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Asperger's Syndrome
Updated: Jul 7, 2010
Introduction
Background
Asperger disorder is a form of pervasive developmental disorder characterized by persistent impairment in social interactions, repetitive behavior patterns, and restricted interests. Unlike autistic disorder, no significant aberrations or delays occur in language development or cognitive development. Asperger disorder is generally evident in children older than 3 years and occurs more often in boys.
Children with this disorder often exhibit a limited capacity for spontaneous social interactions, a failure to develop friendships, and a limited number of intense and highly focused interests. Although some people with Asperger disorder may have certain communication problems, including poor nonverbal communication and pedantic speech, many individuals have good cognitive and verbal skills. Bowler and colleagues have reported that, although people with Asperger disorder have fewer memories, the experiences of remembering are qualitatively similar in people with Asperger disorder compared with healthy control subjects.1 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 may make great intellectual contributions while demonstrating social insensitivity or even apparent indifference toward loved ones. Published case reports of individuals with Asperger disorder suggest an association with the capacity to accomplish cutting-edge research in computer science, mathematics, and physics. Although 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. For 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 with disorders such as high-functioning autism (HFA). The image below depicts an autism screening checklist.
Autism screening checklist.
The Autism Screening Checklist is helpful in identifying children with characteristics of autism spectrum disorders. It differentiates children with autism spectrum disorders from children with schizophrenia and other psychoses.
A score of "no" on the Autism Screening Checklist items 2, 4, and 11 and score of "yes" on items 4, 5, 6, 7, 8, 9, 10, 12, and 13, occurs in some children with autism and in children with other disorders. A score of "yes" on items 14, 15, 16, 17, 18, and 19 occurs in children with schizophrenia and other disorders, not in children with autism. A score of "yes" on items 1 and 3 occurs in healthy children and in children with autism. A score of "yes" on items 2 and 11 occurs in healthy children, not children with autism. The higher the score for "no" on items 2, 4, and 11 and for "yes" on items 5, 6, 7, 8, 9, 10, 12, and 13, on the Autism Screening Checklist (see the Multimedia section for a printable version), the more likely the presence of an autism spectrum disorder. A score of "no" on item 2 and "yes" on item 12 may occur in people with Asperger syndrome.
De Spiegeleer and Appelboom (2007) have pointed out that Asperger syndrome is an autism spectrum disorder.2 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.3,4,5 The relationship between obstetric complications and Asperger disorder is unclear.4,5 Events in early development may play a role in the pathogenesis of Asperger disorder.4,5
Neuroimaging of individuals with Asperger disorder and related conditions is described in PET Scanning in Autism Spectrum Disorders, an article that also includes hypotheses about the possible pathophysiology of Asperger disorder.
People with Asperger disorder demonstrate problems analyzing configurations. These deficits likely contribute to problems in facial recognition in people with Asperger disorder.6 Gaigg and Bowler (2007) hypothesize that impairments in the connections between the amygdala and associated structures of the brain may play a role in the pathogenesis of the symptoms of Asperger disorder.7
Frequency
United States
Because of the divergent diagnostic criteria used in the United States and Canada, estimates of Asperger disorder frequency widely vary. 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 criteria.
Likely, many people with Asperger disorder are undiagnosed in North America. Many people with Asperger disorder are probably members of the general population without an awareness of their diagnosis. Family and friends probably accommodate the signs of Asperger disorder as idiosyncrasies of the individual.
If Asperger disorder is diagnosed, then social skills training and other psychological interventions may be provided to the person. Additionally, some benefits may be available to people who have disabilities such as Asperger disorder.
International
A population study in Sweden estimates the prevalence of Asperger disorder as 1 case in 300 children. Although 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.
Mortality/Morbidity
Individuals with Asperger disorder appear to have normal lifespans; however, 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.
Clinical
History
Developmental history
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.
Social problems
Children with Asperger disorder may have difficulties with peer relations and may be rejected by other children. Depression and loneliness may occur in adolescents with Asperger syndrome.8
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. They may also be unaware that friendship often precedes courtship and engagement. 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.9 Such problems may continue into adulthood.
For example, a case vignette is as follows: 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 he should 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; she requests a divorce. Immediately after the divorce the patient wants to marry another woman. He complains that he is unable to find a suitable woman in 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.10 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 reported.
The child may not understand why people become upset when he or she breaks social rules.
Communication abnormalities
Use of gestures is frequently limited.
Body language or nonverbal communication may be awkward and inappropriate.
Facial expressions may be absent or inappropriate.
Pragmatic errors are commonly produced by children with Asperger disorder in response to questions. Children with Asperger disorder often produce irrelevant responses.11
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 an inability to use language in social contexts, a lack of sensitivity about interrupting others, and irrelevant commentary.
Speech may be unusually formal or used in idiosyncratic ways that others do not understand.
Individuals may vocalize their thoughts without censoring. Personal remarks inappropriate to most social environments may be uttered routinely.
The amount of speech may also widely vary 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 that are 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.
Activities
Children exhibit peculiar and narrow interests, excluding other activities.
These interests may be so important that the children do not develop typical relationships with their family, school, and community.
Sensory sensitivity
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.
Physical
Screening for a theory of mind
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.12,13,14,15,16
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.17
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 Asperger syndrome.
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.18
The 2 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 2 dolls and names them by saying, "This is Sally. This is Anne."19
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?"19
Typical children, adolescents, and adults with a theory of mind 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?"19 Both typical and atypical children, adolescents, and adults usually state that the marble is in the box. The clinician then asks the patient, "Where was the marble in the beginning?"19 Both typical and atypical children, adolescents, and adults 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 patient.
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?"19
Typical children, adolescents, and adults with a theory of mind 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. The clinician may proceed to the imagination task. Otherwise, the clinician then asks the patient, "Where is the marble really?"19
Both typical and atypical children, adolescents, and adults respond that the marble is in the clinician's pocket. The clinician next asks the patient, "Where was the marble in the beginning?"19 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?"20 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?"20 If the patient reports any mental image, then the clinician asks, "What are you thinking of?"20 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.
Physical findings
Typical physical findings in children with Asperger syndrome include the following:
- Lax joints are often observed (eg, an immature or unusual grasp for handwriting and other fine hand movements).
- Clumsiness is common.
- Affected children may exhibit anomalies of locomotion, balance, manual dexterity, handwriting, rapid movements, rhythm, and imitation of movements.
- Individuals exhibit impaired ball-playing skills.
Causes
Although its etiology is unknown, Asperger disorder is a behavioral syndrome caused by one or more influences acting on the CNS. Reports of families with multiple members meeting the criteria for this disorder suggest a genetic contribution to development of the disorder. Asperger disorder and autistic disorder are likely genetically related. Unfavorable experiences in the prenatal, perinatal, and postnatal periods may increase the likelihood of Asperger disorder3,4,5
More on Asperger's Syndrome |
 Overview: Asperger's Syndrome |
| Differential Diagnoses & Workup: Asperger's Syndrome |
| Treatment & Medication: Asperger's Syndrome |
| Follow-up: Asperger's Syndrome |
| Multimedia: Asperger's Syndrome |
| References |
| Next Page » |
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Further Reading
Keywords
Asperger syndrome, Asperger disorder, Asperger's syndrome, Asperger's, autistic psychopathy, high-functioning autism, HFA, hyperlexia, nonverbal learning disorder, NLD, personality disorder, pervasive developmental disorder
