developmental disabilities and pervasive developmental disorders

Developmental Disabilities and Pervasive Developmental disorders

Dr. Sophia HryckoApril 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

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 A. 1 % 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 A. 1 % 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 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 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.

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 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%

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) 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 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 C. Pervasive

developmental disorder NOS

D. Asperger’s Disorder E. Rett’s 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

#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 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

#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/thumbnail

s.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