DSM-IV
Below is the section of DSM-IV that deals with Pervasive Developmental Disorders.
In each section, it will refer to Criterion A, etc, to view the diagnostic criteria
that they refer to, click on the each subtitle (i.e. 299.0 Autistic Disorder).
Pervasive Developmental Disorders
Pervasive Developmental Disorders are characterized by severe and pervasive impairment
in several areas of development: reciprocal social interaction skills, communication
skills, or the presence of stereotyped behavior, interests, and activities. The
qualitative impairments that define these conditions are distinctly deviant relative to
the individual's developmental level or mental age. This section contains Autistic
disorder, Rett's Disorder, Childhood Disintegrative Disorder, Asperger's Disorder, and
Pervasive Developmental Disorder Not Otherwise Specified. These disorders are usually
evident in the first years of life and are often associated with some degree of Mental
Retardation, which if present, should be coded on Axis II. The Pervasive Developmental
Disorders are sometimes observed with a diverse group of other general medical conditions
(e.g., chromosomal abnormalities, congenital infections, structural abnormalities of the
central nervous system). If such conditions are present, they should be noted on Axis III.
Although terms like "psychosis" and "childhood schizophrenia" were
once used to refer to individuals with these conditions, there is considerable evidence to
suggest that the Pervasive Developmental Disorders are distinct from Schizophrenia
(however, an individual with Pervasive Developmental Disorder may occasionally later
develop Schizophrenia).
299.0 Autistic Disorder
Diagnostic Features
The essential features of Autistic Disorder are the presence of markedly abnormal or
impaired development in social interaction and communication and a markedly restricted
repertoire of activity and interests. Manifestations of the disorder vary greatly
depending on the developmental level and chronological age of the individual. Autistic
Disorder is sometimes referred to as early infantile autism, childhood autism, or
Kanner's autism.
The impairment is reciprocal social interaction is gross and sustained. There may be
marked impairment in the use of multiple nonverbal behaviors (e.g., eye-to-eye gaze,
facial expression, body postures and gestures) to regulate social interaction and
communication (Criterion A1a). There may be failure to develop peer relationships
appropriate to developmental level (Criterion A1b) that may take different forms at
different ages. Younger individuals may have little or no interest in establishing
friendships. Older individuals hay have an interest in friendship but lack understanding
of the conventions of social interaction. There may be a lack of spontaneous seeking to
share enjoyment, interests, or achievements with other people (e.g., not showing,
bringing, or pointing out objects they find interesting) (Criterion A1c). Lack of social
or emotional reciprocity may be present (e.g., not actively participating in simple social
or emotional reciprocity may be present (e.g., not actively participating in simple social
play or games, preferring solitary activities, or involving others in activities only as
tools or "mechanical" aids) (Criterion A1d). Often an individual's awareness of
others is markedly impaired. Individuals with this disorder may be oblivious to other
children (including siblings), may have no concept of the needs of others, or may not
notice another person's distress.
The impairment in communication is also marked and sustained and affects both verbal
and nonverbal skills. There may be delay in, or total lack of, the development of spoken
language (Criterion A2a). In individuals who do speak, there may be marked impairment in
the ability to initiate or sustain a conversation with others (Criterion A2b), or a
stereotyped and repetitive use of language or idiosyncratic language (Criterion A2c).
There may also be a lack of varied, spontaneous make-believe play or social imitative play
appropriate to developmental level (Criterion A2d). When speech does develop, the pitch,
intonation, rate, rhythm, or stress may be abnormal (e.g., tone of voice may be
monotonous, or contain question like rises at ends of statements). Grammatical structures
are often immature and include stereotyped and repetitive use of language (e.g.,
repetition of words or phrases regardless of meaning; repeating jingles or commercials) or
metaphorical language (i.e., language that can only be understood clearly by those
familiar with the individual's communication style). A disturbance in the comprehension of
language may be evidenced by an inability to understand simple questions, directions, or
jokes. Imaginative play is often absent or markedly impaired. These individuals also tend
not to engage in the simple imitations games or routines of infancy or early childhood or
do so only out of context or in a mechanical way.
Individuals with Autistic Disorder have restricted, repetitive, and stereotyped
patterns of behavior, interests, and activities. There may be an encompassing
preoccupation with one or more stereotyped and restricted patterns of interest that is
abnormal either in intensity or focus (Criterion A3a); an apparently inflexible adherence
to specific, nonfunctional routines or rituals (Criterion A3b); stereotyped and repetitive
motor mannerisms (Criterion A3c); or a persistent preoccupation with parts of objects
(Criterion A3d). Individuals with Autistic Disorder display a markedly restricted range of
interests and are often preoccupied with one narrow interest (e.g., with amassing facts
about meteorology or baseball statistics). They may line up an exact number of play things
in the same manner over and over again or repetitively mimic the actions of a television
actor. They may insist on sameness and show resistance to or distress over trivial changes
(e.g., a younger child may have a catastrophic reactions to a minor change in the
environment such as a new set of curtains or a change in place at the dinner table). There
is often an interest in nonfunctional routines or rituals or an unreasonable insistence on
following routines (e.g., taking exactly the same route to school every day). Stereotyped
body movements include the hands (clapping, finger flicking) or whole body (rocking,
dipping, and swaying). Abnormalities of posture (e.g., walking on tiptoe, odd hand
movements and body postures) may be present. These individuals show a persistent
preoccupation with parts of objects (buttons, parts of the body). There may also be a
fascination with movement (e.g., the spinning wheels of toys, the opening and closing of
doors, and electric fan or other rapidly revolving object). The person may be highly
attached to some inanimate object (e.g., a piece of string or rubber band).
The disturbance just be manifest by delays or abnormal functioning in at least one of
the following areas prior to age 3 years; social interaction, language as used in social
communication, or symbolic or imaginative play (Criterion B). There is typically no period
of unequivocally normal development, although 1 or 2 years of relatively normal
development has been reported in some instances. In a minority of cases, parents report
regression in language development, generally manifest as the cessation of speech after a
child has acquired from 5 to 10 words. By definition, if there is a period of normal
development, it cannot extend past age 3 years. The disturbance must not be better
accounted for by Rett's Disorder or Childhood Disintegrative Disorder (Criterion C).
Associated Features and Disorders
Associated descriptive features and mental disorders. In most cases, there is an
associated diagnosis of Mental Retardation, commonly in the moderate range (IQ 35-50).
Approximately 75% of children with Autistic Disorder function at a retarded level. There
may be abnormalities in the development of cognitive skills. The profile of cognitive
skills is usually uneven, regardless of the general level of intelligence (e.g., a 4
1/2-year-old girl with Autistic Disorder may be able to read, i.e., hyperlexia). In many
higher-functioning children with Autistic Disorder, the level of receptive language (i.e.,
language comprehension) is below that of expressive language (e.g., vocabulary).
Individuals with Autistic Disorder may have a range of behavioral symptoms, including
hyperactivity, short attention span, impulsivity, aggressiveness, self-injurious
behaviors, and, particularly in young children, temper tantrums. There may be odd
responses to sensory stimuli (e.g., a high threshold for pain, oversensitivity to sounds
or being touched, exaggerated reactions to light or odors, fascination with certain
stimuli). There may be abnormalities in eating (e.g., limiting diet to a few foods, Pica)
or sleeping (e.g., recurrent awakening at night with rocking). Abnormalities of mood or
affect (e.g., giggling or weeping for no apparent reason, an apparent absence of emotional
reaction) may be present. There may be a lack of fear in response to real dangers, and
excessive fearfulness in response to harmless objects. A variety of self-injurious
behaviors may be present (e.g., head banging or find, hand, or wrist biting). In
adolescence or early adult life, individuals with Autistic Disorder who have the
intellectual capacity for insight may become depressed in response to the realization of
their serious impairment.
Associated laboratory findings. When Autistic Disorder is associated with a general
medical condition, laboratory findings consistent with the general medical condition will
be observed. There have been reports of group differences in measures of serotonergic
activity, but these are not diagnostic for Autistic Disorder. Imaging studies may be
abnormal in some cases, but no specific pattern has been clearly identified. EEG
abnormalities are common even in the absence of seizure disorders.
Associated physical examination findings and general medical conditions. Various
nonspecific neurological symptoms or signs may be noted (e.g., primitive reflexes, delayed
development of hand dominance) in Autistic Disorder. The condition is sometimes observed
in association with a neurological or other general medical condition (e.g., encephalitis,
phenylketonuria, tuberous sclerosis, fragile X syndrome, anoxia during birth, maternal
rubella). Seizures may develop (particularly in adolescence) in as many as 25% of cases.
When other general medical conditions are present, they should be noted on Axis III.
Specific Age and Gender Features
The nature of the impairment is social interaction may change over time in Autistic
Disorder and may vary depending on the developmental level of the individual. In infants,
there may be a failure to cuddle; an indifference or aversion to affection or physical
contact; a lack of eye contact, facial responsiveness, or socially directed smiles; and a
failure to respond to their parents' voices. As a result, parents may be concerned
initially that the child is deaf. Young children with this disorder may treat adults as
interchangeable or may cling mechanically to a specific person. Over the course of
development, the child may become more willing to be passively engaged in social
interaction and may even become more interested in social interaction. However, even in
such instances, the child tends to treat other people in unusual ways (e.g., expecting
other people to answer ritualized question in specific ways, having little sense of other
people's boundaries, and being inappropriately intrusive in social interaction). In older
individuals, tasks involving long-term memory (e.g., train timetables, historical dates,
chemical formulas, or recall of the exact words of songs heard years before) may be
excellent, but the information tends to be repeated over and over again, regardless of the
appropriateness of the information of the social context. Rates of the disorder are four
to five times higher in males than in females. Females with the disorder are more likely,
however, to exhibit more severe Mental Retardation.
Prevalence
Epidemiological studies suggest rates of Autistic Disorder of 2-5 cases per 10,000
individuals.
Course
By definition, the onset of Autistic Disorder is prior to age 3 years. In some
instances, parents will report that they have been worried about the child since birth or
shortly afterward because of the child's lack of interest in social interaction.
Manifestations of the disorder in infancy are more subtle and difficult to define than
those seen after age 2 years. In a minority of cases, the child may be reported to have
developed normally for the first year (or even 2 years) of life. Autistic Disorder follows
a continuous course. In school-age children and adolescents, developmental gains in some
areas are common (e.g., increased interest in social functioning as the child reaches
school age). Some individuals deteriorate behaviorally during adolescence, whereas others
improve. Language skills (e.g., presence of communicative speech) and overall intellectual
level are the strongest factors related to ultimate prognosis. Available follow-up studies
suggest that only a small percentage of individuals with the disorder go on as adults to
live and work independently. In about one-third of cases, some degree of partial
independence is possible. The highest functioning adults with Autistic Disorder typically
continue to exhibit problems in social interaction and communication along with markedly
restricted interests and activities.
Familial Pattern
There is an increased risk of Autistic Disorder among siblings of individuals with the
disorder.
Differential Diagnosis
Periods of developmental regression may be observed in normal development, but these
are neither as severe or as prolonged as in Autistic Disorder. Autistic Disorder must be
differentiated from other Pervasive Developmental Disorders. Rett's Disorder
differs from Autistic Disorder in its characteristic sex ratio and pattern of deficits.
Rett's Disorder has been diagnoses only in females, whereas Autistic Disorder occurs much
more frequently in males. In Rett's Disorder, there is a characteristic pattern of head
growth deceleration, loss of previously acquired purposeful hand skills, and the growth
deceleration, loss of previously acquired purposeful hand skills, and the appearance of
poorly coordinated gait or trunk movements. Particularly during the preschool years,
individuals with Rett's Disorder may exhibit difficulties in social interaction similar to
those observed in Autistic disorder, but these tend to be transient. Autistic disorder
differs from Childhood Disintegrative Disorder, which has a distinctive pattern of
developmental regression following at least 2 years of development. In Autistic Disorder,
developmental abnormalities are usually noted within the first year of life. When
information on early development is unavailable or when it is not possible to document the
required period of normal development, the diagnosis of Autistic Disorder should be made. Asperger's
Disorder can be distinguished from Autistic Disorder by the lack of delay in language
development. Asperger's Disorder is not diagnosed if criteria are met for Autistic
Disorder.
Schizophrenia with childhood onset usually develops after years of normal, or near
normal, development. An additional diagnosis of Schizophrenia can be made if an individual
with Autistic Disorder develops the characteristic features of Schizophrenia with
active-phase symptoms prominent delusions or hallucinations that last for at least 1
month. In Selective Mutism, the child usually exhibits appropriate communication
skills in certain contexts and does not have the severe impairment in social interaction
and the restricted patterns of behavior associated with Autistic Disorder. In Expressive
Language Disorder and Mixed Receptive-Expressive Language Disorder, there is a
language impairment, but it is not associated with the presence of a qualitative
impairment in social interaction and restricted, repetitive, and stereotyped patterns of
behavior. It is sometimes difficult to determine whether an additional diagnosis of
Autistic Disorder is warranted in an individual with Mental Retardation, especially
if the Mental Retardation is Severe or Profound. An additional diagnosis of Autistic
Disorder is reserved for those situations in which there are qualitative deficits in
social and communicative skills and the specific behaviors characteristic of Autistic
Disorder are present. Motor stereotypies are characteristic of Autistic Disorder; an
additional diagnosis of Stereotypic Movement Disorder is not given when these are
better accounted for as part of the presentation of Autistic Disorder.
299.80 Rett's Disorder
Diagnostic Features
The essential feature of Rett's Disorder is the development of multiple specific
deficits following a period of normal functioning after birth. Individuals have an
apparently normal prenatal and perinatal period (Criterion A1) with normal psychomotor
development through the first 5 months of life (Criterion A2). Head circumference at birth
is also within normal limits (Criterion A3). Between ages 5 and 48 months, head growth
decelerates (Criterion B1). There is a loss of previously acquired purposeful hand skills
between ages 5 and 30 months, with the subsequent development of characteristic
stereotyped hand movements resembling hand-wringing or hand washing (Criterion B2).
Interest in the social environment diminishes in the first few years after the onset of
this disorder (Criterion B3), although social interaction may often develop later in the
course. Problems develop in the coordination of gait or trunk movements (Criterion B4).
There is also severe impairment in expressive and receptive language development, with
severe psychomotor retardation (Criterion B5).
Associated Features and Disorders
Rett's Disorder is typically associated with Severe or Profound Mental Retardation,
which, if present, should be coded on Axis II. There are no specific laboratory findings
associated with the disorder. There may be an increased frequency of EEG abmornalities and
seizure disorder in individuals with Rett's Disorder. Nonspecific abnormalities on brain
imaging have been reported.
Prevalence
Data are limited to mostly case series, and it appears that Rett's Disorder is much
less common than Autistic Disorder. This disorder has been reported only in females.
Course
The pattern of developmental regression is highly distinctive. Rett's Disorder has its
onset prior to age 4 years, usually in the first or second year of life. The duration of
the disorder is lifelong, and the loss of skills is generally persistent and progressive.
In most instances, recovery is quite limited, although some very modest developmental
gains may be made and interest in social interaction may be observed as individuals enter
later childhood or adolescence. The communicative and behavioral difficulties usually
remain relatively constant throughout life.
Differential Disorders
Periods of developmental regression may be observed in normal development, but these
are neither as severe or as prolonged as in Rett's Disorder. For the differential between
Rett's Disorder and Autistic Disorder, [see Autistic Disorder, Differential
Diagnosis]. Rett's Disorder differs from Childhood Disintegrative Disorder and Asperger's
Disorder in its characteristic sex ratio, onset, and pattern of deficits. Rett's
Disorder has been diagnosed only in females, whereas Childhood Disintegrative Disorder and
Asperger's Disorder appear to be more common in males. The onset of symptoms in Rett's
Disorder can begin as early as age 5 months, where in Childhood Disintegrative Disorder
the period of normal development is typically more prolonged (i.e., at least until age 2
years). In Rett's Disorder, there is a characteristic pattern of head growth deceleration,
loss of previously acquired purposeful hand skills, and the appearance of poorly
coordinated gait or trunk movements. In contrast to Asperger's Disorder, Rett's Disorder
is characterized by a severe impairment in expressive and receptive language development.
299.10 Childhood Disintegrative
Disorder
Diagnostic Features
The essential feature of Childhood Disintegrative Disorder is a marked regression in
multiple areas of functioning following a period of at least 2 years of apparently normal
development (Criterion A). Apparently normal development is reflected in age-appropriate
verbal and nonverbal communication, social relationships, play, and adaptive behavior.
After the first 2 years of life (but before age 10 years), the child has a clinically
significant loss of previously acquired skills in at least two of the following areas:
expressive or receptive language, social skills or adaptive behavior, bowel or bladder
control, play, or motor skills (Criterion B). Individuals with this disorder exhibit the
social and communicative deficits and behavioral features generally observed in Autistic
Disorder ... There is qualitative impairment in social interaction (Criterion C1) and in
communication (Criterion C2), and restricted, repetitive, and stereotyped patterns of
behavior, interests, and activities (Criterion C3). The disturbance is not better
accounted for by another specific Pervasive Developmental Disorder or by Schizophrenia
(Criterion D). This condition has also be termed Heller's syndrome, dementia
infantilis, or disintegrative psychosis.
Associated Features and Disorders
Childhood Disintegrative Disorder is usually associated with Severe Mental Retardation,
which, if present, should be coded on Axis II. Various nonspecific neurological symptoms
or signs may be noted. There seems to be an increased frequency of EEG abnormalities and
seizure disorder. Although it appears likely that the condition is the result of some
insult to the developing central nervous system, no precise mechanism has been identified.
The condition is occasionally observed in association with a general medical condition
(e.g., metachromatic leukodystrophy, Schilder's disease) that might account for the
developmental regression. In most instances, however, extensive investigation does not
reveal such a condition. If a neurological or other general medical condition is
associated with the disorder, it should be recorded on Axis III. The laboratory findings
will reflect any associated general medical conditions.
Prevalence
Epidemiological data are limited, but Childhood Disintegrative Disorder appears to be
very rare and much less common than Autistic Disorder. Although initial studies suggested
an equal sex ration, the most recent data suggest that the condition is more common among
males.
Course
By definition, Childhood Disintegrative Disorder can only be diagnosed if the symptoms
are preceded by at least 2 years of normal development and the onset is prior to age 10
years. When the period of normal development has been quite prolonged (5 or more years),
it is particularly important to conduct a thorough physical and neurological examination
to assess for the presence of a general medical condition. In most cases, the onset is
between ages 3 and 4 years and may be insidious or abrupt. Premonitory signs can include
increased activity levels, irritability, and anxiety followed by a loss of speech and
other skills. Usually the loss of skills reaches a plateau, after which some limited
improvement may occur, although improvement is rarely marked. In other instances,
especially when the disorder is associated with a progressive neurological condition, the
loss of skills is progressive. This disorder follows a continuous course, and in the
majority of cases, the duration is lifelong. The social, communicative, and behavioral
difficulties remain relatively constant throughout life.
299.80 Asperger's
Disorder
Diagnostic Features
The essential features of Asperger's Disorder are severe and sustained impairment in
social interaction (Criterion A) and the development of restricted, repetitive patterns of
behavior, interests, and activities (Criterion B) (see p. 66 in Autistic Disorder for a
discussion of Criteria A and B). The disturbance must cause clinically significant
impairment in social, occupational, or other important areas of functioning (Criterion C).
In contrast to Autistic Disorder, there are no clinically significant delays in language
(e.g., single words are used by age 2 years, communicative phrases are used by age 3
years) (Criterion D). In addition, there are no clinically significant delays in cognitive
development or in the development of age-appropriate self-help skills, adaptive behavior
(other than in social interaction), and curiosity about the environment in childhood
(Criterion E). The diagnosis is not given if the criteria are met for any other specific
Pervasive Developmental Disorder or for Schizophrenia (Criterion F).
Associated Features and Disorders
Asperger's Disorder is sometimes observed in association with general medical
conditions that should be coded on Axis III. Various nonspecific neurological symptoms or
signs may be noted. Motor milestones may be delayed, and motor clumsiness is often
observed.
Prevalence
Information on the prevalence of Asperger's Disorder is limited, but it appears to be
more common in males.
Course
Asperger's Disorder appears to have a somewhat later onset than Autistic Disorder, or
at least to be recognized somewhat later. Motor delays or motor clumsiness may be noted in
the preschool period. Difficulties in social interaction may become more apparent in the
context of school. It is during this time that particular idiosyncratic or circumscribed
interests (e.g., a fascination with train schedules) may appear or be recognized as such.
As adults, individuals with the condition may have problems with empathy and modulation of
social interaction. This disorder apparently follows a continuous course and, in the vast
majority of cases, the duration is lifelong.
Familial Pattern
Although the available data are limited, there appears to be an increased frequency of
Asperger's Disorder among family members of individuals who have the disorder.
Differential Diagnosis
Asperger's Disorder is not diagnosed if criteria are met for another Pervasive
Developmental Disorder or for Schizophrenia. For the differential diagnosis
with Autistic Disorder, [see Autistic Disorder]. For the differential diagnosis
with Rett's Disorder, [see Rett's Disorder]. For the differential diagnosis with Childhood
Disintegrative Disorder, [see Childhood Disintegrative Disorder]. Asperger's Disorder
must also be distinguished from Obsessive-Compulsive Disorder and Schizoid
Personality Disorder. Asperger's Disorder and Obsessive-Compulsive Disorder share
repetitive and stereotyped patterns of behavior. In contrast to Obsessive-Compulsive
Disorder, Asperger's Disorder is characterized by a qualitative impairment in social
interaction and a more restricted pattern of interests and activities. In contrast to
Schizoid Personality Disorder, Asperger's Disorder is characterized by stereotyped
behaviors and interests and by more severely impaired social interaction.
299.80 Pervasive Developmental Disorder Not Otherwise
Specified (Including Atypical Autism)
This category should be used when there is a severe and pervasive impairment in the
development of reciprocal social interaction or verbal and nonverbal communication skills,
or when stereotyped behavior, interests, and activities are present, but the criteria are
not met for a specific Pervasive Developmental Disorder, Schizophrenia, Schizotypal
Personality Disorder, or Avoidant Personality Disorder. For example, this category
includes atypical autism"--presentations that do not meet the criteria for Autistic
Disorder because of late age at onset, atypical symptomatology, or subthreshold
symptomatology, or all of these.
Last up-dated November 24, 2007
