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

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

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

3 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

4 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

5 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, Used with permission.

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

7 #1A  DSM-IV-TR lists the prevalence of mental retardation in the US as A. 1 % B. 3% C. 5% D. 6% E. None of the above

8 #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%

9 #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%

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.

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

12 #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 3 rd grade level E. All of the above

13 #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 3 rd grade level E. All of the above

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

15 #4  A. Adrenoleukodystroph y  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

16 #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

17 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.

18 #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

19 #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

20 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

21 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 %)  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 :

22 Down Syndrome  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 Down syndrome. Note depressed nasal bridge, epicanthal folds, mongoloid slant of eyes, low-set ears, and large tongue.

23 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

24 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

25 #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.

26 #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.

27 #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

28 #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

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

30 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.

31 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/  Fair skin, blue eyes, blond hair

32 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

33 Tuberous Sclerosis Figure 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.

34 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 %  10 % with moderate to profound MR  ADHD, anxiety, mood problems

35 Fetal Alcohol Syndrome

36 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

37 #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

38 #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

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

40 Autistic Disorder  Diagnostic Features 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 / (range 2.3 to 30.8/ )

41 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

42 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

43 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

44 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

45 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

46 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

47 #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

48 #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)

49 #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

50 #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

51 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

52 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%

53 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

54 #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.

55 #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.

56 #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.

57 #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.

58 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

59 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

60 Rett’s Disorder cont  Prevalence rate: 1/ – 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)

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

62 #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

63 #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

64 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

65 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

66 Childhood Disintegrative Disorder  Prevalence: 1.7 per  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

67 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

68 #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.

69 #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.

70 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

71 Asperger’s Disorder cont.  Prevalence estimated to 1 in  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

72 Evaluation  History 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

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

74 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.

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

76 #15  A. Autistic disorder  B. Childhood disintegrative disorder  C. Pervasive developmental disorder NOS  D. Asperger’s Disorder  E. Rett’s disorder  - Normal development for the 1 st 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 and impairment in social interaction, communication (language or symbolic play) before age 3

77 #15A  A. Autistic disorder  B. Childhood disintegrative disorder  C. Pervasive developmental disorder NOS  D. Asperger’s Disorder  E. Rett’s disorder  - Normal development for the 1 st 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 and impairment in social interaction, communication (language or symbolic play) before age 3 A

78 #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

79 #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

80 #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

81 #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

82 #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

83 #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

84 #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

85 #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

86 References   ls.htm ls.htm   Fra X:  Handbook of Developmental Disabilities SL Odom, RH Horner, ME Snell, J Blacher eds The Guilford Press

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

88 References  Volkmar F, Cook et al 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 (7): 938 and 38: 12:  Mental Retardation: A Review of the Past 10 Years. Part 1. B.H. King et al J. Am. Acad. Child Adole. Psych. 36:12, (1664 – 1671 for part II)

89 #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.

90 #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.

91 #21  What percentage of autistic individuals exhibits special abilities or splinter skills? A. Less than 1% B. 10 % C. 25 % D. 50 % E. 80 %

92 #21A  What percentage of autistic individuals exhibits special abilities or splinter skills? A. Less than 1% B. 10 % C. 25 % D. 50 % E. 80 %

93 #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

94 #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

95 #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

96 #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

97 #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

98 #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


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