Developmental And Behavioral
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autistic psychopathy, high-functioning autism, HFA, hyperlexia,
developmental disorder—not otherwise specified, PDD-NOS, pragmatic language
disorder, right hemisphere dysfunction, schizoid personality, semantic pragmatic
disorder, sensory integration disorder, persistent impairment in social interactions,
repetitive behavior patterns, restricted interests, pedantic speech, early childhood
motor delays, clumsiness,
fine motor difficulty
, gait anomalies, odd movements,
social insensitivity, severe social impairment,
abnormalities in speech, abnormalities in language, oddities in pitch, abnormalities
in intonation, abnormalities in prosody, abnormalities in rhythm,
lax joints, anomalies of locomotion, anomalies of balance, anomalies of manual
rhythm, anomalies of imitation of movements, impaired ball-playing skills, doll-play
paradigm, miscomprehension of language nuance, inability to use language in
social contexts, lack of sensitivity about interrupting others, irrelevant commentary,
absent facial expressions, inappropriate facial expressions, peer relation difficulties
, Adjunct Assistant Professor, Department of
Psychiatry, New York University School of Medicine; Fellow in Nuclear Medicine, Russell H
Morgan Department of Radiology and Radiological Science,
Johns Hopkins University School
James Robert Brasic, MD, MPH, is a member of the following medical societies:
Academy of Child and Adolescent Psychiatry
American Academy of Neurology
Movement Disorders Society
Editor(s): Carol Diane Berkowitz, MD, Executive Vice Chair, Professor, Department of
Pediatrics, Harbor-University of California at Los Angeles Medical Center; Mary L Windle,
Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Caroly Pataki, MD, Associate Program
Director, Clinical Associate Professor, Department of Psychiatry and Biobehavioral Sciences,
Division of Child and Adolescent Psychiatry, Neuropsychiatric Institute and Hospital, UCLA;
Professor, Departments of Psychiatry and Pediatrics, Northwestern University Medical
School; and Murray M Kappelman, MD, Professor, Departments of Pediatrics and
Psychiatry, University of Maryland School of Medicine
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Cornelia De Lange
by severe persistent impairment in social interactions, repetitive behavior patterns, and
restricted interests. Unlike an autistic disorder, no significant delay occurs in language
development or cognitive development. Asperger disorder is generally evident in children older
than 3 years and primarily occurs in boys.
Children with this disorder often exhibit a limited capacity for empathy, a failure to develop
friendships, and a limited number of intense and highly focused interests. While people with
Asperger disorder may have certain communication problems, including poor nonverbal
communication and pedantic speech, many individuals have good cognitive and verbal skills.
Physical symptoms may include early childhood motor delays, clumsiness, fine motor
difficulty, gait anomalies, and odd movements.
Individuals with Asperger disorder have normal or even superior intelligence, and they may
make great intellectual contributions, while demonstrating social insensitivity or even apparent
indifference toward loved ones. Published case reports of men with Asperger disorder suggest
an association with the capacity to accomplish cutting-edge research in computer science,
mathematics, and physics. While the deficits manifested by those with Asperger disorder are
often debilitating, many individuals experience positive outcomes, especially those who excel
in areas not dependent on social interaction. Persons with Asperger disorder have exhibited
outstanding skills in mathematics, music, and computer sciences. Many are highly creative,
and many prominent individuals demonstrate traits suggesting Asperger syndrome. As an
example, biographers describe Albert Einstein as a person with highly developed
mathematical skills who was unaware of social norms and insensitive to the emotional needs
of family and friends.
Although normal language and cognitive development differentiate Asperger disorder from
other developmental disorders, the severe social impairment associated with this condition
overlaps disorders such as high-functioning autism (HFA). For clinical management purposes,
Asperger disorder and HFA may be considered together. Impaired social skills are associated
with several other conditions (eg, developmental learning disability of the right hemisphere,
nonverbal learning disability, schizoid personality disorder, semantic-pragmatic processing
disorder, social-emotional learning disabilities).
For further information about conditions characterized by social impairments, restricted
interests, and mental retardation, see
Pervasive Developmental Disorder: Autism
Pathophysiology: The pathophysiology of Asperger disorder is unknown.
Some individuals with Asperger disorder have a history of problems in the prenatal and
neonatal periods and during delivery. The relationship between obstetric complications and
Asperger disorder is unclear. Events in early development may play a role in the pathogenesis
of Asperger disorder.
Neuroimaging of individuals with Asperger disorder and related conditions is described in
, an article that also includes hypotheses about the
possible pathophysiology of Asperger disorder.
In the US: Because of the divergent diagnostic criteria used in the United States and
Canada, estimates of Asperger disorder frequency vary widely. Various studies indicate
rates ranging from 1 case in 250-10,000 children. Additional epidemiologic studies are
needed, using widely accepted criteria and a screening instrument that targets these
Internationally: A population study in Sweden estimates the prevalence of Asperger
disorder as 1 case in 300 children. While this estimate is convincing for Sweden, the
findings may not apply elsewhere because they are based on a homogeneous
population. Extrapolating from this study, Asperger disorder may be more common than
clinicians once thought; pediatricians, family physicians, general practitioners, and other
health professionals may underdiagnose this disorder.
CME available for
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Brain and Nervous
they seem to endure an increased prevalence of comorbid psychiatric maladies (eg,
depression, mood disorders, obsessive-compulsive disorder, Tourette disorder).
Race: Asperger disorder has no racial predilection.
Sex: The estimated male-to-female ratio is approximately 4:1.
Age: Asperger disorder is commonly diagnosed in the early school years and less frequently
during early childhood or even adulthood.
Section 3 of 10
Interview parents about prenatal history and maternal health factors that may
have affected the pregnancy.
Include a thorough evaluation of social behaviors, language, interests, routines,
physical coordination, and sensory sensitivity, starting from birth.
Children with Asperger disorder may have difficulties with peer relations and may
be rejected by other children.
Outside the realm of immediate family members, the affected child may exhibit
inappropriate attempts to initiate social interaction and to make friends. Within the
immediate family, the child is often loving and affectionate.
Alternatively, an affected child may not display affection to parents or other family
members. A lack of bonding and warmth with parents and other guardians may
seem apparent, typically resulting from the child's lack of social skills.
Separations from parents because of work and divorce may be particularly
stressful for these children. Changing homes, communities, and neighborhoods
may also exacerbate symptoms.
Individuals with Asperger disorder may have particular difficulty in dating and
marriage. Boys and men with Asperger disorder may decide to marry suddenly
without the dating and courtship that typically precede a union. Individuals with
Asperger disorder may want to marry despite the lack of awareness of the many
social interactions that usually lead up to matrimony. For example, in the movie
Roger Dodger, an inexperienced youth with traits suggesting Asperger disorder
encounters difficulty in relations with women (Chaisson and Kidd, 2002). Such
problems may continue into adulthood.
Case vignette: A 50-year-old surgeon, who is an accomplished
amateur musician with a PhD in mathematics and who has traits
consistent with Asperger disorder, decides that it is time to marry
and have children. He has always lived at home with his parents.
Because he has trouble establishing relationships with women in his
ethnic group locally, he goes overseas to marry a cousin less than
half his age. He leaves his parents home for the first time to rent an
apartment with his wife. They have no sexual relationship. She finds
no career for herself in her new country, so she requests a divorce.
Immediately after the divorce the patient wants to marry another
his ethnic group.
People with Asperger disorder may benefit from counseling and social skills
training. Attwood (1998) provides exercises for parents to use to foster social
skills in their children. These activities can be modified for the needs of adults
with Asperger disorder. Psychotherapy is often helpful for individuals to recognize
their deficits in social skills.
People with Asperger disorder are vulnerable to depression, even suicide, after a
perceived rejection in a social situation such as dating and marriage. Clinicians
must be aware of the risk of depression and institute prompt interventions when
major depression occurs.
Socially inappropriate behavior and failure to understand social cues may be
The child may not understand why people become upset when he or she breaks
Use of gestures is frequently limited.
Body language or nonverbal communication may be awkward and inappropriate.
Facial expressions may be absent or inappropriate.
Speech and hearing
Affected children demonstrate several abnormalities in speech and language,
including pedantic speech and oddities in pitch, intonation, prosody, and rhythm.
Miscomprehension of language nuance (eg, literal interpretations of figures of
speech) is common.
Individuals often exhibit practical speech problems, including inability to use
language in social contexts, lack of sensitivity about interrupting others, and
Speech may be unusually formal or used in idiosyncratic ways that others do not
Individuals may vocalize their thoughts without censoring. Personal remarks
inappropriate to most social environments may be uttered routinely.
The amount of speech may also vary greatly and reflect the individual's current
emotional state more than the communication requirements of the social setting.
Some individuals may be verbose and others taciturn. Furthermore, the same
individual may demonstrate excesses and paucity of speech intermittently.
Some individuals may display selective mutism, speaking not at all to most
people and excessively to specific people. Some may choose to talk only to
people they like. Thus, speech may reflect idiosyncratic interests and preferences
of the individual.
The form of language chosen may include metaphors meaningful only to the
speaker. The message meant by the speaker may not be understood by those
who hear it, or the message may be meaningful only to a few people who
understand the private language of the speaker.
Children often exhibit auditory discrimination and distortion, particularly when the
child encounters 2 or more people speaking simultaneously.
These interests may be so important that the children do not develop typical
relationships with their family, school, and community.
Children may show sensitivity to sound, touch, taste, sight, smell, pain, and
temperature. For example, a child may demonstrate either extreme or diminished
sensitivity to pain.
Children may be particularly sensitive to the texture of foods.
Children may exhibit synesthesia, including a sensory response to an
environmental stimulus in a different sensory modality.
Key features of the deficit manifested in people with Asperger syndrome pertain to their
inability to understand the thoughts of other people and themselves. A typical child can
recognize the thoughts of other children and himself or herself and hypothesize how other
people are likely to respond to life occurrences. The lack of this comprehension in a person
with Asperger syndrome is termed a deficiency in the formation of a theory of the mind.
A theory of the mind can be thought of as a form of intuition in which young children learn how
other children respond to common situations. Children usually develop the skill to predict other
children's responses to common occurrences before they begin school. Some people with
Asperger syndrome appear never to develop a theory of mind.
Because most children have the ability to understand the mental processes of themselves and
others since early childhood, pediatricians and other clinicians need to recognize that children
with Asperger syndrome often lack abilities to intuit the thoughts of others and themselves.
Pediatricians and other clinicians may be shocked to recognize that otherwise intelligent
children with Asperger syndrome lack simple mental abilities to grasp situations that appear
obvious to even typical preschool children. Therefore, screening for a theory of mind is an
important process a pediatrician can use to identify some of the core behavioral symptoms of
Clinicians can screen for a theory of mind in a few minutes in offices, homes, and other
everyday settings with minimal props. Screening for a theory of mind involves a doll-play
paradigm and an imagination task.
The two components of the doll-play paradigm constitute a fundamental procedure to
demonstrate the presence of a theory of mind. The clinician and the patient are seated at
opposite ends of a table. The clinician shows the patient two dolls and names them by saying,
"This is Sally. This is Anne" (Baron-Cohen, 1985).
For the first procedure in the doll-play paradigm, the clinician tells and shows Sally placing a
marble in a basket. The clinician then removes Sally from the room and closes the door,
leaving her outside. The clinician then tells and shows Anne removing the marble from the
basket and placing it in a box. The clinician then brings Sally back into the room. The clinician
asks the patient, "Where will Sally look for the marble?” (Baron-Cohen, 1985)
Typical children, adolescents, and adults with a theory of mind will indicate that Sally will look
for the marble in the basket where she placed it before leaving the room. If this response is
elicited, the child passes the doll-play paradigm, and the subsequent sections and the clinician
may then proceed to the imagination task.
If the patient does not indicate that Sally will look for the marble in the basket where she
placed it before leaving the room, the clinician proceeds with questions to clarify the patient's
understanding of the situation. The clinician asks the patient, "Where is the marble really?"
state that the marble is in the box. The clinician then asks the patient, "Where was the marble
in the beginning?" (Baron-Cohen, 1985) Both typical and atypical children, adolescents, and
adults will usually state that the marble was originally in the basket.
The first procedure of the doll-play paradigm identifies the absence of a theory of mind when
an affected child, adolescent, or adult indicates that Sally will look for the marble in the box.
The patient thereby indicates an assumption that Sally, like the patient, will look for the marble
in the box because the patient knows that the marble is in the box. The ability to recognize that
Sally, unlike the patient, was absent and does not know that the marble was moved from the
basket into the box is an example of a theory of mind of Sally as distinct from that of the
For the second procedure of the doll-play paradigm, the clinician tells and shows Sally placing
a marble in a basket. The clinician then removes Sally from the room and closes the door,
leaving Sally outside. The clinician then tells and shows Anne removing the marble from the
basket and placing it in the clinician's pocket. The clinician then brings Sally back into the
room. The clinician then asks the patient, "Where will Sally look for the marble?"
Typical children, adolescents, and adults with a theory of mind will respond that Sally will look
in the basket because Sally last placed it in the basket. If this response is elicited, then the
patient passes the doll play paradigm so the clinician may proceed to the imagination task.
Otherwise, the clinician then asks the patient, "Where is the marble really?” (Baron-Cohen,
clinician's pocket. The clinician next asks the patient, "Where was the marble in the
beginning?” (Baron-Cohen, 1985) Both typical and atypical children, adolescents, and adults
respond that the marble was in the basket originally.
As for the first step in the doll-play paradigm, an absence of a theory of mind is identified when
an affected child, adolescent, or adult indicates that Sally will look for the marble in the
clinician's pocket. Affected children, adolescents, and adults repeatedly incorrectly think that
Sally will know the location of the marble because they do. Affected individuals do not
recognize that Sally's understanding of the placement of the marble is different from theirs
because she was absent when it was moved. This is evidence of deficits in the ability to
formulate a theory of mind in the affected person.
The final activity in the screen for a theory of mind is the imagination task. In this procedure,
the clinician tells the patient, "Now, I want you to close your eyes and think about a big white
teddy bear. Make a picture in your head of a big white teddy bear. Can you see the white
teddy?" (Baron-Cohen, Psychiatric Clin North Am, 1991) Typical children, adolescents, and
adults report the visualization of a big white teddy bear. If the patient does not report the
image of a big white teddy bear, then the clinician asks, "What can you see when you close
your eyes?" (Baron-Cohen, Psychiatric Clin North Am, 1991) If the patient reports any mental
image, then the clinician asks, "What are you thinking of?" (Baron-Cohen, Psychiatric Clin
North Am, 1991) Typical children, adolescents, and adults readily report the visualization of a
big white teddy bear with these stimuli.
The next activity of the imagination task is a repetition of the first part with the substitution of a
big red balloon for the white teddy bear. Typical children, adolescents, and adults readily
report the visualization of a big red balloon.
For the final activity of the imagination task, the clinician asks the patient to identify the first
picture of the task. Typical children, adolescents, and adults readily report that they first
imagined a big white teddy bear. The ability to remember an earlier mental image is evidence
of a theory of mind. The inability to recognize one's own prior mental images suggests the lack
of a theory of mind; therefore, the report that a big red balloon was first item imagined is
evidence of the absence of a theory of mind.
Typical children show evidence of having a theory of the mind before beginning school. Thus,
inability to correctly perform any of the theory of mind screening procedures in a school-aged
child suggests the need to refer the child for additional evaluation.