Markedly restricted repertoire of activities and interests.
This restriction may take various forms. In the younger child there may be
resistance or even catastrophic reactions to minor changes in the environment,
e.g., the child may scream when his or her place at the dinner table is changed.
There is often attachment to objects such as a string or rubber band. Motor
stereotypes include hand-clapping, peculiar hand movements, rocking, and
dipping and swaying movements of the whole body. In the older child there may
be an insistence on following routines in a precise way, e.g., taking the same
route to a favorite restaurant. There may be fascination with movement, such as
passively staring at an electric fan or other rapidly revolving object. The child
himself may be skillful at making all kinds of objects spin, so that he can watch
them, or may spin himself around. The child may be exclusively interested in
buttons, parts of the body, or playing with water.
Verbal stereotypes include repetition of words or phrases regardless of
meaning. In older children, tasks involving long-term memory, for example, recall
of the exact words of songs heard years before, train timetables, historical dates,
or chemical formulae, may be excellent, but the information tends to be repeated
over and over again, regardless of the social context and the appropriateness of
the information.
Associated features. In general, the younger the child and the more severe
the handicaps, the more associated features are likely to be present. They may
include the following;
1. Abnormalities in the development of cognitive skills. The profile of specific
skills is usually uneven, regardless of general level of intelligence. In most cases
there is an associated diagnosis of Mental Retardation, most commonly in the
moderate range
2. Abnormalities of posture and motor behavior, such as stereotypes
(arm-flapping, jumping, grimacing) in response to excitement, walking on tiptoe,
odd hand and body postures, and poor motor coordination.
3. Odd responses to sensory input, such as ignoring some sensations (e.g.,
pain, heat, cold), displaying oversensitivity to certain sensations (e.g., covering
ears to shut out some sounds; dislike of being touched), and being fascinated by
some sensations (e.g., exaggerated reaction to lights or odors).
4. Abnormalities in eating, drinking, or sleeping (e.g., limiting diet to few
foods, excessive drinking of fluids, recurrent awakening at night with
rocking).
5. Abnormalities of mood (e.g., labile mood, giggling or weeping for no
apparent reason, apparent absence of emotional reactions, lack of fear of real
dangers, excessive fearfulness in response to harmless objects or events,
generalized anxiety and tension).
6. Self-injurious behavior, such as head-banging, or finger-, hand-, or
wrist-biting.
Other mental disorders, such as Major Depression, may occur during
adolescence and adult life. They are most easily recognized in people who have
stifficient speech to describe symptoms accurately.
Age at onset. Onset is reported by parents to be before age three in the
great majority of cases. Very few cases are reported with an onset after five or
six. However, it may be difficult to establish age at onset retrospectively unless
those who cared for the child during the early years are able to give accurate
information about language development, sociability, and play. Manifestations in
infancy are more subtle and hard to define than those seen after two years of
age. Parents of only children may be unaware of the problems until the child is
observed with other children (for example, on entering school), and may then
date the age at onset from that point, although a careful history often reveals that
the abnormalities were present earlier. Parents may also date onset from a
particular event, such as the birth of a sibling, or from the time when the child
experienced a severe illness or accident or emotional trauma. In such cases, it is
difficult to know whether subtle signs of the disorder may not have been present
before the event.
In extremely rare cases, there is a period of apparently normal development
followed by rapid disintegration of social and cognitive skills and development of
the characteristic features of a Pervasive Developmental Disorder. Such cases
have been termed Heller's syndrome or disintegrative psychosis, but according to
this manual, should be classified as either Autistic Disorder or Pervasive
Developmental Disorder Not Otherwise Specified.
Course. Manifestations of the disorder are, in almost all cases, lifelong,
although they vary with chronological age and severity of the handicaps. Some
children experience an improvement in social, language, and other skills at about
age five to six years; in a few cases, this may be very marked.
Puberty can bring changes in either direction. Cognitive functions and social
skills may decline or improve independently of each other. There is often an
exacerbation of aggressive, oppositional, or other troublesome behavior, which
may last for many years. A small minority of the children eventually are able to
lead independent lives, with only minimal signs of the essential features of the
disorder; but the social awkwardness and ineptness may persist. Most remain
handicapped, with marked signs of the disorder. Factors related to long-term
prognosis include IQ and the development of social and language skills.
Degree of impairment. The degree of impairment varies. In the majority of
cases, a structured environment is necessary throughout life. In very rare cases,
the person may complete college or even graduate education.
Complications. The major complication is the development of epileptic
seizures. Most of those who develop seizures have an IQ below 50. In about
25% or more of cases of Autistic Disorder, there has been one or more episodes
of seizure by the time the person reaches adulthood; in a sizable minority, onset
of seizures is in adolescence.
In adolescence or early adult life, depression in response to partial
realization of handicaps is common in those of higher levels of ability Catatonic
phenomena, particularly excitement or posturing, or an undifferentiated psychotic
state with apparent delusions and hallucinations can occur in response to stress,
but often clear rapidly if the stress is removed.
Prevalence. Studies in England and the United States, using criteria similar to
those in this manual, suggest that the prevalence of Autistic Disorder is
approximately 4 to 5 children in every 10,000. Autistic Disorder was previously
thought to be more common in upper socioeconomic classes, but studies
suggest that this finding was a function of referral bias. The prevalence of
Pervasive Developmental Disorder (Autistic Disorder and Pervasive
Developmental Disorder Not Otherwise Specified) has been estimated at 10 to
15 children in every 10,000.
Sex ratio. Pervasive Developmental Disorder is more common among males
than females, studies showing ratios ranging from 2:1 to 5:1 * Most studies of
Autistic Disorder show a ratio of 3:1 or 4:1.
Predisposing Factors. A very wide range of pre-, peri- and postnatal
conditions causing brain dysfunction are thought to predispose to the
development of Pervasive Developmental Disorders. Autistic Disorder has been
reported in association with maternal rubella, untreated phenylketonuria,
tuberous sclerosis, anoxia during birth, encephalitis, infantile spasms, and fragile
X syndrome. In the past, certain abnormalities of parental personality and
child-rearing practices were thought to predispose to the development of Autistic
Disorder, but controlled studies have not confirmed this view.
Familial pattern. Autistic Disorder is apparently more common in the siblings
of children with the disorder than in the general population.
Differential diagnosis. Mental Retardation and Pervasive Developmental
Disorder often coexist, but it should be understood that most people with even
severe Mental Retardation do not have the essential features of Pervasive
Developmental Disorder in that they are sociable and can communicate-even
nonverbally if they have no speech. Differential diagnosis in people with severe
or profound Mental Retardation may be difficult. When interest and pleasure in
social approaches are evident, through eye contact, facial expression, bodily
movements, and vocalizations, the diagnosis of Pervasive Developmental
Disorder should not be made.
The diagnosis of Schizophrenia is extremely rare in childhood, whereas
Pervasive Developmental Disorder is almost always first diagnosed in infancy or
childhood. As adults, people with Pervasive Developmental Disorder may have
many of the "negative symptoms" of the residual phase of Schizophrenia, such
as social isolation and withdrawal, markedly peculiar behavior, blunted or
inappropriate affect and oddities of language. The stereotyped, repetitive acting
of a particular role-of an object, animal, or individual by a person with Pervasive
Developmental Disorder may be mistaken for a delusion. If the criteria for Autistic
Disorder are met, the additional diagnosis of Schizophrenia should be made only
in the rare instances in which prominent delusions or hallucinations meeting the
criteria for Schizophrenia can be documented. Schizophrenia, however,
preempts a diagnosis of Pervasive Developmental Disorder Not Otherwise
Specified.
Hearing impairments and Specific Developmental Language and Speech
Disorders affect the development of understanding and/or use of speech. Some
visual impairments result in poor eye contact, and can be associated with staring
at repetitive hand movements. Disorders involving only sensory and perceptual
impairment can be differentiated from Pervasive Developmental Disorder by the
presence of social interaction and a desire for communication appropriate for the
person's mental age.
In Schizoid and Schizotypal Personality Disorders there are deficits in
interpersonal relatedness. The diagnosis of Autistic Disorder preempts the
diagnosis of these personality disorders. However, these personality disorders
preempt the diagnosis of Pervasive Developmental Disorder Not Otherwise
Specified.
In Tic Disorders and Stereotypy/Habit Disorder there are stereotyped body
movements, but there is no qualitative impairment in reciprocal social
interaction.
Page 3
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This restriction may take various forms. In the younger child there may be resistance or even catastrophic reactions to minor changes in the environment, e.g., the child may scream when his or her place at the dinner table is changed. There is often attachment to objects such as a string or rubber band. Motor stereotypes include hand-clapping, peculiar hand movements, rocking, and dipping and swaying movements of the whole body. In the older child there may be an insistence on following routines in a precise way, e.g., taking the same route to a favorite restaurant. There may be fascination with movement, such as passively staring at an electric fan or other rapidly revolving object. The child himself may be skillful at making all kinds of objects spin, so that he can watch them, or may spin himself around. The child may be exclusively interested in buttons, parts of the body, or playing with water.
Verbal stereotypes include repetition of words or phrases regardless of meaning. In older children, tasks involving long-term memory, for example, recall of the exact words of songs heard years before, train timetables, historical dates, or chemical formulae, may be excellent, but the information tends to be repeated over and over again, regardless of the social context and the appropriateness of the information.
Associated features. In general, the younger the child and the more severe the handicaps, the more associated features are likely to be present. They may include the following;
1. Abnormalities in the development of cognitive skills. The profile of specific skills is usually uneven, regardless of general level of intelligence. In most cases there is an associated diagnosis of Mental Retardation, most commonly in the moderate range
2. Abnormalities of posture and motor behavior, such as stereotypes (arm-flapping, jumping, grimacing) in response to excitement, walking on tiptoe, odd hand and body postures, and poor motor coordination.
3. Odd responses to sensory input, such as ignoring some sensations (e.g., pain, heat, cold), displaying oversensitivity to certain sensations (e.g., covering ears to shut out some sounds; dislike of being touched), and being fascinated by some sensations (e.g., exaggerated reaction to lights or odors).
4. Abnormalities in eating, drinking, or sleeping (e.g., limiting diet to few foods, excessive drinking of fluids, recurrent awakening at night with rocking).
5. Abnormalities of mood (e.g., labile mood, giggling or weeping for no apparent reason, apparent absence of emotional reactions, lack of fear of real dangers, excessive fearfulness in response to harmless objects or events, generalized anxiety and tension).
6. Self-injurious behavior, such as head-banging, or finger-, hand-, or wrist-biting.
Other mental disorders, such as Major Depression, may occur during adolescence and adult life. They are most easily recognized in people who have stifficient speech to describe symptoms accurately.
Age at onset. Onset is reported by parents to be before age three in the great majority of cases. Very few cases are reported with an onset after five or six. However, it may be difficult to establish age at onset retrospectively unless those who cared for the child during the early years are able to give accurate information about language development, sociability, and play. Manifestations in infancy are more subtle and hard to define than those seen after two years of age. Parents of only children may be unaware of the problems until the child is observed with other children (for example, on entering school), and may then date the age at onset from that point, although a careful history often reveals that the abnormalities were present earlier. Parents may also date onset from a particular event, such as the birth of a sibling, or from the time when the child experienced a severe illness or accident or emotional trauma. In such cases, it is difficult to know whether subtle signs of the disorder may not have been present before the event.
In extremely rare cases, there is a period of apparently normal development followed by rapid disintegration of social and cognitive skills and development of the characteristic features of a Pervasive Developmental Disorder. Such cases have been termed Heller's syndrome or disintegrative psychosis, but according to this manual, should be classified as either Autistic Disorder or Pervasive Developmental Disorder Not Otherwise Specified.
Course. Manifestations of the disorder are, in almost all cases, lifelong, although they vary with chronological age and severity of the handicaps. Some children experience an improvement in social, language, and other skills at about age five to six years; in a few cases, this may be very marked.
Puberty can bring changes in either direction. Cognitive functions and social skills may decline or improve independently of each other. There is often an exacerbation of aggressive, oppositional, or other troublesome behavior, which may last for many years. A small minority of the children eventually are able to lead independent lives, with only minimal signs of the essential features of the disorder; but the social awkwardness and ineptness may persist. Most remain handicapped, with marked signs of the disorder. Factors related to long-term prognosis include IQ and the development of social and language skills.
Degree of impairment. The degree of impairment varies. In the majority of cases, a structured environment is necessary throughout life. In very rare cases, the person may complete college or even graduate education.
Complications. The major complication is the development of epileptic seizures. Most of those who develop seizures have an IQ below 50. In about 25% or more of cases of Autistic Disorder, there has been one or more episodes of seizure by the time the person reaches adulthood; in a sizable minority, onset of seizures is in adolescence.
In adolescence or early adult life, depression in response to partial realization of handicaps is common in those of higher levels of ability Catatonic phenomena, particularly excitement or posturing, or an undifferentiated psychotic state with apparent delusions and hallucinations can occur in response to stress, but often clear rapidly if the stress is removed.
Prevalence. Studies in England and the United States, using criteria similar to those in this manual, suggest that the prevalence of Autistic Disorder is approximately 4 to 5 children in every 10,000. Autistic Disorder was previously thought to be more common in upper socioeconomic classes, but studies suggest that this finding was a function of referral bias. The prevalence of Pervasive Developmental Disorder (Autistic Disorder and Pervasive Developmental Disorder Not Otherwise Specified) has been estimated at 10 to 15 children in every 10,000.
Sex ratio. Pervasive Developmental Disorder is more common among males than females, studies showing ratios ranging from 2:1 to 5:1 * Most studies of Autistic Disorder show a ratio of 3:1 or 4:1.
Predisposing Factors. A very wide range of pre-, peri- and postnatal conditions causing brain dysfunction are thought to predispose to the development of Pervasive Developmental Disorders. Autistic Disorder has been reported in association with maternal rubella, untreated phenylketonuria, tuberous sclerosis, anoxia during birth, encephalitis, infantile spasms, and fragile X syndrome. In the past, certain abnormalities of parental personality and child-rearing practices were thought to predispose to the development of Autistic Disorder, but controlled studies have not confirmed this view.
Familial pattern. Autistic Disorder is apparently more common in the siblings of children with the disorder than in the general population.
Differential diagnosis. Mental Retardation and Pervasive Developmental Disorder often coexist, but it should be understood that most people with even severe Mental Retardation do not have the essential features of Pervasive Developmental Disorder in that they are sociable and can communicate-even nonverbally if they have no speech. Differential diagnosis in people with severe or profound Mental Retardation may be difficult. When interest and pleasure in social approaches are evident, through eye contact, facial expression, bodily movements, and vocalizations, the diagnosis of Pervasive Developmental Disorder should not be made.
The diagnosis of Schizophrenia is extremely rare in childhood, whereas Pervasive Developmental Disorder is almost always first diagnosed in infancy or childhood. As adults, people with Pervasive Developmental Disorder may have many of the "negative symptoms" of the residual phase of Schizophrenia, such as social isolation and withdrawal, markedly peculiar behavior, blunted or inappropriate affect and oddities of language. The stereotyped, repetitive acting of a particular role-of an object, animal, or individual by a person with Pervasive Developmental Disorder may be mistaken for a delusion. If the criteria for Autistic Disorder are met, the additional diagnosis of Schizophrenia should be made only in the rare instances in which prominent delusions or hallucinations meeting the criteria for Schizophrenia can be documented. Schizophrenia, however, preempts a diagnosis of Pervasive Developmental Disorder Not Otherwise Specified.
Hearing impairments and Specific Developmental Language and Speech Disorders affect the development of understanding and/or use of speech. Some visual impairments result in poor eye contact, and can be associated with staring at repetitive hand movements. Disorders involving only sensory and perceptual impairment can be differentiated from Pervasive Developmental Disorder by the presence of social interaction and a desire for communication appropriate for the person's mental age.
In Schizoid and Schizotypal Personality Disorders there are deficits in interpersonal relatedness. The diagnosis of Autistic Disorder preempts the diagnosis of these personality disorders. However, these personality disorders preempt the diagnosis of Pervasive Developmental Disorder Not Otherwise Specified.
In Tic Disorders and Stereotypy/Habit Disorder there are stereotyped body movements, but there is no qualitative impairment in reciprocal social interaction.