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Title: Developmental Disabilities and Pervasive Developmental disorders


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 213. 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.
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 3rd
    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 3rd
    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. Adrenoleukodystrophy
  • B. Retts 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. Retts 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 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 gt CT if physical findings
  • Shevell et al Neurology 2003 60 367-380

22
Down Syndrome
                                               
          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 gt auditory processing
  • Increased risk of depression and dementia as adult

23
Fragile X
  • Mutation of the FMRI gene at Xq27.3. Full
    mutation CGG trinucleotide repeat gt 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 15 000 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 1/12 000
  • 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 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.
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 30- 45
  • 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. Downs 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. Downs 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
  • Retts Disorder
  • Childhood Disintegrative Disorder
  • Aspergers 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 / 10 000 (range 2.3 to 30.8/
    10 000)

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 elses
    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 ones 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 4-51)
  • 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
Retts 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
Retts 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
Retts 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)

61
Retts 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
  • Retts 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
  • Retts 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 gt 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 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, Schilders leukoencephalopathy

67
Aspergers 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
  • Aspergers 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
  • Aspergers 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
Aspergers 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
Aspergers 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

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
  • - 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. Aspergers Disorder
  • E. Retts disorder

77
15A
  • - 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. Aspergers Disorder
  • E. Retts disorder

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 Turners syndrome
  • D. XXY Kinefelters syndrome
  • E. XXYY and XXXY Klinefelters 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 Turners syndrome
  • D. XXY Kinefelters syndrome
  • E. XXYY and XXXY Klinefelters 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 childs
    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 childs
    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
  • 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

87
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

88
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. 3612, 1656- 1663 (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. Downs 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. Downs 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. Downs 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. Downs 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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