Asperger Syndrome 

Asperger syndrome (Asperger disorder, Asperger’s syndrome) is a form of pervasive developmental disorder characterized by persistent impairment in social interactions and by repetitive behavior patterns and restricted interests. Unlike autistic disorder, no significant aberrations or delays occur in language development or in cognitive development. Asperger syndrome is generally evident in children older than age 3 years and occurs most often in males. (See Etiology and Epidemiology.)

De Spiegeleer and Appelboom pointed out that Asperger syndrome is an autism spectrum disorder.^[1] Although normal language and cognitive development differentiate Asperger syndrome from other developmental disorders, the severe social impairment associated with this condition overlaps with disorders such as high-functioning autism (HFA). (See Diagnostic Considerations.)

Children with this syndrome 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 syndrome may have certain communication problems, including poor nonverbal communication and pedantic speech, many individuals have good cognitive and verbal skills. (See History.)

Bowler and colleagues reported that although people with Asperger syndrome have fewer memories, the experiences of remembering are qualitatively similar in people with Asperger syndrome compared with healthy control subjects.^[2]

Physical symptoms may include early childhood motor delays, clumsiness, fine motor difficulty, gait anomalies, and odd movements. (See Physical Examination.)

Individuals with Asperger syndrome 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 syndrome suggest an association with the capacity to accomplish cutting-edge research in computer science, mathematics, and physics, as well as outstanding creative work in art, film, and music. Although the deficits manifested by those with Asperger syndrome are often debilitating, many individuals experience positive outcomes, especially those who excel in areas not dependent on social interaction. (See Prognosis and Treatment.)

Persons with Asperger syndrome 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 have described 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.

For clinical management purposes, Asperger syndrome 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).

The Autism Screening Checklist (seen below) 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 "yes" on items 1, 3, and 4 of the Autism Screening Checklist occurs in healthy children and in children with autism spectrum disorders, including Asperger syndrome. Some children with autism and other autism spectrum disorders demonstrate normal development for the first couple of years or so and then demonstrate a regression with loss of language skills. Children with autism may or may not speak. Therefore, children with autism may score “no” or “yes” on item 4. Children with Asperger syndrome develop speech at the usual age. They may display oddities of speech characteristic of autism and Asperger syndrome. A score of "yes" on items 2 and 11 occurs in healthy children, not in children with autism spectrum disorders and other pervasive developmental disorders.

A score of "no" on the Autism Screening Checklist items 2 and 11 and a score of "yes" on items 5, 6, 7, 8, 9, 10, 12, and 13 occurs in some children with autism spectrum disorders. 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, the more likely the presence of an autism spectrum disorder. 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 Asperger syndrome and other autism spectrum disorders. A score of "no" on item 2 and "yes" on item 12 may occur in people with Asperger syndrome.

Go to Pervasive Developmental Disorder and Autism for complete information on these topics.

Associated morbidities

Depression and hypomania are common among adolescents and adults with Asperger syndrome, particularly those with a family history of these conditions. (Caregivers of persons with Asperger syndrome may be prone to depression as well.)

An increased risk of suicide is observed in persons with Asperger syndrome, with risks possibly rising in proportion to the number and severity of comorbid maladies. Asperger syndrome is probably undiagnosed in many suicide cases because of the dearth of awareness of the condition's existence and the ineffective and unreliable tools used to identify it. Therefore, people with Asperger syndrome who commit suicide are probably reported as having other or undiagnosed psychiatric problems. In cases of unexpected suicide, Asperger syndrome is a strong possibility.

Every person who interacts with people with Asperger syndrome can benefit from developing an awareness of the symptoms of depression. When these symptoms occur in people with Asperger syndrome, family, friends, and others, the afflicted person can be guided to receive the needed help.

Several criteria have been identified to diagnose depression. A major depression is characterized by the presence of the symptoms on a sustained basis for at least 2 weeks. In other words, transient sadness lasting a few hours does not qualify as major depression. In order to meet the criteria for a symptom of depression, the symptom must interfere with the person’s life, possibly in educational, occupational, or social settings.

The key hallmarks include depression and anhedonia. Anhedonia is characterized by the inability to experience pleasure. Anhedonia is a symptom of depression. Either depression or anhedonia must be present to diagnose major depression. The presence of depression can be elicited by asking the person, “Do you feel low, blue, sad, down in the dumps?” The presence of anhedonia can be elicited by asking if the person experiences pleasure from activities that usually produce pleasure.

Symptoms of depression

In addition to depression and anhedonia, 7 other symptoms of depression are noted below.

Disturbances of eating are typical in depression; the person may lose weight when not dieting or may gain weight. A change of 5% of the body weight in 1 month qualifies as a symptom of depression. Alternatively, the person may experience a marked decrease or increase in appetite.

Sleep disturbances are common in depression. The person may experience insomnia. Difficulty falling asleep may be reported. Ask the person, “Do you wake up in the middle of sleeping? Do you wake up earlier in the morning than usual?” Alternatively, the person may sleep more than usual.

Disturbances of activity levels often occur in depression. The person may move much more frequently or much less frequently than usual. This may lead others to comment that the activity level has changed.

The person may have a loss of energy and a persistent feeling of tiredness.

The person may have difficulty concentrating.

The person may experience feelings of guilt, helplessness, and hopelessness. Ask the person, “Do you feel worthless?”

Addressing patient depression

Clinicians must be aware of the risk of depression and institute prompt interventions when major depression occurs.

The person may have thoughts that life is not worth living. The person may consider, plan, attempt, or commit suicide. This symptom requires immediate evaluation by a mental health professional. Involuntary psychiatric hospitalization is indicated if the person is acutely suicidal.

Throughout the process of interacting with a person who has depression, the person needs to be informed that the depression will probably pass. Unlike other progressive mental disorders, depression is usually a remitting illness. In other words, the depression typically resolves entirely without treatment. However, treatment likely hastens the onset of recovery.

Still, a person with depression may be convinced that recovery is not possible. This may be a result of the temporary feeling of hopelessness common in depression. The belief that the person will never recover may lead to suicide. For this reason, people with depression must be told that the depression will probably completely resolve. Inform the person that sometimes people’s minds play tricks on them and that they will probably completely recover.

People who are depressed may need assistance to obtain help from mental health professionals. If a person is suicidal, call emergency services (eg, 911) to ask for an ambulance for a person with a mental disorder. People who are a danger to themselves merit commitment to mental hospitals for treatment to protect them from hurting themselves.

People with Asperger syndrome are vulnerable to depression, even suicide, after a perceived rejection in a social situation such as dating and marriage.

Additional morbidities

People with Asperger syndrome can have other neuropsychiatric disorders, including Tourette syndrome, anorexia nervosa, and schizophrenia; treating such comorbid disorders may be beneficial.

Other concerns

Changes to a child's environment may exacerbate symptoms of Asperger syndrome. Therefore, minimize separations if the child is fond of family members, teachers, or others.

People with Asperger syndrome may be entitled to benefits for people with disabilities.

The etiology of Asperger syndrome is unknown. Some individuals with Asperger syndrome have a history of problems in the prenatal and neonatal periods and during delivery,^{[3, 4, 5]} but the relationship between obstetric complications and Asperger syndrome is unclear.^{[4, 5]} Unfavorable experiences in the prenatal, perinatal, and postnatal periods may increase the likelihood of Asperger syndrome.^{[3, 4, 5, 6, 7]}

Events in early development may play a role in the pathogenesis of Asperger syndrome.^{[4, 5]}

People with Asperger syndrome demonstrate problems analyzing configurations. These deficits likely contribute to problems in facial recognition in people with Asperger syndrome.^[8] Gaigg and Bowler hypothesized 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 syndrome.^[9]

Although its etiology is unknown, Asperger syndrome is a behavioral syndrome caused by 1 or more influences acting on the central nervous system (CNS). Reports of families with multiple members meeting the criteria for this disorder suggest a genetic contribution to development of the disorder. Asperger syndrome and autistic disorder are likely genetically related.

For more information, see PET Scanning in Autism Spectrum Disorders.

Incidence in North America

Because of the divergent diagnostic criteria used in the United States and Canada, estimates of the prevalence of Asperger syndrome 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 criteria.

Likely, many people in North America with Asperger syndrome are undiagnosed. Many people with Asperger syndrome are probably members of the general population without an awareness of their diagnosis. Family and friends probably accommodate the signs of Asperger syndrome as idiosyncrasies of the individual.

If Asperger syndrome is diagnosed, then social skills training and other psychologic interventions may be provided to the person. Additionally, some benefits may be available to people who have disabilities such as Asperger syndrome.

International incidence

A population study in Sweden estimated the prevalence of Asperger syndrome 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 syndrome may be more common than clinicians once thought; pediatricians, family physicians, general practitioners, and other health professionals may underdiagnose this disorder.

Race predilection

Asperger syndrome has no racial predilection.

Sex predilection

The estimated male-to-female ratio is approximately 4:1.

Age predilection

Asperger syndrome is commonly diagnosed in the early school years and less frequently during early childhood or in adulthood.

Individuals tend to have a better prognosis when they have supportive families who are knowledgeable about Asperger syndrome.

Individuals with Asperger syndrome may be taught specific social guidelines, but the underlying social impairment is believed to be lifelong.

Mortality and morbidity

Individuals with Asperger syndrome appear to have a normal lifespan; however, they seem to endure an increased prevalence of comorbid psychiatric maladies (eg, depression, mood disorders, obsessive-compulsive disorder, Tourette disorder).

Comorbid psychiatric disorders, when present, significantly affect the patient's prognosis.

People with developmental disabilities, including Asperger syndrome, are vulnerable to sexual abuse. The most severely disabled are at highest risk for sexual abuse. For this reason, parents and caregivers must be alert to avoid situations inviting sexual abuse. Additionally, children with Asperger syndrome must be trained to recognize impending sexual abuse and to develop plans of action to abort possible sexual abuse.^[10] .

For patient education information, see the Brain and Nervous System Center, as well as Asperger Syndrome.

Resources

Individuals with Asperger syndrome (and related conditions), their families, teachers, and communities benefit from the experiences of other individuals with this disorder and from the experiences of their advocates. The following organizations provide information and advice to persons with Asperger syndrome and related conditions:

• ASPEN (Asperger Syndrome Education Network, Inc.). 9 Aspen Circle; Edison, NJ 08820. Telephone: 732-321-0880; Email: info@aspennj.org
• Asperger Norfolk. Old Lion Cottage; Thurne, Great Yarmouth; NR29 3AP; United Kingdom. Telephone: 01 692 670 864
• www.aspie.com
• http://www.jkp.com/ (Jessica Kingsley Publishers). 116 Pentonville Road; London, N1 9JB; United Kingdom. Email: post@jkp.com; Telephone: +44 (0)20 7833 2307; Fax: +44 (0)20 7837 2917
• Jessica Kingsley Publishers, Inc. 400 Market Street, Suite 400; Philadelphia, PA 19106. Telephone (toll free ordering): 866-416-1078; Telephone (main office): 215-922-1161; Fax: 215-922-1474; Email: orders@jkp.com

Also contact the above resources for information about assessment and treatment facilities located near the patient. Individuals with Asperger syndrome and their families benefit from intensive assessments and treatment interventions.

Several other resources have been recorded in a manual for parents of young people with Asperger syndrome.^[11] This excellent guide for lay people who encounter people with Asperger syndrome provides practical suggestions for day-to-day life.