pervasive developmental disorders and childhood psychosis

7/31/2019 Pervasive Developmental Disorders and Childhood Psychosis

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Pervasive Developmental Disorders and

Childhood Psychosis

Nelson Series


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Autism Asperger Syndrome Rett Syndrome Childhooddisintegrativedisorder

Pervasivedevelopmentaldisorder nototherwise specified

Delayed and

disorderedcommunication

Similar to autism

except languagerelatively intact

Almost always affects

girls

Clinically significant

regression in skills(language, socialskills, bowel, bladdercontrol, play motorskills) before 10 yr ofage

Features of 1 of the

other autism spectrumdisorders, butinsufficient for adiagnosis of autismspecifically

Atypical socialinteraction

Usually not cognitivelydelayed

Regression in skillsbetween 6 and 18 moof age

Restricted range ofinterests

Repetitive handmovements

Onset before 3 yearsof age


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Epidemiology

The prevalence rate of all pervasive developmentaldisorders appears to be 58.7 per 10,000 children.

This prevalence rate includes autism (22/10,000), Aspergersyndrome (11/10,000), Pervasive Developmental Disordernot otherwise specified (24.8/10,000), and childdisintegrative disorder (0.9/10,000).


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Alan

As an infant, Alan was playful and affectionate. At 6 months old,he could sit up and crawl. He began to walk and say words at 10

months and could count by 13 months. One day, in his 18thmonth, his mother found him sitting alone in the kitchen,repeatedly spinning the wheels of her vacuum cleaner with suchpersistence and concentration, he didn't respond when shecalled. From that day on, she recalls, "It was as if someone had

pulled a shade over him." He stopped talking and relating toothers. He often tore around the house like a demon. Hebecame fixated on electric lights, running around the houseturning them on and off. When made to stop, he threw atantrum, kicking and biting anyone within reach.


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Autism Spectrum Disorder

neurodevelopmental disorder of unknown etiology,but with a strong genetic basis

typically diagnosed before 36 mo of age

characterized by a behavioral phenotype thatincludes qualitative impairment in the areas oflanguage development or communication skills,social interactions and reciprocity, and imagination

and play

See diagnostic criteria
http://diagnostic%20criteria%20for%20autism.docx/http://diagnostic%20criteria%20for%20autism.docx/


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Autism Spectrum Disorder all children with autism manifest some degree of

impairment in the areas of reciprocal social interaction,communication, and restrictive and repetitive

stereotypical patterns of behavior, interests, or activities

although there is no pathognomonic symptom orbehavior seen, most children have some impairment in

joint attention or pretend play


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Autism Spectrum Disorder Joint Attention

ability to use eye contact and pointing for the purposes of sharing

experiences with others typically develops by 18 mo

other precursor skills to joint attention that are often absent in childrenwith autism are protoimperative pointing (the use of pointing to obtainan object of desire) and protodeclarative pointing (the use of pointingto an object of interest simply to have another share in the interest with

him or her)

the symptoms of autism can vary in the severity of their presentation


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Verbal Abilities

can range from being nonverbal to having advanced speech,capable of imitating songs, rhymes, or television

commercials most notable in children with autism is the quality of their

speech and language

speech may have an odd prosody or intonation and may becharacterized by echolalia, pronoun reversal, nonsense

rhyming, and other idiosyncratic language forms


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Intellectual functioning

vary from mental retardation to superior intellectualfunctioning in select areas

Some children with autism show typical development incertain skills and may even show areas of strength inspecific areas, such as puzzles, art, or music

Play skills in children with autism are typically aberrant,characterized by little symbolic play, ritualistic rigidity, and

preoccupation with parts of objects


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Autism Spectrum Disorder Stereotypical body movements, a marked need for sameness,

and a very narrow range of interests are also common

The child is often withdrawn and spends hours in solitary play

Ritualistic behavior - reflecting the child's need to maintain aconsistent, predictable environment

Disruptions of routineTantrum-like rages

Eye contact is typically minimal or absent

Visual scanning of hand and finger movements, mouthing of

objects, and rubbing of surfaces heightened awareness ofand sensitivity to some stimuli

Diminished responses to pain and lack of startle responses tosudden loud noises lowered sensitivity to other stimuli.


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Autism Spectrum Disorder 6090% concordance rate for monozygotic twins and

a 0% concordance rate for dizygotic twins

92% concordance rate for monozygotic twins and a30% concordance rate for dizygotic twins for thebroader spectrum of social and communicationdifficulties

it is believed that multiple genes interact with varied

environmental causes to produce the disorder, andthat the causative genes may vary from onepopulation to another


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Autism Spectrum Disorder certain genes are believed to be more strongly

implicated in the heritability of autism

chromosome 7q (seen in the similar behavioral phenotype of

specific language impairment disorder)

chromosome 2q

chromosome 15q1113 (seen in Prader-Willi syndrome andAngelman syndrome, both of which manifest traits of rigidityand stereotypical behaviors)


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Autism Spectrum Disorder Autism and Asperger disorder are 4 and 8 times more prevalent

in males than in females, respectively, suggesting a strong X-linked component.

has also been linked with other neurodevelopmental disorders,including seizure disorder, fragile X syndrome, and tuberoussclerosis.

autism is not associated with certain emotionally distantparenting styles (refrigerator mothers).

Many excellent epidemiologic studies have established thatthere is no association between the administration of themeasles-mumps-rubella vaccine and the development of autism


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Autism Spectrum Disorder Retrospective analysis of head circumference in children with

autism, in conjunction with MRI studies, has shown differencesin the brain structure of children with autism compared with

children without autism. The head circumference of children with autism is normal or

slightly smaller than normal at birth until 2 mo of age.

Longitudinal studies of children with autism showed anabnormally rapid increase in head circumference from 614 mo

of age, which was largely concluded by the end of the 2nd yr oflife.


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Autism Spectrum Disorder MRI studies done at 24 yr of age

show that autistic toddlers haveincreased brain volumecharacterized by increasedvolume of the cerebellum,

cerebrum, and amygdalacompared with normal volumes.

The abnormal growth in the first 2yr is most marked in the frontal,temporal, cerebellar, and limbic

regions of the brain, the areas ofthe brain responsible for higher-order cognitive, language,emotional, and social functions,which are most impaired inautism.


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Autism Spectrum Disorder It is believed that the early abnormal growth

processes in the brain in the first 2 yr of life underliethe emergence of preclinical behavioral abnormalities

seen in autism.

This period of early, accelerated brain growthappears to stop early in childhood and is followed byabnormally slow or arrested growth, resulting in areas

of underdeveloped and abnormal circuitry in parts ofthe brain.


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Diagnosis

Aberrant social skill development is the hallmark of autismspectrum disorders (ASDs), and early social skill deficits mayinclude abnormal eye contact, failure to orient to name, failure to

use gestures to point or show, a lack of interactive play, failureto smile, lack of sharing, and lack of interest in other children.

Important early RED FLAGS Combined language and socialdelays and regression in language or social milestones

Early signs include unusual use of language or loss of language

skills, nonfunctional rituals, inability to adapt to new settings,lack of imitation, and absence of imaginary play


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Diagnosis

Several screening tools have been developed to aidin the early detection of children with ASDs. The Checklist for Autism in Toddlers (CHAT) is a screening tool

designed for use with 18 mo old children in primary care settings. The Modified Checklist for Autism in Toddlers (M-CHAT) is a 23-

item parent questionnaire modified from the CHAT. It has showngood sensitivity and specificity (0.87% and 0.99%, respectively),which suggests its utility as a screening tool.

The Pervasive Developmental Disorders Screening Test (PDDST)is a parent-completed survey that targets children from birth3 yr ofage and incorporates a 3-tiered approach: 1 for the primary careclinic, 1 for the developmental clinic, and 1 for the multidisciplinaryautism clinic.


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Diagnosis

In children with ASDs, intelligence, as measured byconventional psychologic testing, usually falls in the functionallyretarded range

Deficits in language and socialization make it difficult to obtainan accurate estimate of the autistic child's intellectual potential.


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Diagnosis

Autistic children also show deficits in their understanding of whatthe other person might be feeling or thinking, a so-called lack ofa theory of mind.

On some psychologic tests, children with autism pay moreattention to specific details while overlooking the entire gestalt ofthe object, demonstrating a lack of central coherence.


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Diagnosis

Do a thorough physicalexamination, with specialattention paid to headcircumference

Twenty-five percent of childrenwith an ASD can havemacrocephaly, but enlargedhead size may not be apparentuntil after the 2nd yr of life

An audiologic evaluation and acomprehensive speech andlanguage evaluation should beundertaken in any child withlanguage delays


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Diagnosis

Chromosomal analysis should be performed if thechild has evidence of mental retardation anddysmorphic features

An electroencephalogram should be performed inchildren with ASDs who have symptoms ofdevelopmental regression or suspicion of seizures.


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Treatment

Behavioral Therapy

Intensive therappy beginning before 3 yr of age

and targeted toward speech and languagedevelopment, is successful in improving bothlanguage capacity and later social functioning

The training method focuses on the acquisition ofcompliance behavior, imitation activities, languageacquisition, and integration with peers.

Treatment is most successful when geared towardthe individual's particular behavior patterns andlanguage function.


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Treatment

Working with the family of an autistic child is vital tothe child's overall care.

Educational programming should begin as early aspossible, preferably by age 24 yr

Older children and adolescents with relatively higherintelligence, but with poor social skills and psychiatricsymptoms (depression, anxiety, obsessive-

compulsive disorder) may require psychotherapy,behavioral or cognitive behavioral therapy, andpharmacotherapy


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Treatment

Pharmacotherapy Open-label trials of atypical neuroleptics (risperidone, olanzapine)

have shown effectiveness in treating target behaviors, and in

some instances, have also improved social relatedness Clomipramine, a tricyclic antidepressant that inhibits serotonin

reuptake, has demonstrated usefulness in reducing compulsionsand stereotypies ( but lowers the seizure threshold, can causeagranulocytosis, and has cardiotoxic and behavior toxicity effects)

The SSRI appear to be somewhat effective in diminishinghyperactive, agitated, and obsessive-compulsive behaviors,although there have not yet been sufficient, controlled studiesregarding their utility


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Prognosis

Some children, especially those with speech, may grow up tolive self-sufficient, employed, albeit isolated, lives in thecommunity.

Because early, intensive therapy may improve language andsocial function, delayed diagnosis may lead to a poor outcome.

There is no increased risk of schizophrenia in adulthood, but thecost of delayed diagnosis across the life span is high.

A better prognosis is associated with higher intelligence,

functional speech, and less bizarre symptoms and behavior. The symptom profile for some children may change as they

grow older and seizures or self-injurious behavior becomesmore common.


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Asperger Disorder

Children with Asperger disorder have aqualitative impairment in the development of

reciprocal social interaction, often showingrepetitive behaviors and restricted,obsessional, idiosyncratic interests.

They have deficits in nonverbal and

pragmatic aspects of communication (facialexpressions, gestures).

They do not have the severe languageimpairments that characterize autism.


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Asperger Disorder

Though somewhat socially aware, these childrenappear to others to be peculiar or eccentric.

They are awkward and clumsy and have unusualpostures and gait.

To meet the diagnostic criteria for Aspergersyndrome, a child must manifest impairments insocial interactions and show restrictive, repetitive

patterns of behavior, interests, or achievements withother people

These disturbances must cause significantimpairments in social or occupational functioning.


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Asperger Disorder

Unlike children with autism, those diagnosed withAsperger syndrome have a history of normallanguage milestones, with single words used by age

2 yr and communicative phrases used by age 3 yr.

There are often similar traits in family members.

Prevalence is estimated to be approximately11/10,000 children.


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Asperger Disorder Group social skill training is the hallmark of

intervention, although children with Aspergerdisorder appear to be at high risk for other

psychiatric disorders, particularlyoppositional-defiant disorder and mooddisorders.

Cognitive-behavioral therapy has been usefulin patients with associated anxiety, whereasrisperidone may improve negative symptomssimilar to those seen in schizophrenia.


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This disorder, also known as Heller dementia, is a rare conditionof unknown cause.

It is characterized by normal development up to 24 yr of age,

followed by severe deterioration of mental and socialfunctioning, with regression to a very impaired autistic statebefore age 10 yr.

Language, social skills, and imagination are profoundly affected;bowel and bladder control may be lost; and motor stereotypies

and seizures are often present. Although this condition may be the result of an underlying

neurologic illness, none has been identified. The prognosis isalways poor.


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a neurodevelopmental disorder resulting from

a genetic mutation of the MECP2gene

an X-linked dominant disorder affectingpredominantly girls and few boys

has a prevalence of 1/10,000

development is initially normal, but thenrapidly regresses in the latter of the 1styear of life.


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Children initially have normal prenatal and perinatal development,

with normal head circumference and normal psychomotordevelopment until 5 mo of age

After this period of normal development, all of the following are

observed: deceleration of head growth at 548 mo, with development of

microcephaly; l

oss of previously acquired purposeful hand skills at 530 mo, withsubsequent development of stereotyped hand movements (hand-

wringing); loss of social engagement;

poorly coordinated gait or trunk movements;

severely impaired receptive and expressive language development, withsevere psychomotor retardation.


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Childhood Schizophrenia

Psychotic reactions in older children tend tomore closely resemble the psychoses of

adulthood, and the same diagnostic criteriaapply
http://diagnostic%20criteria%20for%20childhood%20schizophrenia.docx/http://diagnostic%20criteria%20for%20childhood%20schizophrenia.docx/


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Clinical Manifestations

prominent symptoms include thought disorder,disorganized speech, delusions, and hallucinations

delusions, and hallucinations, in addition to later

onset, higher intelligence scores, and fewer perinatalcomplications, differentiate schizophrenia fromautism

may have paranoid delusions, aggressive behavior,

hebephrenic silliness, social withdrawal, andalternating moods not apparently related toenvironmental stimuli.


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The typical age of onset is late adolescence toearly adulthood.

Early-onset schizophrenia (before puberty or inearly adolescence) is rare.

Schizophrenic children show significant

premorbid maladjustments, including socialwithdrawal, disruptive behaviors, developmentaldelays, and speech and language problems.


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Auditory hallucinations are seen in 80% ofschizophrenic children.

Delusions and formal thought disordersusually do not present until midadolescence.

Children with early-onset schizophrenia showpreliminary evidence of progressive

ventricular enlargement, a decrease in totalcerebral volume, and a decline in intellectualfunctioning. (Poor prognosis)


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Affective blunting and disturbed interpersonalrelationships predict psychotic adult

psychopathology In schizophrenic children, initial presenting

symptoms cluster around violent aggression andschool problems.

Psychotic symptoms are 1st recognized at 211yr, followed 2 yr later by a diagnosis ofschizophrenia.


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Before diagnosis, children with schizophrenia often havea prodromal period characterized by deficits in attention,impaired language and verbal memory, poor gross motor

skills, and impaired coordination. Most children receive a psychiatric diagnosis before the

development of psychosis, with the most commondiagnoses being pervasive developmental disorder,

attention-deficit/hyperactivity disorder, and depression.


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In the premorbid period before the diagnosis ofschizophrenia, affected children showed higher rates ofsocial withdrawal and greater global impairment, and had

fewer friends. Individuals with various psychotic processes are often

misdiagnosed as having schizophrenia.


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Treatment

Multimodal therapeutic approach

Parent training is necessary

Individual therapy designed to build a positive alliance

Neuroleptic therapy hallucinations and psychoticdelusions, these medications appear to have lower risksof extrapyramidal symptoms and tardive dyskinesia

Clozapine appears to be the most effectiveantipsychotic medication for refractory cases, but therisk of agranulocytosis and seizures limits its use


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Acute Phobic Hallucinations Hallucinations often signify a serious disorder that

requires immediate attention, especially with :

Altered levels of consciousness Delusions

Confusion

Any abnormalities on mental status examination

Acute phobic hallucinations are benign andcommon and occur in previously healthypreschool children


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Acute Phobic Hallucinations Hallucinations are often visual or tactile, last 1060 min,

and occur at any time, but most often at night

The differential diagnosis includes drug overdose or

poisoning, high fever, encephalitis, and psychosis

Symptoms may persist for 13 days, slowly abating over12 wk. Treatment with benzodiazepines may bebeneficial.

pervasive developmental disorders and childhood psychosis

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