Developmental Disabilities and Pervasive Developmental disorders LEC: Pervasive Developmental Disabilities and Developmental Disabilities Developmental Disabilities and Pervasive Developmental disorders Dr. Sophia Hrycko April 15, 2010

Objectives To review Developmental Disabilities To review Pervasive Developmental Disorders To discuss comorbidity and treatment options

Developmental Disability Often diagnosed in infancy Mental retardation is the result of a pathological process in the brain characterized by limitations in intellectual and adaptive function. Areas of function affected: communication, self-care, independence, functional/academic skills, work, health, leisure, safety

DSM-IV-TR Mental retardation requires intellectual deficits (IQ measured by standardized test) and deficit in adaptive function (use of measure with deficits in at least two areas of deficits, Vineland Adaptive Behavior Scale: communications, daily living skills, socialization and motor skills) Manifested before age of 18

TABLE 21–3. Clinical features of mental retardation LEC: Pervasive Developmental Disabilities and Developmental Disabilities TABLE 21–3. Clinical features of mental retardation Source. Reprinted from American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th Edition, Text Revision. Washington, DC, American Psychiatric Association, 2000. Used with permission.

#1 DSM-IV-TR lists the prevalence of mental retardation in the US as B. 3% C. 5% D. 6% E. None of the above

#1A DSM-IV-TR lists the prevalence of mental retardation in the US as B. 3% C. 5% D. 6% E. None of the above

#2 When IQ is used as the sole criterion for mental retardation, the prevalence rate is estimated to be: A. 0.5 % B. 1% C. 2 % D. 3% E. 10%

#2A When IQ is used as the sole criterion for mental retardation, the prevalence rate is estimated to be: A. 0.5 % B. 1% C. 2 % D. 3% E. 10%

Epidemiology About 1 % of the population. 1.5 time more common in men High mortality rates with severe or profound MR because of complications associated with physical disorders.

Etiology Genetic Developmental/Acquired Environmental/social Down syndrome Fragile X Prader-Willi syndrome PKU Neurofibromatosis Tuberous sclerosis Developmental/Acquired Environmental/social

#3 Moderate Mental retardation A. Reflects an IQ range of 25 to 40 B. Is seen in approximately 3 to 4 % of persons with mental retardation C. Has an identifiable organic etiology in the vast majority of cases D. Usually is associated with the ability to achieve academic skills at the second to 3rd grade level E. All of the above

#3A Moderate Mental retardation A. Reflects an IQ range of 25 to 40 B. Is seen in approximately 3 to 4 % of persons with mental retardation C. Has an identifiable organic etiology in the vast majority of cases D. Usually is associated with the ability to achieve academic skills at the second to 3rd grade level E. All of the above

Acquired/developmental Prenatal: rubella, CMV, Syphilis, Toxoplasmosis, Herpes, AIDS, fetal alcohol syndrome Complications of pregnancy Perinatal Infection, head trauma, etc.

#4 A. Adrenoleukodystrophy B. Rett’s disorder C. Acquired immune deficiency syndrome AIDS D. Rubella E. Cytomegalic virus CMV F. Toxoplasmosis Mental retardation with periventricular intracerebral calcifications, jaundice, microcephaly and hepatosplenomegaly Progressive encephalopathy and MR in 50% of children born to mother with this disorder An X-linked MR syndrome that is degenerative and affects only females Diffulse demyelination of cerebral cortex leading to visual and intellectual impairment, seizures, and spasticity, and adrenocortical insufficiency MR, microcephay, microphthalmia, congenital heart disease, deafness, cataracts

#4A A. Adrenoleukodystrophy B. Rett’s disorder C. Acquired immune deficiency syndrome AIDS D. Rubella E. Cytomegalic virus CMV F. Toxoplasmosis (MR, diffuse intracerebral calcifications, hydrocephalus, seizures and chorioretinitis Mental retardation with periventricular intracerebral calcifications, jaundice, microcephaly and hepatosplenomegaly E Progressive encephalopathy and MR in 50% of children born to mother with this disorder C An X-linked MR syndrome that is degenerative and affects only females B Diffulse demyelination of cerebral cortex leading to visual and intellectual impairment, seizures, and spasticity, and adrenocortical insufficiency A MR, microcephaly, microphthalmia, congenital heart disease, deafness, cataracts D

Comorbidity Up to 2/3 of individuals with MR have comorbid mental disorders. The more severe the MR, the higher the risk for other mental disorders. Disruptive and conduct-disorder behaviors are more frequent in Mild MR Autistic disorder more common with severely retarded individuals.

#5 Common manifestations of anxiety in persons with mental retardation include A. Aggression B. Agitation C. Repetitive behaviors D. Self-injury E. All of the above

#5A Common manifestations of anxiety in persons with mental retardation include A. Aggression B. Agitation C. Repetitive behaviors D. Self-injury E. All of the above

Evaluation Complete history and physical exam Will need to evaluate Intellectual function (WISC or WPPSI) and Adaptive function (Vineland Adaptive Behavior Scale) Sensory screening ( speech, hearing) Laboratory studies: Genetic testing, metabolic testing, thyroid/lead screening, imaging

Practice Parameters: Evaluation of child with Global Develop. Delay Metabolic screening NOT indicated in initial evaluation (yield 1%) Routine cytogenetic studies and molecular testing for FRA X mutation recommended (yield 3.5-10%) Consider Rett syndrome in girls with unexplained moderate to severe delay Serum lead when identifiable risk EEG NOT recommended initially unless features of epilepsy Imaging with MRI > CT if physical findings Shevell et al Neurology 2003 60: 367-380

Down Syndrome Trisomy 21, 95% nondisjunction 1 in 1000 live births                                                          Down syndrome. Note depressed nasal bridge, epicanthal folds, mongoloid slant of eyes, low-set ears, and large tongue. Trisomy 21, 95% nondisjunction 1 in 1000 live births 1 in 80 at 40 yrs Hypotonia, upward slanted palpebral fissures, midface depression, flat wide nasal bridge, simian crease, short stature, increased incidence of thyroid anomaly and congenital heart disease. Passive, affable 25% ADHD Verbal processing > auditory processing Increased risk of depression and dementia as adult

Fragile X Mutation of the FMRI gene at Xq27.3. Full mutation: CGG trinucleotide repeat > 200 to 230 repeats Prevalence 1/1000 male births and 1/3000 female birth Second most known cause of MR of genetic origin (10-12% MR in men) long face, large ears, midface hypoplasia, arched palate                                          

Fragile X Macroorchidism Short stature, strabismus, joint laxity ADHD, anxiety, speech/language delays, shyness, irritability, stereotypies. LD in some female. Male: moderate to severe MR Female: mild MR

#6 Fragile X syndrome A. Has a phenotype that includes postpubertal microorchidism B. Affects only males C. Usually causes severe to profound MR D. Has a phenotype that includes large head and large ears. E. All of the above.

#6A Fragile X syndrome A. Has a phenotype that includes postpubertal microorchidism B. Affects only males C. Usually causes severe to profound MR D. Has a phenotype that includes large head and large ears. E. All of the above.

#7 Which of the following disorders is least often associated with Fragile X syndrome: A. Autistic disorder B. Schizotypal personality disorder C. Attention deficit/hyperactivity disorder D. Bipolar disorder E. Social anxiety disorder

#7A Which of the following disorders is least often associated with Fragile X syndrome: A. Autistic disorder B. Schizotypal personality disorder C. Attention deficit/hyperactivity disorder D. Bipolar disorder E. Social anxiety disorder

Praeder-Willi Syndrome                                                                Deletion on long arm of chr. 15q11-15q13 (70% paternal, rest maternal uniparental disomy) 1 in 15 000 birth Hyperphagia Obesity Small hands/feet Short stature Microorchidism Fair hair/light skin Almond shaped eyes

Praeder-Willi Syndrome                                                                                                   Obsessions and compulsions High rates of behavior problems: aggression, temper tantrums, emotional lability, daytime sleepiness Increased risk for OCD, affective and impulse control disorders.

Phenylketonuria Autosomal Recessive defect in phenylalanine hydroxylase 12q.24.1 or cofactor 11q22.3-q23.3 Cause accumulation of phenylalanine if untreated and will result in MR (mild to profound), microcephaly, delayed speech, seizures and behavior problems (self-injury, hyperactivity) Prevalence 1/12 000 Fair skin, blue eyes, blond hair

Tuberous Sclerosis Autosomal Dominant Mutation in TSC1 gene (hamartin) 9q34 or the TSC2 tumor suppressor gene (tuberin) 16p13 Prevalence 1/6 000 Spectrum of MR, none (30%) to profound Epilepsy, autism, hyperactivity, impulsivity, aggression, self-injurious behaviors, sleep problems

Tuberous Sclerosis                                                                                                                                                                                        Figure 589-2 Tuberous sclerosis. A, CT scan with subependymal calcifications characteristic of tuberous sclerosis. B, The MRI demonstrates multiple subependymal nodules in the same patient (black arrow). Parenchymal tubers are also visible on both the CT and the MRI scan as low-density areas in the brain parenchyma.

Neurofibromatosis type 1 Autosomal dominant 17q11.2 Prevalence 1/3 000 (NF2 1/33 000, 22q) Café au lait spots Neurofibromas Short stature and macrocephaly in 30- 45% 10 % with moderate to profound MR ADHD, anxiety, mood problems                                                                              

Fetal Alcohol Syndrome                                                                                                                                         

Fetal Alcohol Syndrome                                                                          Most common preventable cause of MR 1/3 000 live birth Microcephaly, short stature, midface hypoplasia, short palpebral fissure Thin upper lip, micrognatia, hypoplastic long/smooth philtrum Mild to moderate MR, irritability, memory impairment

#8 A. Prader-Willi syndrome B. Down’s syndrome C. Fragile X syndrome D. Phenylketonuria Attributed to a deletion in chromosome 15 Most commonly occurs via autosomal recessive transmission Abnormalities involving chromosome 21 Occurs via a chromosomal mutation at Xq27.3 Example of a genomic imprinting

#8A A. Prader-Willi syndrome B. Down’s syndrome C. Fragile X syndrome D. Phenylketonuria Attributed to a deletion in chromosome 15 A Most commonly occurs via autosomal recessive transmission D Abnormalities involving chromosome 21 B Occurs via a chromosomal mutation at Xq27.3 C Example of a genomic imprinting A

Pervasive Developmental disorders Autistic Disorder Rett’s Disorder Childhood Disintegrative Disorder Asperger’s Disorder Pervasive Developmental disorder NOS

Autistic Disorder Diagnostic Features Age of onset: prior to age 3 3 main sets of behavioral characteristics:’ - Social abnormality - Language abnormality - Stereotyped repetitive pattern of behavior Age of onset: prior to age 3 Male/Female = 4/1 Prevalence: 9.5 / 10 000 (range 2.3 to 30.8/ 10 000)

Autistic Disorder Course: variable, strongest predictive factors for adult outcome are IQ (below 70 is strongly indicative of poor social adjustment) and the level of language functioning at age 5 Etiology: unknown, evidence of strong genetic component; abnormal serotonergic activity, hyperdopaminergic activity

Diagnostic Criteria Qualitative impairment in Social Interaction Impairment in the use of multiple nonverbal behaviors: eye to eye gaze, facial expression, body postures and gestures to regulate social interaction Failure to develop peer relationships appropriate to developmental level Lack of spontaneous seeking to share enjoyment, interests or achievements with other people (not showing, bringing or pointing out objects of interests) Absence of social or emotional reciprocity

Cont. Qualitative impairment in Communication Delay in or total lack of, development of spoken language (no attempt to compensate with gestures or mime) If speech present, marked impairment in the ability to initial or sustain conversation with others Stereotyped and repetitive use of language or idiosyncratic language Lack of varied, spontaneous make-believe play or social imitative play appropriate to develop. level

Cont. Restricted repetitive and stereotyped patterns of behavior, interest and activities Encompassing preoccupation with one or more stereotyped and restricted patterns of interest that is abnormal either in intensity or in focus Inflexible adherence to specific, nonfunctional routines or rituals Stereotyped and repetitive motor mannerisms: hand or finger flapping or twisting, complex whole-body movements Persistent preoccupation with parts of objects

Consider Evaluation if by: 12 months: No babbling or gesturing (pointing, waving bye-bye) 16 months: No single words 24 months: No spontaneous 2 word phrases (i.e. not just echolalia or repeating someone else’s words) Any age: any loss of any language or social skills

Consider Evaluation if Especially when combined with language delays: Abnormal eye contact Aloofness Not responding to one’s name Not using gestures to point or show Lack of interactive play Lack of interest in other children

#9 Which of the following features does not distinguish autistic disorder from mixed receptive-expressive language disorder? A. Echolalia B. Stereotypies C. Imaginative play D. Associated deafness E. Family history of speech delay

#9A Which of the following features does not distinguish autistic disorder from mixed receptive-expressive language disorder? A. Echolalia B. Stereotypies C. Imaginative play D. Associated deafness E. Family history of speech delay (25% for both autistic and language disorders)

#10 Neurological-biochemical anomalies associated with autistic disorder include A. Grand mal seizures B. Ventricular enlargement on CT scan C. EEG abnormalities D. Increased brain volume E. All of the above

#10A Neurological-biochemical anomalies associated with autistic disorder include A. Grand mal seizures (4 to 32 %) B. Ventricular enlargement on CT scan (20 to 25%) C. EEG abnormalities D. Increased brain volume (cerebellum, frontal lobe and limbic system) E. All of the above

Autistic Disorder Associated Features IQ below 70 for 75% of autistics Uneven cognitive skills Level of receptive language below expressive language Behavioral symptoms: hyperactivity, impulsivity, aggressiveness, self-injurious behavior (head banging, finger/hand/wrist biting), temper tantrums Abnormal mood (giggling or weeping) Lack of fear

Autistic Disorder Associated Findings Abnormal Imaging Studies: underactivation of fusiform gyrus, abnormality in the medial temporal lobe, increase in brain size in some EEG abnormalities: varied, non-specific Non-specific neurological symptoms: primitive reflexes, delayed hand dominance Medical conditions associated with Autism: encephalitis, neurofibromatosis, PKU untreated, tuberous sclerosis, fragile X, anoxia, maternal rubella Epilepsy in 10 – 35%

ex. Word over, video of a dog, boat Sensory problems Temple Grandin A narrow fixation, with assistance can be broadened into a career (tuning piano, reshelving books, computer programming, etc.) Recognize the social limitations and protect from situations beyond their capacity Visual thinking: “all my thinking is in pictures”, abstract concept are represented by pictures. ex. Word over, video of a dog, boat Sensory problems

#11 True statements about autistic disorder include which of the following? A. Girls outnumber boys in individuals with autism without mental retardation B. There is an established and conclusive association between autism and upper socioeconomic status. C. Prevalence rates amy be as high as 1 in 1000 children. D. Abnormalities in functioning must be present by age 2 to meet DSM-IV-TR diagnostic criteria. E. All of the above.

#11A True statements about autistic disorder include which of the following? A. Girls outnumber boys in individuals with autism without mental retardation (MR 75%) (boy: girls 4-5:1) B. There is an established and conclusive association between autism and upper socioeconomic status. C. Prevalence rates may be as high as 1 in 1000 children. D. Abnormalities in functioning must be present by age 2 to meet DSM-IV-TR diagnostic criteria. E. All of the above.

#12 True statements about the role of genetics in autistic disorder include which of the following: A. Twin studies indicate only moderate concordance for monozygotes. B. Family studies show a prevalence of approximately 2 to 3% of autism among siblings of children with autism. C. Unaffected siblings are not at increased risk for language problems. D. It is clear that what is inherited is a specific predisposition to autistic disorder. E. The role of genetic factors in autistic disorder is not well established.

#12A True statements about the role of genetics in autistic disorder include which of the following: A. Twin studies indicate only moderate concordance for monozygotes. (high, not moderate) B. Family studies show a prevalence of approximately 2 to 3% of autism among siblings of children with autism. C. Unaffected siblings are not at increased risk for language problems. (elevated rates of cognitive disorders in non-autistic twin) D. It is clear that what is inherited is a specific predisposition to autistic disorder. E. The role of genetic factors in autistic disorder is not well established.

Rett’s Disorder (Andreas Rett 1966) All of the following are required: 1. Apparently normal prenatal and perinatal development 2. Apparently normal psychomotor development through the first 5 months after birth 3. Normal head circumference at birth

Rett’s Disorder cont. Onset of all of the following after the period of normal development: 1. Deceleration of head growth between ages 5 and 48 months 2. Loss of previously acquired purposeful hand skills between ages 5 and 30 months with the subsequent development of stereotyped hand movements (e.g. hand-wringing or hand washing) 3. Loss of social engagement early in the course 4. Appearance of poorly coordinated gait or trunk movements 5. Severely impaired expressive and receptive language development with severe psychomotor retardation

Rett’s Disorder cont Prevalence rate: 1/ 15000 – 22 000 females 26% incidence of sudden and unexpected death X-linked dominant mutation with lethality in hemizygous males Mutation in the transcription regulatory gene MECP2 Stages: Normal prenatal/perinatal development Period of developmental stagnation Gradual, insidious delay in development, decelerated head and body growth, lack of interest in the environment, loss of previously acquired skills (purposeful hand movements)

Rett’s Disorder cont Developmental plateau (school age) Final phase Severe MR Seizures Motor problems Breathing difficulties (breath-holding spells, air swallowing) Bruxism Scoliosis Final phase Nonambulatory secondary to motor problems, scoliosis

#13 Rett’s disorder A. Is seen only in boys B. Does not involve motor abnormalities C. Is associated with normal intelligence D. Shows no loss of social skills. E. None of the above

#13A Rett’s disorder A. Is seen only in boys B. Does not involve motor abnormalities C. Is associated with normal intelligence D. Shows no loss of social skills. E. None of the above

Childhood Disintegrative Disorder (Heller 1908) Apparently normal development for at least the first 2 years after birth as manifested by the presence of age-appropriate verbal and nonverbal communication, social relationships, play and adaptive behavior Clinically signif. loss of previously acquired skills (before age 10) in > areas: Expressive or receptive language Social skills or adaptive behavior

Childhood Disintegrative Disorder cont. Bowel or bladder control Play Motor skills Abnormalities of functioning in at least 2 areas: Qualitative impairment in social interactions Qualitative impairment in communication Restricted, repetitive and stereotyped patterns of behavior, interests and activities, including motor stereotypies and mannerisms

Childhood Disintegrative Disorder Prevalence: 1.7 per 100 000 Presence of a period of normal development before the onset of the deterioration and loss of skills Typical age of onset 3 to 4 y old Very rare, strong male predominance Deterioration in self-help and motor skills is often marked Apparently normal fife expectancy Has been associated with metachromatic leukodystrophy, Schilder’s leukoencephalopathy

Asperger’s Disorder Impairment in social interactions Marked impairment in the use of multiple nonverbal behaviors such as eye-to-eye gaze, facial expression, body postures, and gestures to regulate social interaction Failure to develop peer relationships appropriate to developmental level A lack of spontaneous seeking to share enjoyment, interests or achievements with other people Lack of social or emotional reciprocity

#14 Asperger’s disorder is characterized by delays in A. Self-help skills B. Curiosity about the environment C. Nonverbal communication D. Receptive language E. None of the above.

#14A Asperger’s disorder is characterized by delays in A. Self-help skills B. Curiosity about the environment C. Nonverbal communication D. Receptive language E. None of the above.

Asperger’s Disorder Restricted repetitive and stereotyped patterns of behavior, interests and activities There is no clinically significant general delay in language There is no clinically significant delay in cognitive development or in the development of age-appropriate self-help skills, adaptive behavior (other than in social interactions) and curiosity about the environment in childhood

Asperger’s Disorder cont. Prevalence estimated to 1 in 10 000 More prevalent in males than females, ratio of 9 to 1 Normal language development but their facial expression, prosody and social gestures are often deficient. Lack “intuitive knowledge” of how to approach others. Delayed motor milestones, motor clumsiness Have to learn social skills through their intellect

Evaluation History Psychiatric examination of the child Pregnancy, neonatal and developmental hx, medical hx, family and psychosocial factors, intervention hx. Psychiatric examination of the child Medical evaluation Physical exam, including neurological exam Audiological/visual exam Psychological evaluation Speech/language/communication assessment OT evaluation

Differential Diagnosis Various PDDs MR not associated with PDD Specific developmental disorder, e.g. language Early onset psychosis

Treatment Plan Multimodal Establish goals for educational interventions Establish target symptoms for intervention Prioritize target symptoms and/or co-morbid conditions Monitor multiple domains of functioning (behavioral adjustment, adaptive skills, academic skills, social/communicative skills, social interactions) Monitor pharmacological interventions for efficacy and side-effects.

Potential Targets for Pharmacotherapy Motor hyperactivity Inattention Repetitive behavior Motor and/or vocal tics Aggression Self-injury

#15 A. Autistic disorder B. Childhood disintegrative disorder - Normal development for the 1st 6mo, followed by a progressive encephalopathy: A better prognosis than other PDD because of the lack of delay in language and cognitive development Some but not all the features of autistic disorder Occurrence at a rate of 2 to 10 per 10 000 and impairment in social interaction, communication (language or symbolic play) before age 3 A. Autistic disorder B. Childhood disintegrative disorder C. Pervasive developmental disorder NOS D. Asperger’s Disorder E. Rett’s disorder

#15A A. Autistic disorder B. Childhood disintegrative disorder - Normal development for the 1st 6mo, followed by a progressive encephalopathy: E A better prognosis than other PDD because of the lack of delay in language and cognitive development: D Some but not all the features of autistic disorder C Occurrence at a rate of 2 to 10 per 10 000 and impairment in social interaction, communication (language or symbolic play) before age 3 A A. Autistic disorder B. Childhood disintegrative disorder C. Pervasive developmental disorder NOS D. Asperger’s Disorder E. Rett’s disorder

#16 Which of the following chromosomal abnormalities is most likely to cause mental retardation? A. Extra chromosome 21 (trisomy 21) B. Fusion of chromosomes 21 and 15 C. XO Turner’s syndrome D. XXY Kinefelter’s syndrome E. XXYY and XXXY Klinefelter’s syndrome variants

#16A Which of the following chromosomal abnormalities is most likely to cause mental retardation? A. Extra chromosome 21 (trisomy 21) B. Fusion of chromosomes 21 and 15 C. XO Turner’s syndrome D. XXY Kinefelter’s syndrome E. XXYY and XXXY Klinefelter’s syndrome variants

#17 The prognosis of autistic disorder is most accurately described by which of the following statements? A. The prognosis is good if the onset of the illness is at birth B. The prognosis is good if the child has normal auditory evoked potentials C. The prognosis is determined by language development D. The prognosis is bad if either of the child’s parents has manic-depressive illness E. None of the above

#17A The prognosis of autistic disorder is most accurately described by which of the following statements? A. The prognosis is good if the onset of the illness is at birth B. The prognosis is good if the child has normal auditory evoked potentials C. The prognosis is determined by language development D. The prognosis is bad if either of the child’s parents has manic-depressive illness E. None of the above

#18 All of the following statements concerning autistic disorder are true EXCEPT A. Incidence appears to be highest in upper socioeconomic strata B. It occurs more commonly in boys than in girls C. It appears to be a neurologically based syndrome D. Mental retardation may or may not occur E. Grand mal seizures frequently occur

#18A All of the following statements concerning autistic disorder are true EXCEPT A. Incidence appears to be highest in upper socioeconomic strata B. It occurs more commonly in boys than in girls C. It appears to be a neurologically based syndrome D. Mental retardation may or may not occur E. Grand mal seizures frequently occur

#19 The hallmark feature of autistic spectrum disorder is: A. Delayed expressive language B. Echolalia C. Functional intelligence quotient in the superior range. D. Inability to relate socially. E. Stereotypy

#19A The hallmark feature of autistic spectrum disorder is: A. Delayed expressive language B. Echolalia C. Functional intelligence quotient in the superior range. D. Inability to relate socially. E. Stereotypy

References http://www.mic.ki.se/Diseases/C16.html http://medgen.genetics.utah.edu/thumbnails.htm http://ca.dir.yahoo.com/Health/diseases Fra X: http://www.fraxa.org Handbook of Developmental Disabilities SL Odom, RH Horner, ME Snell, J Blacher eds. 2007 The Guilford Press

References Child Adol Psych Clin NA 16 (2007) Fragile X syndrome 663-675 VCFS 677-693 Praeder-Willi 695-708 Fetal alcohol spectrum disorder: Canadian guidelines for diagnosis AE Chudley, J Conry, JL Cook, C Loock, T. Rosales, N LeBlanc CMAJ Mar 1, 2005 172 (5 suppl) S1-S21

References Volkmar F, Cook et al 1999. Practice parameters for the assessment and treatment of adolescents and adults with autism and other PDD. J. Am. Acad. Child & Adol. Psych. 38 (12 suppl): 32S- 54 S (erratum 2000 39 (7): 938 and 38: 12: 1611- 1615 Mental Retardation: A Review of the Past 10 Years. Part 1. B.H. King et al 1997. J. Am. Acad. Child Adole. Psych. 36:12, 1656- 1663 (1664 – 1671 for part II)

#20 The most frequent presenting complaint of parents about their autistic child is: A. Their lack of interest in social interaction. B. Their lack of usual play skills. C. Their difficulty tolerating change and variations in their routines. D. Delays in the acquisition of language. E. Stereotyped movements.

#20A The most frequent presenting complaint of parents about their autistic child is: A. Their lack of interest in social interaction. B. Their lack of usual play skills. C. Their difficulty tolerating change and variations in their routines. D. Delays in the acquisition of language. E. Stereotyped movements.

#21 What percentage of autistic individuals exhibits special abilities or splinter skills? A. Less than 1% B. 10 % C. 25 % D. 50 % E. 80 %

#21A What percentage of autistic individuals exhibits special abilities or splinter skills? A. Less than 1% B. 10 % C. 25 % D. 50 % E. 80 %

#22 A decline in IQ begins at appromixately 10 to 15 years in which of the following disorders? A. Down’s syndrome B. Fragile X syndrome C. Cerebral palsy D. Nonspecific mental retardation E. Fetal alcohol syndrome

#22A A decline in IQ begins at approximately 10 to 15 years in which of the following disorders? A. Down’s syndrome B. Fragile X syndrome C. Cerebral palsy D. Nonspecific mental retardation E. Fetal alcohol syndrome

#23 The most common inherited cause of mental retardation is A. Down’s syndrome B. Fragile X syndrome C. Fetal alcohol syndrome D. Prader-Willi syndrome E. None of the above

#23A The most common inherited cause of mental retardation is A. Down’s syndrome (most common chromosomal abnormality leading to MR) B. Fragile X syndrome (most common inherited cause of MR) C. Fetal alcohol syndrome D. Prader-Willi syndrome E. None of the above

#24 Mild mental retardation has been associated with A. Nonspecific causes B. Prader-Willi syndrome C. Females with fragile X syndrome D. Poor socioeconomic background E. All of the above

#24A Mild mental retardation has been associated with A. Nonspecific causes B. Prader-Willi syndrome C. Females with fragile X syndrome D. Poor socioeconomic background E. All of the above