovered that most children with selective words; at 24 months, he spoke 10 words. Two mutism have not experienced a major personal, fam- months after his second birthday, he was speaking with clear and elaborate sentences. ily, or environmental trauma as a trigger for this Physical examination revealed a normal office peculiar behavior. Most recent evidence points to a screening audiogram (25-dB threshold from child’s temperament and behavior consistent with 500–3000 Hz) and the absence of dysmorphic facial anxiety as the clue to more effective management features, cleft palate, middle ear fluid, or a neck strategies. mass. Compliance of the tympanic membrane on I have asked two experienced clinicians to com- pneumo-otoscopy was normal; this was confirmed ment on Peter’s presentation to his pediatrician. Dr. by normal peaks on tympanometry. Peter’s orophar- Isabelle Rapin is Professor of Pediatrics and Neurol- ynx, epiglottis, and uvula were easily visualized and ogy at the Albert Einstein College of Medicine, found to be normal. His facial features, body move- where she has taught pediatric neurology for over ments, and responses to his mother’s commands three decades. I can attest that her reputation as a were consistent with intact auditory receptive func- scholar, teacher, and clinician is unmatched. No pe- tion. In the office, he was calm and remained close to diatric resident has left her clinic without equal re- his mother. His family drawing revealed age-appro- spect for the details of the interview process and the priate fine motor and visual-perceptual skills. All nuances of pediatric neurological examination. Dr. members of his family were drawn with distinctive Rapin has written extensively on neurobehavioral differences in size. Peter was at one end of the family aspects of speech and language disorders in children. group, standing next to his mother. During a 20- Diane Yapko, M.A., is a speech and language pa- minute office assessment, the pediatrician was un- thologist with a remarkable ability to unravel lan- able to engage Peter in spoken speech. guage disorders in children with complex neurolog- ical and behavioral disorders. I asked her to Index terms: selective mutism, mutism, acquired aphasia. comment on management strategies for children with selective mutism.

Martin T. Stein, MD * Originally published in J Dev Behav Pediatr. 1999;20(1) Professor of Pediatrics PEDIATRICS (ISSN 0031 4005). Copyright © 2001 by the American Acad- University of California, San Diego emy of Pediatrics and Lippincott Williams & Wilkins. San Diego, California

926 PEDIATRICS Vol.Downloaded 107 No. 4 from April www.aappublications.org/news 2001 by guest on October 3, 2021 Dr. Isabelle Rapin dren and some 20% of the New York sample had Selective mutism is virtually the only diagnostic delayed or impaired language skills, and some came consideration in this preschooler who was silent in from immigrant homes where another language was school, although he understood what was said to spoken. What characterized both samples was exces- him and was reported to speak normally at home. sive and shyness, not acting out be- However, relying on the parents’ report of normal haviors, or . There was little or no evi- verbal expression at home is inadequate; pediatri- dence for social or emotional trauma as precipitant. cians must insist that the parents bring in an audio or Counseling and psychotherapy were largely inef- videotape, so that they can substantiate the report. If fective. A short open trial of fluoxetine in a dose of it is verified, and if the child is otherwise entirely some 20 mg/day seemed modestly helpful.4 Few normal, the child can safely be spared neuroimaging, long-term outcome data are available, although fam- EEG, and other neurological tests. ily histories suggest that possibly genetic personality If the child is mute or near mute in any environ- traits like anxiety, social , and avoidance may ment, other diagnoses come under consideration (Ta- predispose children to selective mutism. The best ble 1). A transient (the key word is transient, i.e., ploy for management would seem to be acceptance, lasting at most a few hours or a day) aphasia may be sympathetic encouragement, and patience, possibly a postepileptic deficit (Todd’s aphasia) or the mani- with a trial of medication. festation of a migraine, sometimes without head- ache.1 Besides sudden onset deafness—exceedingly Isabelle Rapin, MD Professor of Pediatrics and Neurology rare unless preceded by bacterial meningitis—the Albert Einstein College of Medicine first consideration is an acquired aphasia, since vir- Bronx, New York tually all acquired aphasias in early childhood are expressive, with or without receptive impairment. REFERENCES Without a history of trauma or signs of an infection, 1. Marchioni E, Galimberti CA, Soragna D, et al: Familial hemiplegic an acute brain lesion producing an isolated aphasia migraine versus migraine with prolonged aura: An uncertain diagnosis. is most likely a stroke, perhaps caused by an embo- Neurology 45:33–37, 1995 lus or sickle cell anemia, although a stroke without 2. Steinhausen H-C, Juzi C: Elective mutism: An analysis of 100 cases. associated sensorimotor or other deficit is unlikely. J Am Acad Child Adolesc Psychiatry 35:606–614, 1996 3. Dummit ES III, Klein RG, Tancer NK, et al: Systematic assessment of 50 Acquired epileptic aphasia (Landau-Kleffner syn- children with selective mutism. J Am Acad Child Adolesc Psychiatry drome), without overt clinical seizure but with an 36:653–660, 1997 epileptiform EEG, requires consideration, since it 4. Dummit ES III, Klein RG, Tancer NK, et al: Fluoxetine treatment of may be sudden. A persistent aphasia with an insid- children with selective mutism: An open trial. J Am Acad Child Adolesc ious onset brings up the possibility of a mass lesion Psychiatry 35:615–621, 1996 or a degenerative disease of the brain. Two large series of children with selective mutism, Diane Yapko, M.A. one describing 100 European children,2 the other 50 Primary care pediatricians are increasingly chal- New York children,3 provide concordant data on this lenged to treat a variety of problems that may not be rare frustrating condition of preschoolers and school found in traditional medical training. The case of age children. Girls outnumbered boys 2 to 1. Mean Peter is a typical example. Peter’s symptoms and age of reported onset was 4 years in one series and history are consistent with a diagnosis of selective 21⁄2 years in the other, with a range of 1 to 7 years mutism, as defined in Diagnostic and Statistical (i.e., virtually from first words) in a small proportion Manual of Mental Disorders, 4th edition (DSM-IV).1 of children. The possibility of an autistic spectrum Historically, children with selective mutism have (pervasive developmental) disorder needs to be con- been treated by mental health professionals or sidered in such early cases. The duration was usually speech-language therapists. My professional experi- 5 or more years after exclusion of children who were ence leads me to recommend that physicians con- mute for less than a month, for example, at the start tinue to refer such patients to therapists with exten- of school. Although strict criteria for selective mut- sive pediatric experience and a background in ism would exclude children with developmental lan- cognitive, behavioral, and play therapy. Beyond guage disorders, almost a third of the European chil- making well-considered referrals, however, primary care physicians can skillfully manage these children with a variety of techniques, some of which can be TABLE 1. Mutism in Childhood: Differential Diagnosis illustrated in Peter’s case. Selective mutism correlates more with anxiety dis- Transient aphasias Postepileptic (Todd’s aphasia) orders and social in contrast to the historical Migraine association with oppositional disorders and speech- Deafness language problems.2 This is reflected in the modifi- Acquired aphasias cation of the diagnostic category from “elective” to Trauma Infection “selective” mutism. Some children with selective Stroke mutism may have associated speech-language or be- Acquired epileptic aphasia (Landau-Kleffner syndrome) havioral problems, either at the time of presentation CNS mass lesion or in the past.3 Characteristically, the primary issues CNS degenerative disease are related to anxiety associated with the social con- CNS, central nervous system. text of speaking.

Downloaded from www.aappublications.org/news by guest on October 3, 2021 SUPPLEMENT 927 TABLE 2. Selective Mutism in Children: Guidelines for Parent should be arranged with his parents for later that Education day. Encourage The expectation that your child will begin talking again Diane Yapko, MA All forms of communication (e.g., facial expressions, gestures, Speech-Language Pathologist etc.) Solana Beach, California All attempts at speech (e.g., whispers) Incorporation of teacher in the therapeutic process REFERENCES Discourage Punishment for lack of speech; prevent negative 1. American Psychiatric Association: Diagnostic and Statistical Manual of reinforcement Mental Disorders, 4th ed (DSM-IV). Washington, DC, American Psy- Insistence on speech, which may cause a no-win “power chiatric Association, 1994 struggle” (e.g., “If you want “x” then you have to talk.”) 2. Dummit ES III, Klein RG, Trancer NK, et al: Systematic assessment of 50 Putting him or her “on the spot” to speak in social contexts children with selective mutism. J Am Acad Child Adolesc Psychiatry 36:653–660, 1997 3. Steinhausen H-C, Juzi C: Elective mutism: An analysis of 100 cases. J Am Acad Child Adolesc Psychiatry 35:606–614, 1996 4. Dummit ES, Klein RG, Trancer NK, et al: Fluoxetine treatment of Physicians should avoid searching for a single children with selective mutism: An open trial. J Am Acad Child Adolesc cause of the mutism and especially avoid the sim- Psychiatry 35:615–621, 1996 plistic assumption that it is necessarily rooted in 5. Yapko M: Trance Work: An Introduction to the Practice of Clinical trauma or abuse. There is little empirical evidence to Hypnosis, 2nd ed . . . New York, NY, Brunner/Mazel, 1990 support this claim, and it may alienate the individ- uals with whom one needs to establish a therapeutic Dr. Martin T. Stein alliance, in this case, Peter and his parents.2 Current Selective mutism was described over a century ago research suggests that selective mutism is part of a by the German physician Kussmaul, who wrote personality profile consistent with shy and with- about patients with “aphasia voluntaria.” The early drawn behaviors expressed as mutism when a child psychoanalytic literature contains similar case re- enters school.3 ports. Moritz Tramer, a Swiss child psychiatrist, re- A focused educational approach should be di- ported children with “elective mutism,” a term that rected toward Peter’s parents. Specific suggestions was used to describe this condition in the Diagnostic that follow principles of behavior modification are and Statistical Manual of Mental Disorders, 3rd edi- presented in Table 2. tion (DSM-IIIR). Black and Uhde hypothesized that Fluoxetine has been used effectively with some elective mutism was a variant of social phobia in patients who exhibit selective mutism.4 I have found 19921 and published a systematic study of selective that most parents of these children choose medica- mutism and anxiety disorders in children in 1995.2 tion as a last alternative rather than a first-line ap- The most recent diagnostic classification (DSM-IV) proach. Fluoxetine may be more appropriate for uses the term “selective mutism” to emphasize that those individuals who manifest a chronic form of the disorder is selectively dependent on social con- mutism and for whom previous attempts at behav- text. ioral therapeutic interventions have been ineffective. Studies that link selective mutism to a form of Finally, the clinician’s use of language can be both emphasize antecedent behavior suggestive and influential, and, therefore, must be (shyness, social phobia, avoidance), a family history deliberate.5 Saying, “I wonder which day it will be of anxiety symptoms s(or disorder), and the absence when Peter chooses to talk at school?” or, “I wonder of major psychological as a presenting factor. which friend Peter will choose to talk with first?” are The recent report of a favorable response among important indirect ways of letting Peter know he has children with selective mutism to a selective seroto- choices and that you expect he will speak again. Do nin receptor inhibitor may add support to reframing not speak about your impressions of Peter’s mutism the symptom as an anxiety disorder associated with in front of him. Instead, suggest he play in the wait- biological and environmental risk factors. The path- ing room. If he is not willing to separate from his way to selective mutism might be conceptualized as mother in the office, a phone call to discuss Peter presented in the model in Figure 1.

Fig 1. Conceptualized pathway to selective mutism.

928 SUPPLEMENT Downloaded from www.aappublications.org/news by guest on October 3, 2021 This model surely is an oversimplification to ex- approaches that can be used by pediatric clinicians. plain such an unusual and uncommon symptom. Labbe and Williamson4 use formal behavioral mod- With a high frequency of this temperament profile ification terminology that suggests the mechanism of among children (15–20% of infants; 30–40% of behavioral management strategies: school age children label themselves as shy)3, anxiety Contingency management: Positive reinforcement of disorders in families (5–10%), and the universal oc- verbalization and nonreinforcement for nonverbal currence of life event changes, why is selective mut- responses. ism limited to a small proportion of children with Stimulus fading: Gradually providing opportunities this biological-environmental profile? Perhaps func- (people and places) where verbalization is rewarded. tional neuroimaging studies (with and without neu- Shaping: Rewarding approximations to speech rotransmitter drugs) will provide answers in the fu- (e.g., mouthing words and whispering). ture. Long-term follow-up studies of these children Response cost procedures: Losing tokens or money and their families may also bring about further un- for not speaking. derstanding. Finally, selective mutism may be a final A trial of behavioral management is a reasonable common pathway that is a result of different disor- beginning. The studies using fluoxetine in children ders. Dr. Rapin noted that 20% to 30% of children with selective mutism are promising, but either un- with selective mutism in the two recent studies have controlled (n ϭ 21)5 or controlled, but with a few some form of developmental language delay. Ex- patients (n ϭ 15).6 In these pilot studies, fluoxetine pressive language disorders and articulation disor- was initiated at 10 mg daily, with graduated doses ders were most common. Are these children with guided by a response to verbalization. With a mean selective mutism different from those with normal dose of 21 mg per day (range, 10–60 mg), 76% of the language development? Perhaps some children with children demonstrated increased speech and less early onset of speech and language disorders are anxiety in public settings, including school. Improve- predisposed to a speech avoidance pattern of behav- ment was greater in younger children.5 ior. Management of children with selective mutism REFERENCES begins with consideration of the context, severity, 1. Black B, Uhde TW: Elective mutism as a variant of social phobia. J Am Acad Child Adolesc Psychiatry 31:1091–1094, 1992 and duration of the behavior. Some shy children 2. Black B, Uhde TW: Psychiatric characteristics of children with selective with a history of intense separation reactions will mutism: A pilot study. J Am Acad Child Adolesc Psychiatry 34:847–856, experience a brief period of mutism with the start of 1995 preschool or kindergarten. These episodes are typi- 3. Cheek JM: Shyness, in Parker S, Zuckerman B (eds): Behavioral- cally transient, similar to the behaviors seen at this Developmental Pediatrics: A Handbook for Primary Care. Boston, MA, Little, Brown, 1995, p 285 time during a separation reaction. A resolution fol- 4. Labbe EL, Williamson D: Behavioral treatment of elective mutism: A lows familiarity with a new teacher and new chil- review of the literature. Clin Psychol Rev 4:273–292, 1984 dren. Other children with persistent mutism will 5. Dummit ES III, Klein RG, Tancer NK, et al: Fluoxetine treatment of respond to behavior therapy that offers rewards for children with selective mutism: An open trial. J Am Acad Child Adolesc Psychiatry 35:615–621, 1995 increased speech or attempts at speech (e.g., mouth- 6. Black B, Uhde TW: Treatment of elective mutism with fluoxetine: A ing words or whispers at school, restaurant, or a double blind, placebo-controlled study. J Am Acad Child Adolesc Psy- friend’s home). Ms. Yapko provides several practical chiatry 33:1000–1006, 1994

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