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Asperger syndrome
Specialty	Psychiatry

Asperger syndrome – also called Asperger's syndrome, AS, or just Asperger's – is one of five neurobiological pervasive developmental disorders (PDD), and is characterized by normal intelligence and language development, but deficiencies in social and communication skills. The diagnosis of Asperger syndrome is complicated by the lack of a standard diagnostic screen, and the use of several different screening instruments and sets of diagnostic criteria. The exact cause of AS is unknown, and the prevalence is not firmly established, due partly to the use of differing sets of diagnostic criteria.

Asperger syndrome was named in honor of Hans Asperger, an Austrian psychiatrist and pediatrician, by researcher Lorna Wing, who first used the eponym in a 1981 paper. In 1994, AS was recognized in the Diagnostic and Statistical Manual of Mental Disorders (DSM).

Classification and diagnosis

AS is an autism spectrum disorder, one of five neurological conditions characterized by impairment in language and communication skills, as well as repetitive or restrictive patterns of thought and behavior. The four related disorders are autism, Rett syndrome, childhood disintegrative disorder, and PDD-NOS (pervasive developmental disorder not otherwise specified).

AS is defined in section 299.80 of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) by six main criteria. These criteria define AS as a condition in which there is:

1. qualitative impairment in social interaction
2. the presence of restricted, repetitive and stereotyped behaviors and interests
3. significant impairment in important areas of functioning
4. no significant delay in language
5. no significant delay in cognitive development, self-help skills, or adaptive behaviors (other than social interaction).
6. The symptoms must not be better accounted for by another specific pervasive developmental disorder or schizophrenia.

The diagnosis of AS is complicated by the use of several different screening instruments. The diagnostic criteria of the Diagnostic and Statistical Manual are criticized for being vague and subjective. Other sets of diagnostic criteria for AS are the ICD 10 World Health Organization Diagnostic Criteria, Szatmari Diagnostic Criteria, Gillberg Diagnostic Criteria, and Attwood & Gray Discovery Criteria. The ICD-10 definition has similar criteria to the DSM-IV version. In the ICD-10 the phrase Asperger's syndrome is synonymous with Autistic psychopathy and Schizoid disorder of childhood.

Some doctors believe that AS is not a separate and distinct disorder, referring to it as high functioning autism (HFA). The diagnoses of AS or HFA are used interchangeably, complicating prevalence estimates: the same child can receive different diagnoses, depending on the screening tool the doctor uses, and some children will be diagnosed with HFA instead of AS, and vice versa.

Characteristics

AS is characterized by:

• limited interests or preoccupation with a subject to the exclusion of other activities;
• repetitive behaviors or rituals;
• peculiarities in speech and language;
• socially and emotionally inappropriate behavior and interpersonal interaction
• problems with non-verbal communication; and
• clumsy and uncoordinated motor movements.

The most common and important characteristics of AS can be divided into several broad categories: social impairments, narrow but intense interests, and peculiarities of speech and language. Other features are commonly associated with this syndrome, but are not always regarded as necessary for diagnosis. This section mainly reflects the views of Attwood, Gillberg, and Wing on the most important characteristics of AS; the DSM-IV criteria represent a slightly different view. Unlike most forms of PDDs, AS is often camouflaged, and many people with the disorder blend in with those that do not have it. The effects of AS also depend on how an individual with the syndrome responds to it.

Social impairments

Although there is no single feature that all people with AS share, difficulties with social behavior are nearly universal and are one of the most important defining criteria. People with AS lack the natural ability to see the subtexts of social interaction (e.g., resulting in well-meaning remarks that may offend, or finding it hard to know what is "acceptable") and may lack the ability to communicate their own emotional state. The unwritten rules of social behavior, that mystify so many with AS, have been termed "The Hidden Curriculum". People with AS must learn these social skills intellectually rather than intuitively.

Non-autistics are able to gather information about other people's cognitive and emotional states based on clues gleaned from the environment and the other people's facial expression and body language, but in this respect, people with AS are impaired; this is sometimes called mind-blindness. Mind-blindness involves an impaired ability to: read others' feelings, understand intended meanings and gauge level of interest in a conversation; take into account others' level of knowledge and predict someone's reaction to a comment or action.

Some people with AS make very little eye contact because they find it overwhelming, whereas others have unmodulated, staring eye contact that can cause discomfort in other people. Similarly, the use of gestures may be almost nonexistent or may seem exaggerated and differ from what would normally be considered the most appropriate for a situation.

Narrow, intense interests

AS can involve an intense and obsessive level of focus on things of interest. For example, one person might be obsessed with 1950s professional wrestling, another with national anthems of African dictatorships, and another with building models out of matchsticks. Particularly common interests are means of transport (for example, trains), computers, mathematics, astronomy, geography, history, and dinosaurs. Note that all of these are normal interests in ordinary children; the difference in Asperger children is the unusual intensity of their interest.

Sometimes these interests are lifelong; in other cases, they change at unpredictable intervals. In either case, there are normally one or two interests at any given time. In pursuit of these interests, people with AS often manifest extremely sophisticated reasoning, an almost obsessive focus, and a remarkably good memory for apparently trivial facts (occasionally even eidetic memory). Hans Asperger called his young patients "little professors" because he thought his thirteen-year-old patients had as comprehensive and nuanced an understanding of their field of interest as university professors.

Some clinicians do not entirely agree with this description; for example, Wing and Gillberg both argue that these areas of intense interest typically involve more rote memorization than real understanding, despite occasional appearances to the contrary. However, such a limitation is an artifact of the diagnostic criteria, even under Gillberg's criteria.

People with Asperger syndrome may have little patience for things outside these narrow interests. In school, they may be perceived as highly intelligent underachievers or overachievers, clearly capable of outperforming their peers in their field of interest, yet persistently unmotivated to do regular homework assignments (sometimes even in their areas of interest). Others may be hypermotivated to outperform peers in school. The combination of social problems and intense interests can lead to unusual behavior, such as greeting a stranger by launching into a lengthy monologue about a special interest rather than introducing oneself in the socially-accepted way. However, in many cases adults can outgrow this impatience and lack of motivation and develop more tolerance to new activities and meeting new people.

Speech and language peculiarities

People with AS typically having a highly pedantic way of speaking, using a far more formal register of language than appropriate for a context. A five-year-old child with this condition may regularly speak in language that could easily have come from a university textbook, especially concerning his or her special area of interest.

Literal interpretation is another common, but not universal hallmark of this condition. Attwood gives the example of a girl with AS who answered the telephone one day and was asked, "Is Paul there?" Although the Paul in question was in the house, he was not in the room with her, so after looking around to ascertain this, she simply said "no" and hung up. The person on the other end had to call back and explain to her that he meant for her to find him and get him to pick up the telephone.

People with AS sometimes use words idiosyncratically, including new coinages and unusual juxtapositions. This can develop into a rare gift for humor (especially puns, wordplay, doggerel and satire). A potential source of humor is the eventual realization that their literal interpretations can be used to amuse others. Some are so proficient at written language as to qualify as hyperlexic. Tony Attwood refers to a particular child's skill at inventing expressions, e.g., "tidying down" (the opposite of tidying up) or "broken" (when referring to a baby brother who cannot walk or talk).

Another typical behavioural characteristic is echolalia, which causes the subject to repeat words, or parts of words, like an echo, and palilalia repeating one's own words. Children with AS may show advanced abilities for their age in language, reading, mathematics, spatial skills, or music, sometimes into the 'gifted' range, although these talents may be counterbalanced by appreciable delays in the developmental of other cognitive functions.

Writing peculiarities

A 2003 study investigated the written language of children and youth with AS. They were compared in a standardized test of written language skills and legibility of handwriting. In written language skills, no significant differences were found between standardized scores of both groups; however, in hand-writing skills, the AS participants produced significantly fewer legible letters and words than the neurotypical group. Another analysis of written samples found that people with AS appear to be able to write quantitatively similar to their neurotypical peers using grammatical rules, but have difficulty in producing qualitative writing.

Tony Attwood states that a teacher may spend considerable time interpreting and correcting an AS child's indecipherable scrawl. The child is also aware of the poor quality of his or her handwriting, and may be reluctant to engage in activities that involve extensive writing. Unfortunately for some children and adults, high school teachers and prospective employers may consider the neatness of handwriting a measure of intelligence and personality. People with AS then become embarrassed or angry at their inability to write neatly. The child may require assessment by an occupational therapist and remedial exercises, but modern technology can help minimise this problem. A parent or teacher aide could also act as the child's scribe or proofreader to ensure the legibility of the child's written answers or homework.

People with AS may be very skilled at using computers and keyboards. This allows for possible special dispensation in schoolwork to type rather than write homework and examinations. The presentation of their work is then comparable to that of other children.

Emotional peculiarities

A person with AS may have trouble understanding the emotions of other people: the messages that are conveyed by facial expression, eye contact and body language are often missed. They also might have trouble showing empathy with other people. Thus, people with AS might be seen as egotistical, selfish or uncaring. In most cases, these are unfair labels, because affected people are neurologically unable to understand other people's emotional states. They are usually shocked, upset and remorseful when told that their actions are hurtful or inappropriate. It is clear that people with AS do not lack emotions. However, the concrete nature of attachments they might have (i.e., to objects rather than to people) often seems curious, or even can be cause of concern, to people who do not share their perspective.

Failing to show affection—or not doing so in conventional ways—does not necessarily mean that people with AS do not feel it. Understanding this can lead partners or carers to feel less rejected and to be more understanding. There are usually ways to work around the problems, such as being more explicit about one's needs. For instance, when describing emotions, it can be helpful to be direct and to avoid vague terms such as "upset" when the emotion being described is anger (some individuals with AS would interpret "upset" as mere annoyance, or even nausea). It is often effective to present in clear language what the problem is and to ask the partner with AS to describe what emotions are being felt, or to ask why a certain emotion was being felt. It is helpful if the family member or significant other reads as much as he or she can about AS and any comorbid disorders. Sometimes, the opposite problem occurs; the person with AS is unusually affectionate to significant others and misses or misinterprets signals from the other partner, causing the partner stress.

Other characteristics

Those affected by AS may show a range of other sensory, developmental, and physiological anomalies. Children with AS may evidence a slight delay in the development of fine motor skills. In some cases, people with AS may have an odd way of walking, and may display compulsive finger, hand, arm or leg movements.

In general, orderly things appeal to people with AS. Some researchers mention the imposition of rigid routines (on self or others) as a criterion for diagnosing this condition. It appears that changes to their routines cause inordinate levels of anxiety for some people with this condition.

Some people with AS experience varying degrees of sensory overload and are extremely sensitive to touch, smells, sounds, tastes, and sights. They may prefer soft clothing, familiar scents, or certain foods. Some may even be pathologically sensitive to loud noises (as some people with AS have hyperacusis) or strong smells or dislike being touched; for example, certain children with AS exhibit a strong dislike of having their head touched or their hair disturbed while others like to be touched but dislike loud noises. Sensory overload may exacerbate problems faced by such children at school, where levels of noise in the classroom can become intolerable for them. Some are unable to block out certain repetitive stimuli, such as the constant ticking of a clock. Whereas most children stop registering this sound after a short time and can hear it only if they consciously attend to it, a child with AS can become distracted, agitated, or even (in cases where the child has problems with regulating emotions such as anger) aggressive if the sound persists.

People with severe AS symptoms may frequently be diagnosed with clinical depression, oppositional defiant disorder, antisocial personality disorder, Tourette syndrome, ADHD, general anxiety disorder, bipolar disorder, obsessive compulsive disorder, or obsessive-compulsive personality disorder. Dysgraphia, Dyspraxia, Dyslexia, or Dyscalculia may also be diagnosed; however, while elements from all of these conditions are associated with AS, the cause is usually the syndrome itself, rather than a separate condition. While there may be a correlation between them, the disorders are distinct. Research indicates people with AS may be far more likely to have the associated conditions, but the connection appears to be largely one of resemblance. For example, the "obsessions" common to those with AS are usually focused on a special interest; these differ in nature to those specific to OCD, which tend to be based on anxieties, such as a fear of germs.

Causes

The cause of AS is unknown, but since it was properly defined several possible causes of AS have been investigated by researchers. These possible causes include: genetics, epilepsy, folic acid, a serotonin dysfunction, cerebellar dysfunction, an enlarged amygdala and hippocampus,and other brain problems.

Treatment

Treatment for AS coordinates therapies that address the core symptoms of the disorder: poor communication skills, obsessive or repetitive routines, and physical clumsiness. There is no one best treatment modality for all persons with AS, but most professionals agree that early intervention is beneficial for children.

An effective treatment program generally includes:

• social skills training, to teach children with AS skills to interact more successfully with other children
• cognitive behavioral therapy, to help explosive or anxious children to manage their emotions better and cut back on obsessive interests and repetitive routines
• medication, for co-existing conditions such as depression and anxiety
• occupational or physical therapy, for children with sensory integration problems or poor motor coordination
• specialized speech therapy, to help children who have trouble with the give and take of normal conversation
• parent training and support, to teach parents behavioral techniques to use at home.

AS and high-functioning autism may be considered together for the purpose of clinical management.

These treatments are controversial, based on claims that they may do more harm than good. There is also a viewpoint in the autistic community that AS is not a disease/disorder, but just a variation in neurology.

Prognosis

Persons with AS appear to have normal lifespans, but an increased prevalence of comorbid psychiatric conditions, such as depression, mood disorders, and obsessive-compulsive disorder.

Children with AS can learn to manage their differences, but they may continue to find social situations and personal relationships challenging. Many adults with AS are able to work successfully in mainstream jobs, although they may continue to need encouragement and moral support to maintain an independent life.

Individuals with AS may make great intellectual contributions: published case reports suggest an association with accomplishments in computer science, mathematics, and physics. The deficits associated with AS may be debilitating, but many individuals experience positive outcomes, particularly those who are able to excel in areas not dependent on social interaction, such as mathematics, music, and the sciences.

Epidemiology

The prevalence of AS is not well established, but conservative estimates using the DSM-IV criteria indicate that two to three of every 10,000 children have the condition, making it rarer than autistic disorder. Three to four times as many boys have AS compared with girls. The universality of AS across races, and validity of epidemiologic studies to date, is questioned.

A 1993 broad-based population study in Sweden found that 36 per 10,000 school-aged children met Gillberg's criteria for AS, rising to 71 per 10,000 if suspected cases are included. The estimate is convincing for Sweden, but the findings may not apply elsewhere because they are based on a homogeneous population. The Sweden study demonstrated that AS may be more common than once thought and underdiagnosed. Gillberg estimates 30-50% of all AS is undiagnosed. A survey found that 36 per 10,000 of adults with IQ of 100 or above may meet criteria for AS.

Leekam et al. documented significant differences between Gillberg's criteria and the ICD-10 criteria. Considering its requirement for "normal" development of cognitive skills, language, curiosity and self-help skills, ICD-10 is considerably more narrow than Gillberg's criteria, which more closely matches Hans Asperger's own descriptions.

Like other autism spectrum disorders, AS prevalance estimates for males are higher than for females, but some clinicians believe that this may not reflect the actual incidence among females. Tony Attwood suggests that females learn to better compensate for their impairments because of differences in socialization. The Ehlers & Gillberg study found a 4:1 male to female ratio in subjects meeting Gillberg's criteria for AS, but a lower 2.3:1 ratio when suspected or borderline cases were included.

The prevalence of AS in adults is not well understood, but Baron-Cohen et al. documented that 2% of adults score higher than 32 in his Autism Spectrum Quotient (AQ) questionnaire, developed in 2001 to measure the extent to which an adult of normal intelligence has the traits associated with autism spectrum conditions. All interviewed high-scorers met at least 3 DSM-IV criteria, and 63% met threshold criteria for an ASD diagnosis; a Japanese study found similar AQ Test results.

Most patients presenting in clinical settings with AS have other comorbid psychiatric disorders. Children are likely to present with attention-deficit hyperactivity disorder (ADHD), while depression is a common diagnosis in adolescents and adults. A study of referred adult patients found that 30% presenting with ADHD had ASD as well.

History

In 1944, an Austrian pediatrician named Hans Asperger observed four children in his practice who had difficulty integrating socially. Although their intelligence appeared normal, the children lacked nonverbal communication skills, failed to demonstrate empathy with their peers, and were physically clumsy. Their way of speaking was either disjointed or overly formal, and their all-absorbing interest in a single topic dominated their conversations. Dr. Asperger called the condition “autistic psychopathy” and described it as a personality disorder primarily marked by social isolation.

Hans Asperger and Leo Kanner identified essentially the same population, although the group identified by Asperger was perhaps more "socially functional" than Kanner's. Traditionally, Kannerian autism is characterized by significant cognitive and communicative deficiencies, including delays in or lack of language. However, a person with AS will not show delays in language.

Asperger’s observations, published in German, were not widely known until 1981, when an English doctor named Lorna Wing published a series of case studies of children showing similar symptoms, which she called “Asperger’s” syndrome. Wing’s writings were widely published and popularized. In 1992, the tenth published edition of the World Health Organization’s diagnostic manual and the International Classification of Diseases (ICD-10) included AS, making it a distinct disease and diagnosis. Later, in 1994, the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) and the American Psychiatric Association’s diagnostic reference book also added AS.

Uta Frith (an early researcher of Kannerian autism) wrote that people with AS seem to have more than a touch of autism to them. Others, such as Lorna Wing and Tony Attwood, share Frith's assessment. Dr. Sally Ozonoff, of the University of California at Davis's MIND institute, argues that there should be no dividing line between "high-functioning" autism and AS, and that the fact that some people do not start to produce speech until a later age is no reason to divide the two groups because they are identical in the way they need to be treated.

Cultural and sociological aspects

People with AS may refer to themselves in casual conversation as "aspies", coined by Liane Holliday Willey in 1999, or as an "Aspergian". The term neurotypical (NT) describes a person whose neurological development and state are typical, and is often used to refer to people who are non-autistic.

Autistic people have contributed to a shift in perception of autism spectrum disorders as complex syndromes rather than diseases that must be cured. Proponents of this view reject the notion that there is an 'ideal' brain configuration and that any deviation from the norm is pathological. They demand tolerance for what they call their neurodiversity in much the same way physically handicapped people have demanded tolerance. These views are the basis for the autistic rights and autistic pride movements. Researcher Simon Baron-Cohen has argued that high-functioning autism is a difference and not necessarily a disability. He contends that the term "difference" is more neutral and value-free, and that this small shift in a term could mean the difference between a diagnosis of AS being received as a family tragedy, or interesting information, such as learning that a child is left-handed.

A Wired magazine article, The Geek Syndrome, suggested that AS is more common in the Silicon Valley, a haven for computer scientists and mathematicians. It posited that AS may be the result of assortative mating by geeks in mathematical and technological areas. However, AS can be found in all occupations, and is not limited to those in the math and science fields.

The popularization of the Internet has allowed individuals with AS to communicate with each other in a way that was not possible to do offline because of the rarity and the geographic dispersal of individuals with ASD. As a result of AS individuals' increasing ability to connect with one another, a subculture of "Aspies" has formed. Internet sites have made it easier for individuals to connect with each other.

Social impact

AS may lead to problems in social interaction with peers. These can be severe or mild depending on the individual. Children with AS are often the target of bullying at school because of their idiosyncratic behavior, language, interests, and their impaired ability to perceive and respond in socially expected ways to nonverbal cues, particularly in interpersonal conflict. Children with AS may be extremely literal and may have difficulty interpreting sarcasm. A child or teen with AS is often puzzled by this mistreatment, unaware of what has been done incorrectly. Unlike other pervasive development disorders, most children with AS want to be social but fail to socialize successfully, which can lead to later withdrawal and asocial behavior, especially in adolescence.

Children with AS often display advanced abilities for their age in language, reading, mathematics, spatial skills, and/or music—sometimes into the "gifted" range—but this may be counterbalanced by considerable delays in other developmental areas. This combination of traits can lead to problems with teachers and other authority figures. A child with Asperger’s might be regarded by teachers as a "problem child" or a "poor performer". The child’s extremely low tolerance for what they perceive to be ordinary and mediocre tasks, such as typical homework assignments, can easily become frustrating; a teacher may well consider the child arrogant, spiteful, and insubordinate. Lack of support and understanding, in combination with the child's anxieties, can result in problematic behavior (such as severe tantruming, violent outbursts, and withdrawal.

Adults with AS are not treated as regularly as children, yet may have similar problems. They may find it difficult gaining a job or entering undergraduate or graduate schools because of poor interview skills or a low score on standardized or personality tests. They also may be more vulnerable to poverty than the general population, because of their difficulty getting a job, lack of proper education, and premature social skills. If they do get a job they might be misunderstood and become subject to bullying. They may also have difficulty finding a life partner and getting married because of poor social skills. People with AS report a feeling of being unwillingly detached from the world around them. On the other hand there are Asperger adults who do get married, get a doctorate from a fine university, and hold a job. The intense focus and tendency to work things out logically, a characteristic of Asperger syndrome, often grants people with the syndrome a high level of ability in their field of interest. When these special interests coincide with a materially or socially useful task, the person with Asperger's often can lead a profitable life. The child obsessed with naval architecture may grow up to be an accomplished shipwright. More research is needed on adults with AS.

Notable cases

AS is sometimes viewed as a syndrome with both advantages and disadvantages, and notable adults with AS or autism have achieved success in their fields. Prominent Asperger-diagnosed individuals include Nobel Prize-winning economist Vernon Smith, industrial rocker Gary Numan, Vines frontman Craig Nicholls, and Satoshi Tajiri, the creator of Pokémon.

Some AS researchers speculate that well-known figures, including Albert Einstein, Isaac Newton and Ludwig Wittgenstein, had AS because they showed some AS-related tendencies or behaviors, such as intense interest in one subject, or social problems. Stanley Kubrick is also speculated to have had the syndrome. However, posthumous diagnoses remain controversial, and autistic rights activists use such speculative diagnoses to argue that it would be a loss to society if autism were cured.

See also

• List of further reading on Asperger syndrome

References

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External links

• Stephen Shore's website offering his insight into life with Asperger syndrome
• Asperger's Syndrome in Adults - From Mental Health Matters
• Institute for the Study of the Neurologically Typical - 'Well-known parody of non-autistics by some people from the autism spectrum'
• Fresh Air with Terry Gross - 'National Public Radio (NPR) Program on Asperger's (May 5 2004)'
• Autism Asperger Publishing Company Independent Publisher specialising in books on Autism and Asperger syndrome

• Lists of further links

• Asperger and Autism organizations and services
• DMOZ category on Asperger's syndrome
• MedlinePlus Asperger Syndrome Resources
• AS Family Village library

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• Autism
• Eponymous medical terms
• Genetic disorders
• Neurological disorders
• Syndromes