Pervasive developmental disorders: A hidden disability in adolescence

Pervasive developmental disorders: A hidden disability in adolescence

!OURNAL OF ADOLESCENT HEALTH 1!293;14:32!2-335 CASE REPORTS HOWARD B. DEMB, M.D., AND SUSAN Social skills are discrete verbal and nonverbal behav...

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!OURNAL OF ADOLESCENT HEALTH 1!293;14:32!2-335

CASE REPORTS

HOWARD

B. DEMB, M.D.,

AND SUSAN

Social skills are discrete verbal and nonverbal behaviors as well as other specific cognitive functions which are utilized during an interaction with another person. Good social skills are especially important in adolescence as they are critical for peer acceptance and the ability to make and keep friends. Poor social skills can lead to peer rejection and have been associated not only with social problems, but also with behavioral, emotional, and even academic problems (1). Social skills deficits are observed in several different psychiatric syndromes whose onset is early in lie, including mental retardation (2); pervasive developmental disorders (3-S); the syndrome of disturbed behaviors associated with attention-deficit hyperactivity disorder (9); learning disabilities (10,ll); language disorders (12); childhood onset schizophrenia (13,14); a history of very-low birth weight (VLBW< 1500g) (15);although they may be nonspecific (16). While social skills deficits are typical of many childhood psychiatric disorders, the only two disorders which are currently defined in part by the presence of a social skills disorder are mental retardation and a pervasive developmental disorder. The term pervasive developmental disorder was chosen with the intent of signifying that individuals with this disturbance have a core deficit which involves many basic areas of psychological development. The general category of pervasive developmental disorders has one subgroup, the autistic disorder. The Fromthe Children’s hhation and RehabilitationClinic, Rose F. Kennedy UAP, Albert Einstein College of Medicine, Bronx, New York. Addressreprint requests to:Children’sEvaluationandRehabilitation Clinic, Rose F. Kennedy UAP, Albert Einstein Collegeof Medicine, 1410 Pelham Parkway South, Bronx, NY IO461. Manuscriptaccepted December 5, 2992.

. PINCUS,

M.D.

diagnosis of an autistic disorder is made if an individual currently has at least eight of 16 symptoms or criteria listed in the Diagnostic and Statistical Manual of Mental Disorders (DSM III-R) (17) (see Appendix). The symptoms aie divided into three categories: qualitative impairments in social interactions; qualitative impairments in verbal and nonverbal communication and imaginative activity; and markedly restricted repertoires of activities and interests. The individual’s developmental level must be considered when deciding if the behavior is abnormal, and there must be at least two symptoms of impaired social interaction, and one each of symptoms of impaired communication and restrictions of activities or interests included in the symptom picture in order to meet the criteria for an autistic disorder. When there are qualitative impairments in the development of reciprocal social interactions and of communication and imaginative activities, but the criteria for an autistic disorder, schizophrenia, or schizotypal or schizoid personality disorder are not met, the individual may be classified as having a pervasive developmental disorder not otherwise specified (PDDhKX). Children with nonautistic pervasive developmental disorders (PDDNGS) are heterogeneous with respect to clinical characteristics but generally have better social and communicative skills than those with an autistic disorder, and the prevalence of PDDNOS is higher than that of the autistic disorder (17). A group of children diagnosed as preschoolers as having an atypical pervasive developmental disorder, the DSM III (18) diagnostic equivalent of the PDDNOS, were described in 1987 (5). They were described as having social skills which were variable, rather than as having failed to develop interpersonal relationships or as totally lacking responsiveness to,

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The patients were referred for diagnosis and treatment to the Adolescent Unit (AU) of the Children’s Evaluation and Rehabilitation Center, of the Rose F. Kennedy Center, of the Albert Einstein College of Medicine. All five had been evaluated earlier in their lives and at an average age of less than 5 years had been given one or more of the following d&noses: mental retardation; attention-deficit disorder with hyperactivity; oppositional defiant disorder; expressive and/or receptive language disorder; or a learning disability. The chief complaints at the time of their initial evaluations were hyperactivity; a short attention span; delayed language development; generally delayed development; or school problems. The adolescents were, on average, slightly more than 14 years old at the time of their referral to the AU. The chief complaints then w’ere rage attacks; oppositlonal/deflant behaviors; sexually provocative talk to or about girls of minority ethnicity; provocative (critical/embarrassing) comments to strangers with physical handicaps; academic difficulties; aggression; or, spending a good deal of time and money calling 900 numbers.

sire and advancing age, coupled PJvithhis behavior, led his parents to worry about their own and their son’s welfare. J.L. was the product of a full-term gestation to a primiparous 25-year old. The pregnancy was complicated by high blood pressure in the iast week and a partial abruptio placenta. Birth weight was 5 lb 5 oz. Medical history was unremarkable except for irritability as an infant and encopresis until age 13 years. Family history was significant for a maternal uncle with a learning disability. The patient was referred by his pediatrician for his first evaluation at our center at the age of 3 years 6 months. At that time he was speaking only a few words, was distractible, head-banged, was hyperactive, and had problems with gross and fine motor skills. Throughout his childhood, J.L. was followed at our center for speech delay, behavioral problems which included an inability to relate well to peers, and educational planning. His behavior made it necessary to start him on medication at age 10 years. After methylphenidate failed he was put on thioridazine, which he continues to take At 14 years 5 months, J.L. asked many inappropriate questions in a loud and pedantic voice, He was hyperactive and exhibited poor contact as he spoke. His responses to questions were vague and tangential. His difficulties in articulating ideas and tendency toward concreteness were inconsistent with his relatively adequate verbal I.Q. He was preoccupied with, and indicated a good deal of information about, areas of his city with high minority populations. He said all of his friends were female “because they are black.” He did not want to go on to high school the following year becaltee he was afraid he would be teased and his lunch would be stolen. A physical examination revealed him to be hyperreflexic and clumsy but otherwise normal.

case 1: J.L.

Case 2: J.A.

J.L. was referred to the Children’s Evaluation and Rehabilitation Center, AU, at the age of 14 years 5 months. He was brought by his parents who were concernedabout a 3-year escalation in longstanding behavior problems. The parents described rage attacks, primarily directed at his mother, and making frequent sexually provocative comments to or about girls of minority ethnic& J.L.‘s most recent rage attack had involved him punching his mother because two cousins had misplaced his keys. His large

J.A. was brought for an evaluation to the Adolescent IJnit of our center at the age of 15 years, 10 months. This was her first multidisciplinary evaluation, other then those done by the school system for long-term behavior and educational difficulties. Her parents requested clarification of their daughter’s diagnosis and help with planning for her future, voicing concern about her safety. Her immature and provocative behavior, they felt, could place her in sexual danger. She would sit on the laps of boys who were

or iflterest in, people. The variability made parents feel that the children were “not listening” at times. The children were often oppositional. In addition, many were described as being hyperactive, impulsive, or distractible. This paper describes adolescents with longstanding difficulties in both language development and social interaction with adults and other children, who were first diagnosed as having a PDDNOS in adolescence. Their past histories include longsianding communication problems, behaviors which were described as hyperactive, oppositional, and aggressive, and difficulties getting along with peers.

case Histories

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strangers to her, and had recently been found only partially clothed with another girl at school. She had few friends and spent most of her time with her family. During an overnight at her home she thought she would “have fun” with the girls who were sleeping by pouring water on them. They got up and left. She required constant supervision, including a need to be bathed by her mother in order to stay clean. J.A. was born 4 weeks premature after an otherwise normal pregnancy of a 27-year-old multiparous woman. The patient’s birth weight was 4 lb 9 oz. At 3 days of age, the patient had a respiratory arrest and was transferred to a tertiary care hospital. Her neonatal course was again complicated by an elevated bilirubin which required phototherapy. She was discharged at 20 days of age. The medical history was complicated by hypothyroidism which was undiagnosed from age 4 until age 8 years during which time she c.,.dnot grow. At age 8 years she was started on replan,:?rnenttherapy. At age 5 years, she began to have p&t ma1 seizures which continued until age 10 yc+. She took valproic acid until it was discontinued( 3 months prior to this evaluation. At age 9 years, she was struck by an automobile while riding a bicyicle. She hit her head, but there was no loss of consci/ousness or other significant injury. She was encopretic until age 10 years. Family history is significanlt for a maternal cousin with a learning disability. The parents reI rate then other called that J.A. developed at a slower children, and that language skills~were particularly delayed. In 5th grade she started special education classes where she has remained. Behavioral problems were first reported at the age of 9 years. At that time J.A. began to display poor impulse control and inappropriate and aggressive behavior towards peers. Frequently, in an attempt\ to make friends, she would give away new or valuable belongings. At the time of the curren.f&&ration, J.A. presented as an immature fzlolescent who tried very hard, but unsuccessfully, to communicate using teenage jargon or “in group siang” (19).She actually communicated in a very concrete fashion and smiled inappropriately, except w,hen describing relationships with peers, when &lieseemed sad. She spoke at length about a rock group with which she was said to be “obsessed,” fantasized about marrying the lead singer, and stated that all her clothes and bedsheets bore the emblem of this group. At her physical examination she was found to be mildly overweight with no other significant findings.

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Case 3: E.M. EM. was first seen at our center tor an evaluation at 12years, 7 months. She was referred by her school for clarification of her behavior problems and a&tance with management. E.M.‘s great aunt and legal guardian, was requesting residential placement because her hyperactivity, impulsivity, and defiant attitude had become too much for the aunt to cope with. She was also concerned that E.M.‘s happropriate comments toward strangers, particularly those who were disabled, would endanger her. E.M. was becoming increasingly oppositional at home and at school. She had been recently suspended from school for setting fire to the bathroom. Her long-term history of poor peer relations was worsening. E.M.‘s only quiet time involved listening to music. She was quite knowledgeable about rock groups and could remain absorbed for hours listening to tapes. E.M. was born Co a 19-year-old primiparous woman with a history of chronic schizophrenia and, later, mental retardation. E.M. came to live with her great aunt at 4 months of age after her biologic mother attempted suicide. Little is known of the patient’s early medicai history, and later medid hhtory was noncontributory. E.M. was first evaluated at age 2 years because she was “a little slow.“’ In the first grade she was tested and placed in special education. At age 7 years, she was treated with increasing doses of methylphenidate over a 2-year period. The medication was discontinued because of severe rebound hyperactivity

.

At the time of this evaluation, the patient was an unstigmatized girl who spoke with little affect. Her speech was inconsistent; at times it was fast and flat, and at times she mumbled. She was perseverative and at times tangential. She moved her arms in a circular motion as she spoke. Her eye contact was poor, and she was easily distractible. E.M. said that she had friends but that she often tried to make them jealous, and that they fought a lot. She related that the kids in her class frequently hit her. She spoke about not liking those with handicaps. She frequently asked the examiner the price of her clothes. At one point when she guessed $10.00 for a pair of shoes and was told that they in fact cost more, she told the examiner that she had “paid too much.” Her physical examination revealed many minor neurological abnormalities including confusion with directionality and spatial orientation, poor fund of knowledge, inability to name parts of things and

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clumsy gross and fine motor coordination. On a standardized test of intelligence she scored in the range of mild mental retardation. Case 4: M.P. M.P. was referred to the AU of our Center at 14 yeam 2 months by his school guidance counselor. He was said to- have poor social skills and to be “obsessed with sexual fantasies.” On one occasion, he was said to masturbate in front of his counselor. He was said to have touched girls on their breasts and buttocks. He was also described as withdrawn, laughing inappropriately, and as having inappropriate “emotional outbursts.” At the time of our evaluation, the school indicated that M.P. was a “loner” with no friends, and tha-r he tended to repeat things that he was told in an annoying fashion. He had become increasingly withdrawn and isolated in the previous year. At home, he watched many music videos and otherwise spent his time in bed. He had joined a Boys Club but was suspended for pulling a fire nlarm after a dare. The patient was the produL: of full-term uncomplicated pregnancy and delivery to a 27-year-old primiparous woman. His birth weight was 8 pounds 7 ounces. Neonatal course was unremarkable, as were early developmental milestones and his subsequent medical history. Family history is significant on both sides for mental illness, mental retardation, and autism. The patient’s brother was evaluated at our center for school failure and was diamosed as having a pervasive developmental disorder. At age 4 years M.P. was evaluated for hyperactivity, negativism, and manner&tic behaviors. There was a brief trial of methylphenidate which was discontinued because of side effects. Since age 4 years, he has been in special education classes, alternating between classes intended for primarily leamingdisabled children and those aimed at children with emotional problems. He has a longstanding history of manneristic behaviors and talking to himself. M.P. presented as an anxious and guarded adolescent who tended to perseverate. His thinking was at times tangential. His affect was frequently bland and blunted. He focused on particular subjects, such as cable television. He repeated the examiner’s questions in an echolalis fashion. He would occasionally masturbate during testing. He frequently interrupted the examiner to ask where her pictures of movie stars were. &ring his examination, he pulled off pieces of the chair and ate them. He appeared often to have troublefocusing and conce,* rating on

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what was being asked of him, and he needed directions repeated. His eye contact was minimal. On physical examination he was of average size and without stigmata. His examination was essentially normal. Psychological testing resulted in a diagnosis of mild mental retardation.

Case 5: J.F. J.F. was 13 years 7 months when he was referred to the AU of this Center by both his school and his grandmother who were requesting help in finding residential placement. At the time of our evahation, J.F.‘s behavior, which had always been problematic, was further deteriorating. The school noted that, although he was able to have his language needs met, he often used it inappropriately, often “talking on and on and beoaming tangential whether or not anyone was listening.” His interactions with others were markedly impaired. The school reported that his “social interactions are at times rolte and superficial.” He would often “inaccurately assess and describe other’s behavior and situations,” and this “misreading of social cues” resulted in their sense of his “impaired social interactions.” J.F. was also exhibiting some behaviors which had both manic and obsessional and/or compulsive qualities to them. He had begun to repeatedly use the phone at home to make prolonged long distance phone calls, and this had resulted in phone bills of thousands of dollars. The calls were to people he did not know at ‘980’numbers. He would also stay up all night watching cabIe television news. J.F. was becoming increasingly aggressive in school and at home. He had been taken to a psychiatric emergency room after attacking MS grandmother, who had disconnected the phone. He had been suspended twice from school for aggressive behavior toward other children and teachers. J.F. was born by vaginal delivery to a drug- and alcohol-addicted mother. His birth weight was 8 pounds. His biologic mother abandoned him when he was 4 months old, and he has lived with his maternal grandmother since that time. Past medical history is significant for a seizure disorder which began at 18 months of age as grand ma1 seizures which changed to temporal lobe seizures which stopped at age 9 years. He had eye problems since birth, which have been diagnosed as optic atrophy, severe myopia, bilateral exotropia, nystagmus, left gaze preference, and right gaze paresis. Many of his

June 1993

family members have been diagnosed with mental illness. His grandmother noticed some developmental delay from the onset, and he had his first formal evaluation at 18 months of age. At that time, he was found to be visually impaired and to have cerebral palsy. At the age of 4 years, he was evaluated for the first time at our Center for impulsive, distractible, and hyperactive behavior. When J.F. was 8 years old he required another evaluation for worsening behavior. He was started on methylphenidate which he continued to take until age 13years despite lack of improved behavior. At that time, he had a trial of two different neuroleptics. Both were discontinued for intolerable side effects, without improvement in behavior. Tegretol and clomipramine were then tried and have adequately controlled his aggressive and compulsive behaviors until the present. During our evaluation, J.F. presented as a boy who seemed bright but talked continuously. His thoughts were tangential and loose. He frequently made grandiose statements. It was clear he had watched cable television news continuously on the weekend before his appointments. He repeated statements made at the Clarence Thomas Congressional Hearings in a perseverative fashion. He spoke in a nasal and pedantic way and described his “shock and dismay” at some of the allegations made about the Supreme Court nominee. At one point he took off his recently acquired glasses and wanted the examiner to try them on so as to see how she looked in them. He was not testable with the WISCR because of his short attention span. His behavior was immature, emotionally labile, and he became increasingly active as testing went on, On physical examination, J.F. was a thn boy of average height. His eye contact was poor, partially as a result of his paretic gaze. His neurologic examination was remarkable for a right hemiparesis and bilateral gross and fine motor clumsiness.

Discussion The adolescents described all presented with externalizing or disruptive behaviors. While these behaviors are less typical then the withdrawn or internalizing behaviors of children with the more classical form of autistic or autistic-like pervasive de,velopmental disorder, they, nonetheless, represent impaired social skills. One of the more striking features of these individuals as adolescents was their desire to interact with peers and adults, and their

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inability to do SO in a socially acceptable manner. Owing to an inability to predict the consequences of their actions or words on others, their efforts to interact tended to put off or push away the target of their interactive effort, rather than result in a positive social interaction. Or their interaction was such that the adolescent got into trouble by carrying out a socidy inappropriate “dare.” These adolescents have been described as “nerds” (20) while other adjectives used by parents or professionals were “nudgy, ” “goofy,” or “inadvertently provocative.” The communicatjon disorders associated with adolescents diagnosed in adolescence as having PDDNOS are often not of the type which results in an earlier diagnosis of an expressive or receptive language disorder. More often the communication disorder was characterized as an expressive language “difficulty” which was felt to be due to overaP1 cognitive limitations rather than a sign of psychopathology. When seen in adolescence, however, the communication patterns of these adolescents is clearly atypical for any de.gree of cognitive limitation. Thus the use of language to make contact (i.e., “talking to anyone about anything”) or to provoke (i.e., making sexually provocative comments to girls of minority ethnicity, or provocative comments about people with obvious physical handicaps) becomes part of a matrix of communication and social skills difficulties rather than a sign of only a language impairment or intellectual deficit. Once a diagnosis of PDDNOS is made some of the abnormal behaviors can be seen as part of the syndrome rather then as specific diagnoses. For example, because children with atypical pervasive developmental disorders (PDD) are so often found to have hyperactivity and or oppositionalism in their behavior profile (7) the old Axis I (DSM III-R, 1987) diagnosis attention-deficit hyperactivity disorder is not necessary once the Axis Il (DSM III-R, 1987)diagnosis of a nonautistic PDD is made. Likewise, because a language/communication disorder is part of the triad of deficits common to all individuals with a PDD,the additional diagnosis of a language dip order is not made when PDD is the diagnosis. kcaucse of the complex and multifaceted nature of a nonautistic PDD (sometimes referred to as Multiplex Developmental Disorder) (21), parents and professionals, especially teachers, are often quite puzzled about the nature of the adolescents disability and equally perplexed about what 20 do for or about them. There is often a history of it Variety of therapies, (i.e., psychotherapy or counseUing, speech and/or language therapy, academic remedia-

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tion, occupational therapy, physical therapy, pharmacotherapy) and school and or class changes (i.e., public S&OO~, parochial school, classes for languageimpaired children, classes for learning-disabled children, Classes for children with mental retardation and behavior problems, classes for children with emotional handicaps), but at the time of referral both parents and professionals are still looking for the right therapy or school placement. When a pervasive developmental disorder is used as a concept to describe a disorder which includes longstanding deficits in both social and communication skills as well as perhaps some repetitive or ritualized behavior, the parents often react as if a light has been turned on. What had been labeled as “hyper” or “oppositional” behavior are now seen as part of a more global social-communicative skills disorder. Old complaints of “not listening” are now understood to be part of a larger deficit in the ability to carry out tasks which involve a requirement to respond appropriately in a social situation. Similarly, what was sometimes viewed as acceptable, but perhaps overly formal or pedantic expressive language, is then often discoveredto be a skillful USCof delayed echolalia or parroting in an attempt to convey ideas by using phrases or even entire sentences overheard at home, in the community, on recordsor tapes, or on television. Then parents get a feeling that they are beginning to understand the nature of their addescents’ difficulties. The histo~ of these children highlights issues of comorbidity1,1children with nonarytisticPDD. Comorbidityhas been studied in autistic children and adolescents and adults (22-24). Our cases point to a high incidence of signs and symptoms of an attention-deficithyperactivity disorder, or an oppositional defiant disorder, in the early histories of these adolescents; and later signs and symptoms of an effectivedisorderor an obsessive-compulsivedisorder. A study of latency-aged children diagnosed as having a nonautistic PDD (4) found symptoms consistent with a diagnosis of a schizophrenic spectrum disorder (schizotypal personality disorder) and of a conduct disorder, An earlier study (5)found that symptoms of PDD cut across all intellectual levels. These findings should alert clinicians to the fact that there can be a wide range of cognitive abilities or psychiatric disorders coexistent with or superimposed on a pervasive developmental disorder. Often, it is the mmorbid disorder which results in the referral for treatment in adolescents with a nonautistic PDD. However, it is important to recognize the underly_

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ing PDD when making treatment plans, lest the goals of treatment be unrealistic. Even successful treatment of hyperactivity, depreesion, obsessivecompulsive behaviors, or symptoms of a schizophrenic spectrum disorder will not result in a change of the underlying socio-communicative disorder. The current research with regard to the mosf effective treatment of the symptoms of a PDD involves dealing directly with the social skills deficits via social skills training (25,26), and treatment of the communication disorder via language therapy. Combined social skills and communication therapy with children and adolescents with nonautistic PDD is the most efficient model (27-29). However, the broad spectrum of disorders often encountered in individuals with PDDNOS often requires a multidisciplinary approach which includes educational, speech and language and mental health therapies with or without medication. In this paper we have tried to alert clinicians who deal with adolescents to the nature of the presentation in adolescence of individuals with a particular combination of social skills and communication deficits which often results in social ostracism.

Appendix DSM III-R Criteriafor a Pervasive Developmental Disorder A. Qualitative impairments in reciprocal social interactions 1) marked lack of awareness of the existence or feelings of others 2) absent or abnormal seeking of comfort at times of distress 3) absent or impaired imitation 4) absent or abnormal social play 5) impairment in ability to make peer friendships; demonstrates lack of understanding of conventions of social situations B. Qualitative impairments in communication 1) no mode of communication 2) abnormal nonverbal communication 3) absence of imaginative activity 4) marked abnormalities in the production of speech 5) marked abnormalities in the form or content of speech 6) marked irnpairmeni in the ability to initiate or sustain a conversation with others

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C. Markedly restricted repertoire of activities and interests 1) stereotyped body movements 2) persistent preoccupation with parts of objects 3) marked distress over changes in trivial aspects of environment 4) unreasonable insistence on following routines in precise detail 5) markedly restricted range of interests and a preoccupation with one narrow interest

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25. Olley JG. The TEACCH curriculum for teaching social behavior to children with autism. In: Scihopler E, Mesibou GB, eds. Social Behavior and Autism. New York: Plenum, 1986. 26. Rutter M. Autism: Educational issues. Spec Education 1970; 59&10. 27. American Psychiatric Association. Tmatment of psychiatric disorders: A task force rewrt of the American Psvchiatric Association. Washington, k: American Psychiatrir Association, 1989:26&74. 28. Mesibov GB. A cognitive program for teaching social behaviors to verbal autistic adolescents and adults. In: Schopler E, Mesibov GB, eds. Social Behavior and Autism. New York: Plenum, 1986~265-83. 29. McGee GG, Krantz PJ, McClannahan LE. Conversational skills for autistic adolescents: Teaching assertiveness in naturalistic game settings. J Autism Dev Dis 1984;14:319-30.