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Wikipedia

Asperger syndrome

March 13, 2023

"Asperger's" redirects here. For other uses, see Asperger (disambiguation).

Asperger syndrome (AS), also known as Asperger's, is a neurodevelopmental condition characterized by significant difficulties in social interaction and nonverbal communication, along with restricted and repetitive patterns of behaviour and interests.[7] The syndrome is no longer recognised as a diagnosis in itself, having been merged with other conditions into autism spectrum disorder (ASD).[12][13][14] It was considered[15] to differ from other diagnoses that were merged into ASD by relatively unimpaired spoken language and intelligence.[16]

Asperger syndrome
Other namesAsperger's syndrome, Asperger disorder (AD), Asperger's, Sukhareva's syndrome,[1] schizoid disorder of childhood,[2] autistic psychopathy,[2] high-functioning autism,[3] level 1 autism spectrum disorder[4]
Restricted interests or repetitive behaviors may be features of Asperger's. This boy is playing with a magnetic construction toy.
Pronunciation
SpecialtyClinical psychology, psychiatry, pediatrics, occupational medicine
SymptomsProblems with social interaction and non-verbal communication, restricted interests, and repetitive behavior[7]
ComplicationsSocial isolation, employment problems, family stress, bullying, self-harm[8]
Usual onsetBefore two years old[7]
DurationLong-term[7]
CausesPoorly understood[7]
Diagnostic methodBased on the symptoms[9]
MedicationFor associated conditions[10]
Frequency37.2 million globally (0.5%) (2015)[11]

The syndrome was named after the Austrian pediatrician Hans Asperger, who, in 1944, described children in his care who struggled to form friendships, did not understand others' gestures or feelings, engaged in one-sided conversations about their favourite interests, and were clumsy.[17] In 1994, the diagnosis of Asperger's was included in the fourth edition (DSM-IV) of the American Diagnostic and Statistical Manual of Mental Disorders; however, with the publication of DSM-5 in 2013 the syndrome was removed, and the symptoms are now included within autism spectrum disorder along with classic autism and pervasive developmental disorder not otherwise specified (PDD-NOS).[7][18] It was similarly merged into autism spectrum disorder in the International Classification of Diseases (ICD-11) as of 2021.[19][20]

The exact cause of Asperger's is poorly understood.[7] While it has high heritability, the underlying genetics have not been determined conclusively.[21][22] Environmental factors are also believed to play a role.[7] Brain imaging has not identified a common underlying condition.[21] There is no single treatment, and the UK's National Health Service (NHS) guidelines suggest that 'treatment' of any form of autism should not be a goal, since autism is not 'a disease that can be removed or cured'.[23] According to the Royal College of Psychiatrists,[24] while co-occurring conditions might require treatment, 'management of autism itself is chiefly about the provision of the education, training and social support/care required to improve the person's ability to function in the everyday world'. The effectiveness of particular interventions for autism is supported by only limited data.[21] Interventions may include social skills training, cognitive behavioral therapy, physical therapy, speech therapy, parent training, and medications for associated problems, such as mood or anxiety.[10] Autistic characteristics tend to become less obvious in adulthood,[24] but social and communication difficulties usually persist.[25]

In 2015, Asperger's was estimated to affect 37.2 million people globally, or about 0.5% of the population.[11] The exact percentage of people affected has still not been firmly established.[21] Autism spectrum disorder is diagnosed in males more often than females,[26] and females are typically diagnosed at a later age.[27][28] The modern conception of Asperger syndrome came into existence in 1981, and went through a period of popularization.[29][30][31] It became a standardized diagnosis in the 1990s,[32] and was retired as a diagnosis in 2013.[14] Many questions and controversies about the condition remain.[25]

Classification

The extent of the overlap between Asperger syndrome and high-functioning autism (HFA – autism unaccompanied by intellectual disability) is unclear.[33][34][35] The ASD classification is to some extent an artifact of how autism was discovered,[36] and may not reflect the true nature of the spectrum;[37] methodological problems have beset Asperger syndrome as a valid diagnosis from the outset.[38][39] In the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), published in May 2013,[40] Asperger syndrome, as a separate diagnosis, was eliminated and folded into autism spectrum disorder.[41] Like the diagnosis of Asperger syndrome,[42] the change was controversial[42][43] and thus Asperger syndrome was subsequently not removed from the WHO's ICD-10;[44] however, it was removed from the ICD-11.[45]

The World Health Organization (WHO) previously defined Asperger syndrome (AS) as one of the autism spectrum disorders (ASD) or pervasive developmental disorders (PDD), which are a spectrum of psychological conditions that are characterized by abnormalities of social interaction and communication that pervade the individual's functioning, and by restricted and repetitive interests and behavior. Like other neurodevelopmental disorders, ASD begins in infancy or childhood, has a steady course without remission or relapse, and has impairments that result from maturation-related changes in various systems of the brain.[46]

Characteristics

 
People with Asperger syndrome often display restricted or specialized interests, such as this boy's interest in stacking cans.

As a pervasive developmental disorder, Asperger syndrome is distinguished by a pattern of symptoms rather than a single symptom. It is characterized by qualitative impairment in social interaction, by stereotyped and restricted patterns of behavior, activities, and interests, and by no clinically significant delay in cognitive development or general delay in language.[47] Intense preoccupation with a narrow subject, one-sided verbosity, restricted prosody, and physical clumsiness are typical of the condition, but are not required for diagnosis.[33] Suicidal behavior appears to occur at rates similar to those without ASD.[48]

Social interaction

Further information: Asperger syndrome and interpersonal relationships

A lack of demonstrated empathy affects aspects of social relatability for persons with Asperger syndrome.[49] Individuals with Asperger syndrome experience difficulties in basic elements of social interaction, which may include a failure to develop friendships or to seek shared enjoyments or achievements with others (e.g., showing others objects of interest); a lack of social or emotional reciprocity; and impaired nonverbal behaviors in areas such as eye contact, facial expression, posture, and gesture.[21]

People with Asperger syndrome may not be as withdrawn around others, compared with those with other forms of autism; they approach others, even if awkwardly. For example, a person with Asperger syndrome may engage in a one-sided, long-winded speech about a favorite topic, while misunderstanding or not recognizing the listener's feelings or reactions, such as a wish to change the topic of talk or end the interaction.[33] This social awkwardness has been called "active but odd".[21] Such failures to react appropriately to social interaction may appear as disregard for other people's feelings and may come across as rude or insensitive.[33] However, not all individuals with Asperger syndrome will approach others. Some may even display selective mutism, not speaking at all to most people and excessively to specific others. Some may choose only to talk to people they like.[50]

The cognitive ability of children with AS often allows them to articulate social norms in a laboratory context,[21] where they may be able to show a theoretical understanding of other people's emotions; however, they typically have difficulty acting on this knowledge in fluid, real-life situations.[33] People with AS may analyze and distill their observations of social interaction into rigid behavioral guidelines and apply these rules in awkward ways, such as forced eye contact, resulting in a demeanor that appears rigid or socially naïve. Childhood desire for companionship can become numbed through a history of failed social encounters.[21]

Violent or criminal behavior

The hypothesis that individuals with AS are predisposed to violent or criminal behavior has been investigated but is not supported by data.[51][52] More evidence suggests that children diagnosed with Asperger syndrome are more likely to be victims, rather than offenders.[53]

A 2008 review found that an overwhelming number of reported violent criminals with Asperger syndrome also had other coexisting psychotic psychiatric disorders such as schizoaffective disorder.[54] This coexistence of psychotic disorders is referred to as comorbid disorders. Comorbid disorders can be completely independent of one another or can have overlap in symptoms and how they express themselves.

Empathy

People with an Asperger profile might not be recognized for their empathetic qualities, due to variation in the ways empathy is felt and expressed. Some people feel deep empathy, but do not outwardly communicate these sentiments through facial expressions or language. Some people come to empathy through intellectual processes, using logic and reasoning to arrive at the feelings. It is also important to keep in mind that many people with Asperger profiles have been bullied or excluded by peers in the past and might therefore be guarded around people, which could appear as lack of empathy. People with Asperger profiles can be and are extremely caring individuals; in fact, it is particularly common for those with the profile to feel and exhibit deep concern for human welfare, animal rights, environmental protection, and other global and humanitarian causes.[55]

Evidence suggests that in the "double empathy problem model, autistic people have a unique interaction style which is significantly more readable by other autistic people, compared to non-autistic people."[56][57][58][59]

Restricted and repetitive interests and behavior

People with Asperger syndrome can display behavior, interests, and activities that are restricted and repetitive and are sometimes abnormally intense or focused. They may stick to inflexible routines, move in stereotyped and repetitive ways, preoccupy themselves with parts of objects, or engage in compulsive behaviors like lining objects up to form patterns.[47]

The pursuit of specific and narrow areas of interest is one of the most striking among possible features of AS.[21] Individuals with AS may collect volumes of detailed information on a relatively narrow topic such as weather data or star names without necessarily having a genuine understanding of the broader topic.[21][33] For example, a child might memorize camera model numbers while caring little about photography.[21] This behavior is usually apparent by age five or six.[21] Although these special interests may change from time to time, they typically become more unusual and narrowly focused and often dominate social interaction so much that the entire family may become immersed. Because narrow topics often capture the interest of children, this symptom may go unrecognized.[33]

Stereotyped and repetitive motor behaviors are a core part of the diagnosis of AS and other ASDs.[60] They include hand movements such as flapping or twisting, and complex whole-body movements.[47] These are typically repeated in longer bursts and look more voluntary or ritualistic than tics, which are usually faster, less rhythmical, and less often symmetrical.[61] However, in addition to this, various studies have reported a consistent comorbidity between AS and Tourette syndrome in the range of 8–20%,[61][62][63][64] with one figure as high as 80% for tics of some kind or another,[64] for which several explanations have been put forward, including common genetic factors and dopamine, glutamate, or serotonin abnormalities.[65]

According to the Adult Asperger Assessment (AAA) diagnostic test, a lack of interest in fiction and a positive preference towards non-fiction is common among adults with AS.[66]

Speech and language

Although individuals with Asperger syndrome acquire language skills without significant general delay and their speech typically lacks significant abnormalities, language acquisition and use is often atypical.[33] Abnormalities include verbosity; abrupt transitions; literal interpretations and miscomprehension of nuance; use of metaphor meaningful only to the speaker; auditory perception deficits; unusually pedantic, formal, or idiosyncratic speech; and oddities in loudness, pitch, intonation, prosody, and rhythm.[21] Echolalia has also been observed in individuals with AS.[67]

Three aspects of communication patterns are of clinical interest: poor prosody, tangential and circumstantial speech, and marked verbosity. Although inflection and intonation may be less rigid or monotonic than in classic autism, people with AS often have a limited range of intonation: speech may be unusually fast, jerky, or loud. Speech may convey a sense of incoherence; the conversational style often includes monologues about topics that bore the listener, fails to provide context for comments, or fails to suppress internal thoughts. Individuals with AS may fail to detect whether the listener is interested or engaged in the conversation. The speaker's conclusion or point may never be made, and attempts by the listener to elaborate on the speech's content or logic, or to shift to related topics, are often unsuccessful.[33]

Children with AS may have a sophisticated vocabulary at a young age and such children have often been colloquially called "little professors"[68] but have difficulty understanding figurative language and tend to use language literally.[21] Children with AS appear to have particular weaknesses in areas of nonliteral language that include humor, irony, teasing, and sarcasm. Although individuals with AS usually understand the cognitive basis of humor, they seem to lack understanding of the intent of humor to share the enjoyment with others.[34] Despite strong evidence of impaired humor appreciation, anecdotal reports of humor in individuals with AS seem to challenge some psychological theories of AS and autism.[69]

Motor and sensory perception

Individuals with Asperger syndrome may have signs or symptoms that are independent of the diagnosis but can affect the individual or the family.[70] These include differences in perception and problems with motor skills, sleep, and emotions.

Individuals with AS often have excellent auditory and visual perception.[71] Children with ASD often demonstrate enhanced perception of small changes in patterns such as arrangements of objects or well-known images; typically this is domain-specific and involves processing of fine-grained features.[72] Conversely, compared with individuals with high-functioning autism, individuals with AS have deficits in some tasks involving visual-spatial perception, auditory perception, or visual memory.[21] Many accounts of individuals with AS and ASD report other unusual sensory and perceptual skills and experiences. They may be unusually sensitive or insensitive to sound, light, and other stimuli;[73] these sensory responses are found in other developmental disorders and are not specific to AS or to ASD. There is little support for increased fight-or-flight response or failure of habituation in autism; there is more evidence of decreased responsiveness to sensory stimuli, although several studies show no differences.[74]

Hans Asperger's initial accounts[21] and other diagnostic schemes[75] include descriptions of physical clumsiness. Children with AS may be delayed in acquiring skills requiring dexterity, such as riding a bicycle or opening a jar, and may seem to move awkwardly or feel "uncomfortable in their own skin". They may be poorly coordinated or have an odd or bouncy gait or posture, poor handwriting, or problems with motor coordination.[21][33] They may show problems with proprioception (sensation of body position) on measures of developmental coordination disorder (motor planning disorder), balance, tandem gait, and finger-thumb apposition. There is no evidence that these motor skills problems differentiate AS from other high-functioning ASDs.[21]

Children with AS are more likely to have sleep problems, including difficulty in falling asleep, frequent nocturnal awakenings, and early morning awakenings.[76][77] AS is also associated with high levels of alexithymia, which is difficulty in identifying and describing one's emotions.[78] Although AS, lower sleep quality, and alexithymia are associated with each other, their causal relationship is unclear.[77]

Causes

Further information: Causes of autism

Hans Asperger described common traits among his patients' family members, especially fathers, and research supports this observation and suggests a genetic contribution to Asperger syndrome. Although no specific genetic factor has yet been identified, multiple factors are believed to play a role in the expression of autism, given the variability in symptoms seen in children.[21][79] Evidence for a genetic link is that AS tends to run in families where more family members have limited behavioral symptoms similar to AS (for example, some problems with social interaction, or with language and reading skills).[10] Most behavioral genetic research suggests that all autism spectrum disorders have shared genetic mechanisms.[21] There may be shared genes in which particular alleles make an individual vulnerable, and varying combinations result in differing severity and symptoms in each person with AS.[10]

A few ASD cases have been linked to exposure to teratogens (agents that cause birth defects) during the first eight weeks from conception. Although this does not exclude the possibility that ASD can be initiated or affected later, it is strong evidence that ASD arises very early in development.[80] Many environmental factors have been hypothesized to act after birth, but none has been confirmed by scientific investigation.[81]

Mechanism

Further information: Autism § Mechanism
 
Functional magnetic resonance imaging provides some evidence for mirror neuron theory.[82]

Asperger syndrome appears to result from developmental factors that affect many or all functional brain systems, as opposed to localized effects.[83]

Although the specific underpinnings of AS or factors that distinguish it from other ASDs are unknown, and no clear pathology common to individuals with AS has emerged,[21] it is still possible that AS's mechanism is separate from other ASDs.[84]

Neuroanatomical studies and the associations with teratogens strongly suggest that the mechanism includes alteration of brain development soon after conception.[80] Abnormal fetal development may affect the final structure and connectivity of the brain, resulting in altered neural circuits controlling thought and behavior.[85] Several theories of mechanism are available; none are likely to provide a complete explanation.[86]

General-processing theories

One general-processing theory is weak central coherence theory, which hypothesizes that a limited ability to see the big picture underlies the central disturbance in ASD.[87] A related theory—enhanced perceptual functioning—focuses more on the superiority of locally oriented and perceptual operations in autistic individuals.[88]

Mirror neuron system (MNS) theory

The mirror neuron system (MNS) theory hypothesizes that alterations to the development of the MNS interfere with imitation and lead to Asperger's core feature of social impairment.[82][89] One study found that activation is delayed in the core circuit for imitation in individuals with AS.[90] This theory maps well to social cognition theories like the theory of mind, which hypothesizes that autistic behavior arises from impairments in ascribing mental states to oneself and others;[91] or hyper-systemizing, which hypothesizes that autistic individuals can systematize internal operation to handle internal events but are less effective at empathizing when handling events generated by other agents.[92]

Diagnosis

Main article: Diagnosis of Asperger syndrome

Standard diagnostic criteria require impairment in social interaction and repetitive and stereotyped patterns of behavior, activities, and interests, without significant delay in language or cognitive development. Unlike the international standard,[46] the DSM-IV-TR criteria also required significant impairment in day-to-day functioning;[47] DSM-5 eliminated AS as a separate diagnosis in 2013, and folded it into the umbrella of autism spectrum disorders.[41] Other sets of diagnostic criteria have been proposed by Szatmari et al.[93] and by Gillberg and Gillberg.[94]

Diagnosis is most commonly made between the ages of four and eleven.[21] A comprehensive assessment involves a multidisciplinary team[10][49][95] that observes across multiple settings,[21] and includes neurological and genetic assessment as well as tests for cognition, psychomotor function, verbal and nonverbal strengths and weaknesses, style of learning, and skills for independent living.[10] The "gold standard" in diagnosing ASDs combines clinical judgment with the Autism Diagnostic Interview-Revised (ADI-R), a semistructured parent interview; and the Autism Diagnostic Observation Schedule (ADOS), a conversation and play-based interview with the child.[25] Delayed or mistaken diagnosis can be traumatic for individuals and families; for example, misdiagnosis can lead to medications that worsen behavior.[95][96]

Underdiagnosis and overdiagnosis may be problems. The cost and difficulty of screening and assessment can delay diagnosis. Conversely, the increasing popularity of drug treatment options and the expansion of benefits has motivated providers to overdiagnose ASD.[97] There are indications AS has been diagnosed more frequently in recent years, partly as a residual diagnosis for children of normal intelligence who are not autistic but have social difficulties.[98]

There are questions about the external validity of the AS diagnosis. That is, it is unclear whether there is a practical benefit in distinguishing AS from HFA or PDD-NOS;[98] different screening tools may render different diagnoses for the same person.[10]

Differential diagnosis

Many children with AS are initially misdiagnosed with attention deficit hyperactivity disorder (ADHD).[21] Diagnosing adults is more challenging, as standard diagnostic criteria are designed for children and the expression of AS changes with age.[99][100] Adult diagnosis requires painstaking clinical examination and thorough medical history gained from both the individual and other people who know the person, focusing on childhood behavior.[66]

Conditions that must be considered in a differential diagnosis along with ADHD include other ASDs, the schizophrenia spectrum, personality disorders, obsessive–compulsive disorder, major depressive disorder, semantic pragmatic disorder, nonverbal learning disorder, social anxiety disorder,[95][99] Tourette syndrome,[61] stereotypic movement disorder, bipolar disorder,[79] social-cognitive deficits due to brain damage from alcohol use disorder,[101] and obsessive–compulsive personality disorder (OCPD).[64][102]

Screening

Parents of children with Asperger syndrome can typically trace differences in their children's development to as early as 30 months of age.[79] Developmental screening during a routine check-up by a general practitioner or pediatrician may identify signs that warrant further investigation.[10][21] The United States Preventive Services Task Force in 2016 found it was unclear if screening was beneficial or harmful among children in whom there are no concerns.[103]

Different screening instruments are used to diagnose AS,[10][75] including the Asperger Syndrome Diagnostic Scale (ASDS); Autism Spectrum Screening Questionnaire (ASSQ); Childhood Autism Spectrum Test (CAST), previously called the Childhood Asperger Syndrome Test;[104] Gilliam Asperger's disorder scale (GADS); Krug Asperger's Disorder Index (KADI);[105] and the autism-spectrum quotient (AQ), with versions for children,[106] adolescents,[107] and adults.[108] None have been shown to reliably differentiate between AS and other ASDs.[21]

Management

Further information: Autism therapies

Treatment attempts to manage distressing symptoms and to teach age-appropriate social, communication, and vocational skills that are not naturally acquired during development.[21] Intervention is tailored to the needs of the individual based on multidisciplinary assessment.[109] Although progress has been made, data supporting the efficacy of particular interventions are limited.[21][110]

Therapies

Managing AS ideally involves multiple therapies that address core symptoms of the disorder. While most professionals agree that the earlier the intervention, the better, there is no treatment combination that is recommended above others.[10] AS treatment resembles that of other high-functioning ASDs, except that it takes into account the linguistic capabilities, verbal strengths, and nonverbal vulnerabilities of individuals with AS.[21] A typical program generally includes:[10]

Of the many studies on behavior-based early intervention programs, most are case reports of up to five participants and typically examine a few problem behaviors such as self-injury, aggression, noncompliance, stereotypies, or spontaneous language; unintended side effects are largely ignored.[115] Despite the popularity of social skills training, its effectiveness is not firmly established.[116] A randomized controlled study of a model for training parents in problem behaviors in their children with AS showed that parents attending a one-day workshop or six individual lessons reported fewer behavioral problems, while parents receiving the individual lessons reported less intense behavioral problems in their AS children.[117] Vocational training is important to teach job interview etiquette and workplace behavior to older children and adults with AS, and organization software and personal data assistants can improve the work and life management of people with AS.[21]

Medications

No medications directly treat the core symptoms of AS.[113] Although research into the efficacy of pharmaceutical intervention for AS is limited,[21] it is essential to diagnose and treat comorbid conditions.[49] Deficits in self-identifying emotions or in observing effects of one's behavior on others can make it difficult for individuals with AS to see why medication may be appropriate.[113] Medication can be effective in combination with behavioral interventions and environmental accommodations in treating comorbid symptoms such as anxiety disorders, major depressive disorder, inattention, and aggression.[21] The atypical antipsychotic medications risperidone, olanzapine and aripiprazole have been shown to reduce the associated symptoms of AS;[21][118][119] risperidone can reduce repetitive and self-injurious behaviors, aggressive outbursts, and impulsivity, and improve stereotypical patterns of behavior and social relatedness. The selective serotonin reuptake inhibitors (SSRIs) fluoxetine, fluvoxamine, and sertraline have been effective in treating restricted and repetitive interests and behaviors,[21][49][79] while stimulant medication, such as methylphenidate, can reduce inattention.[120]

Care must be taken with medications, as side effects may be more common and harder to evaluate in individuals with AS, and tests of drugs' effectiveness against comorbid conditions routinely exclude individuals from the autism spectrum.[113] Abnormalities in metabolism, cardiac conduction times, and an increased risk of type 2 diabetes have been raised as concerns with antipsychotic medications,[121][122] along with serious long-term neurological side effects.[115] SSRIs can lead to manifestations of behavioral activation such as increased impulsivity, aggression, and sleep disturbance.[79] Weight gain and fatigue are commonly reported side effects of risperidone, which may also lead to increased risk for extrapyramidal symptoms such as restlessness and dystonia[79] and increased serum prolactin levels.[123] Sedation and weight gain are more common with olanzapine,[122] which has also been linked with diabetes.[121] Sedative side-effects in school-age children[124] have ramifications for classroom learning. Individuals with AS may be unable to identify and communicate their internal moods and emotions or to tolerate side effects that for most people would not be problematic.[125]

Prognosis

There is some evidence that children with AS may see a lessening of symptoms; up to 20% of children may no longer meet the diagnostic criteria as adults, although social and communication difficulties may persist.[25] As of 2006, no studies addressing the long-term outcome of individuals with Asperger syndrome are available and there are no systematic long-term follow-up studies of children with AS.[33] Individuals with AS appear to have normal life expectancy, but have an increased prevalence of comorbid psychiatric conditions, such as major depressive disorder and anxiety disorders that may significantly affect prognosis.[21][25] Although social impairment may be lifelong, the outcome is generally more positive than with individuals with lower-functioning autism spectrum disorders;[21] for example, ASD symptoms are more likely to diminish with time in children with AS or HFA.[126] Most students with AS and HFA have average mathematical ability and test slightly worse in mathematics than in general intelligence.[127] However, mathematicians are at least three times more likely to have autism-spectrum traits than the general population, and are more likely to have family members with autism.[128]

Although many attend regular education classes, some children with AS may attend special education classes such as separate classroom and resource room because of their social and behavioral difficulties.[33] Adolescents with AS may exhibit ongoing difficulty with self-care or organization, and disturbances in social and romantic relationships. Despite high cognitive potential, most young adults with AS remain at home, yet some do marry and work independently.[21] The "different-ness" adolescents experience can be traumatic.[129] Anxiety may stem from preoccupation over possible violations of routines and rituals, from being placed in a situation without a clear schedule or expectations, or from concern with failing in social encounters;[21] the resulting stress may manifest as inattention, withdrawal, reliance on obsessions, hyperactivity, or aggressive or oppositional behavior.[112] Depression is often the result of chronic frustration from repeated failure to engage others socially, and mood disorders requiring treatment may develop.[21] Clinical experience suggests the rate of suicide may be higher among those with AS, but this has not been confirmed by systematic empirical studies.[130]

Education of families is critical in developing strategies for understanding strengths and weaknesses;[49] helping the family to cope improves outcomes in children.[53] Prognosis may be improved by diagnosis at a younger age that allows for early interventions, while interventions in adulthood are valuable but less beneficial.[49] There are legal implications for individuals with AS as they run the risk of exploitation by others and may be unable to comprehend the societal implications of their actions.[49]

Epidemiology

Main article: Epidemiology of autism

Frequency estimates vary enormously. In 2015, it was estimated that 37.2 million people globally are affected.[11] A 2003 review of epidemiological studies of children found autism rates ranging from 0.03 to 4.84 per 1,000, with the ratio of autism to Asperger syndrome ranging from 1.5:1 to 16:1;[131] combining the geometric mean ratio of 5:1 with a conservative prevalence estimate for autism of 1.3 per 1,000 suggests indirectly that the prevalence of AS might be around 0.26 per 1,000.[132] Part of the variance in estimates arises from differences in diagnostic criteria. For example, a relatively small 2007 study of 5,484 eight-year-old children in Finland found 2.9 children per 1,000 met the ICD-10 criteria for an AS diagnosis, 2.7 per 1,000 for Gillberg and Gillberg criteria, 2.5 for DSM-IV, 1.6 for Szatmari et al., and 4.3 per 1,000 for the union of the four criteria. Boys seem to be more likely to have AS than girls; estimates of the sex ratio range from 1.6:1 to 4:1, using the Gillberg and Gillberg criteria.[133] Females with autism spectrum disorders may be underdiagnosed.[134]

Comorbidities

Main article: Conditions comorbid to autism spectrum disorders

Anxiety disorders and major depressive disorder are the most common conditions seen at the same time; comorbidity of these in persons with AS is estimated at 65%.[21] Reports have associated AS with medical conditions such as aminoaciduria and ligamentous laxity, but these have been case reports or small studies and no factors have been associated with AS across studies.[21] One study of males with AS found an increased rate of epilepsy and a high rate (51%) of nonverbal learning disorder.[135] AS is associated with tics, Tourette syndrome and bipolar disorder. The repetitive behaviors of AS have many similarities with the symptoms of obsessive–compulsive disorder and obsessive–compulsive personality disorder,[64] and 26% of a sample of young adults with AS were found to meet the criteria for schizoid personality disorder (which is characterised by severe social seclusion and emotional detachment), more than any other personality disorder in the sample.[136][137][138] However many of these studies are based on clinical samples or lack standardized measures; nonetheless, comorbid conditions are relatively common.[25]

History

Main article: History of Asperger syndrome

Named after the Austrian pediatrician Hans Asperger (1906–1980), Asperger syndrome is a relatively new diagnosis in the field of autism,[139] though a syndrome like it was described as early as 1925 by Soviet child psychiatrist Grunya Sukhareva (1891–1981),[140] leading some of those diagnosed with Asperger's Syndrome to instead refer to their condition as 'Sukhareva's Syndrome', in opposition to Hans Asperger's association with Nazism.[1] As a child, Asperger appears to have exhibited some features of the very condition named after him, such as remoteness and talent in language.[141][142] In 1944, Asperger described four children in his practice[49] who had difficulty in integrating themselves socially and showing empathy towards peers. They also lacked nonverbal communication skills and were physically clumsy. Asperger described this "autistic psychopathy" as social isolation.[10] Fifty years later, several standardizations of AS as a medical diagnosis were tentatively proposed, many of which diverge significantly from Asperger's original work.[143]

Unlike today's AS, autistic psychopathy could be found in people of all levels of intelligence, including those with intellectual disability.[144] Asperger defended the value of so-called "high-functioning" autistic individuals, writing: "We are convinced, then, that autistic people have their place in the organism of the social community. They fulfill their role well, perhaps better than anyone else could, and we are talking of people who as children had the greatest difficulties and caused untold worries to their care-givers."[17] Asperger also believed some would be capable of exceptional achievement and original thought later in life.[49]

Asperger's paper was published during World War II and in German, so it was not widely read elsewhere. Lorna Wing used the term Asperger syndrome in 1976,[145] and popularized it to the English-speaking medical community in her February 1981 publication[146][147][148] of case studies of children showing the symptoms described by Asperger,[139] and Uta Frith translated his paper to English in 1991.[17] Sets of diagnostic criteria were outlined by Gillberg and Gillberg in 1989 and by Szatmari et al. in the same year.[133] In 1992, AS became a standard diagnosis when it was included in the tenth edition of the World Health Organization's diagnostic manual, International Classification of Diseases (ICD-10). It was added to the fourth edition of the American Psychiatric Association's diagnostic reference, Diagnostic and Statistical Manual of Mental Disorders (DSM-IV), published in 1994.[10]

Hundreds of books, articles, and websites now describe AS and prevalence estimates have increased dramatically for ASD, with AS recognized as an important subgroup.[139] Whether it should be seen as distinct from high-functioning autism is a fundamental issue requiring further study,[49] and there are questions about the empirical validation of the DSM-IV and ICD-10 criteria.[33] In 2013, DSM-5 eliminated AS as a separate diagnosis, folding it into the autism spectrum on a severity scale.[41]

Society and culture

See also: Societal and cultural aspects of autism and Disability rights movement
 
Students and families walk to support Autism Awareness Month.

People identifying with Asperger syndrome may refer to themselves in casual conversation as aspies (a term first used in print in the Boston Globe in 1998).[149][150] Some autistic people have advocated 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 promote tolerance of neurodiversity.[151] These views are the basis for the autistic rights and autistic pride movements.[152] There is a contrast between the attitude of people with AS, who typically do not want to be cured and are proud of their identity; and parents of children with AS, who typically seek assistance and a cure for their children.[153]

Some researchers have argued that AS can be viewed as a different cognitive style, not a disorder,[154] and that it should be removed from the standard Diagnostic and Statistical Manual, much as homosexuality was removed.[155] In a 2002 paper, Simon Baron-Cohen wrote of those with AS: "In the social world, there is no great benefit to a precise eye for detail, but in the worlds of maths, computing, cataloging, music, linguistics, engineering, and science, such an eye for detail can lead to success rather than failure." Baron-Cohen cited two reasons why it might still be useful to consider AS to be a disability: to ensure provision for legally required special support, and to recognize emotional difficulties from reduced empathy.[156] Baron-Cohen argues that the genes for ASD's combination of abilities have operated throughout recent human evolution and have made remarkable contributions to human history.[157]

By contrast, Pier Jaarsma and Welin wrote in 2011 that the "broad version of the neurodiversity claim, covering low-functioning as well as high-functioning autism, is problematic. Only a narrow conception of neurodiversity, referring exclusively to high-functioning autists, is reasonable."[158] They say that "higher functioning" individuals with autism may "not [be] benefited with such a psychiatric defect-based diagnosis ... some of them are being harmed by it, because of the disrespect the diagnosis displays for their natural way of being", but "think that it is still reasonable to include other categories of autism in the psychiatric diagnostics. The narrow conception of the neurodiversity claim should be accepted but the broader claim should not."[158] Jonathan Mitchell, an autistic author and blogger who advocates a cure for autism, has described autism as having "prevented me from making a living or ever having a girlfriend. It's given me bad fine motor coordination problems where I can hardly write. I have an impaired ability to relate to people. I can't concentrate or get things done."[159] He describes neurodiversity as a "tempting escape valve".[160]

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Further reading

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  • Autistic Empire, Are you Autistic? Take the test – an online version of the Adult Asperger's Assessment developed by Cohen, S. et al. (2005) (see Woodbury-Smith MR, "Screening adults for Asperger Syndrome using the AQ: a preliminary study of its diagnostic validity in clinical practice", in §References).
  • Hus, Vanessa; Lord, Catherine (2014). "The Autism Diagnostic Observation Schedule, Module 4: Revised Algorithm and Standardized Severity Scores". Journal of Autism and Developmental Disorders. 44 (8): 1996–2012. doi:10.1007/s10803-014-2080-3. PMC 4104252. PMID 24590409. A public paper re-calibrating the Autism Diagnostic Observation Schedule for appropriate assessment of autistic adults, who typically score lower on measures of impairment than autistic children due to compensatory strategies.
  • Royal College of Psychiatrists (2017), Interview Guide for the Diagnostic Assessment of Able Adults with Autistic Spectrum Disorder – based on the Autism Diagnostic Interview-Revised (ADI-R)

March 13, 2023
asperger, syndrome, asperger, redirects, here, other, uses, asperger, disambiguation, this, article, needs, updated, reason, given, some, parts, article, accurately, reflect, either, criteria, please, help, update, this, article, reflect, recent, events, newly. Asperger s redirects here For other uses see Asperger disambiguation This article needs to be updated The reason given is some parts of the article do not accurately reflect either the new DSM 5 or ICD 11 criteria Please help update this article to reflect recent events or newly available information February 2022 Asperger syndrome AS also known as Asperger s is a neurodevelopmental condition characterized by significant difficulties in social interaction and nonverbal communication along with restricted and repetitive patterns of behaviour and interests 7 The syndrome is no longer recognised as a diagnosis in itself having been merged with other conditions into autism spectrum disorder ASD 12 13 14 It was considered 15 to differ from other diagnoses that were merged into ASD by relatively unimpaired spoken language and intelligence 16 Asperger syndromeOther namesAsperger s syndrome Asperger disorder AD Asperger s Sukhareva s syndrome 1 schizoid disorder of childhood 2 autistic psychopathy 2 high functioning autism 3 level 1 autism spectrum disorder 4 Restricted interests or repetitive behaviors may be features of Asperger s This boy is playing with a magnetic construction toy Pronunciation ˈ ae s p ɜːr ɡ er z 5 dʒ er z 6 SpecialtyClinical psychology psychiatry pediatrics occupational medicineSymptomsProblems with social interaction and non verbal communication restricted interests and repetitive behavior 7 ComplicationsSocial isolation employment problems family stress bullying self harm 8 Usual onsetBefore two years old 7 DurationLong term 7 CausesPoorly understood 7 Diagnostic methodBased on the symptoms 9 MedicationFor associated conditions 10 Frequency37 2 million globally 0 5 2015 11 The syndrome was named after the Austrian pediatrician Hans Asperger who in 1944 described children in his care who struggled to form friendships did not understand others gestures or feelings engaged in one sided conversations about their favourite interests and were clumsy 17 In 1994 the diagnosis of Asperger s was included in the fourth edition DSM IV of the American Diagnostic and Statistical Manual of Mental Disorders however with the publication of DSM 5 in 2013 the syndrome was removed and the symptoms are now included within autism spectrum disorder along with classic autism and pervasive developmental disorder not otherwise specified PDD NOS 7 18 It was similarly merged into autism spectrum disorder in the International Classification of Diseases ICD 11 as of 2021 update 19 20 The exact cause of Asperger s is poorly understood 7 While it has high heritability the underlying genetics have not been determined conclusively 21 22 Environmental factors are also believed to play a role 7 Brain imaging has not identified a common underlying condition 21 There is no single treatment and the UK s National Health Service NHS guidelines suggest that treatment of any form of autism should not be a goal since autism is not a disease that can be removed or cured 23 According to the Royal College of Psychiatrists 24 while co occurring conditions might require treatment management of autism itself is chiefly about the provision of the education training and social support care required to improve the person s ability to function in the everyday world The effectiveness of particular interventions for autism is supported by only limited data 21 Interventions may include social skills training cognitive behavioral therapy physical therapy speech therapy parent training and medications for associated problems such as mood or anxiety 10 Autistic characteristics tend to become less obvious in adulthood 24 but social and communication difficulties usually persist 25 In 2015 Asperger s was estimated to affect 37 2 million people globally or about 0 5 of the population 11 The exact percentage of people affected has still not been firmly established 21 Autism spectrum disorder is diagnosed in males more often than females 26 and females are typically diagnosed at a later age 27 28 The modern conception of Asperger syndrome came into existence in 1981 and went through a period of popularization 29 30 31 It became a standardized diagnosis in the 1990s 32 and was retired as a diagnosis in 2013 14 Many questions and controversies about the condition remain 25 Contents 1 Classification 2 Characteristics 2 1 Social interaction 2 1 1 Violent or criminal behavior 2 2 Empathy 2 3 Restricted and repetitive interests and behavior 2 4 Speech and language 2 5 Motor and sensory perception 3 Causes 4 Mechanism 4 1 General processing theories 4 2 Mirror neuron system MNS theory 5 Diagnosis 5 1 Differential diagnosis 6 Screening 7 Management 7 1 Therapies 7 2 Medications 8 Prognosis 9 Epidemiology 9 1 Comorbidities 10 History 11 Society and culture 12 References 13 Further readingClassificationThe extent of the overlap between Asperger syndrome and high functioning autism HFA autism unaccompanied by intellectual disability is unclear 33 34 35 The ASD classification is to some extent an artifact of how autism was discovered 36 and may not reflect the true nature of the spectrum 37 methodological problems have beset Asperger syndrome as a valid diagnosis from the outset 38 39 In the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders DSM 5 published in May 2013 40 Asperger syndrome as a separate diagnosis was eliminated and folded into autism spectrum disorder 41 Like the diagnosis of Asperger syndrome 42 the change was controversial 42 43 and thus Asperger syndrome was subsequently not removed from the WHO s ICD 10 44 however it was removed from the ICD 11 45 The World Health Organization WHO previously defined Asperger syndrome AS as one of the autism spectrum disorders ASD or pervasive developmental disorders PDD which are a spectrum of psychological conditions that are characterized by abnormalities of social interaction and communication that pervade the individual s functioning and by restricted and repetitive interests and behavior Like other neurodevelopmental disorders ASD begins in infancy or childhood has a steady course without remission or relapse and has impairments that result from maturation related changes in various systems of the brain 46 Characteristics People with Asperger syndrome often display restricted or specialized interests such as this boy s interest in stacking cans As a pervasive developmental disorder Asperger syndrome is distinguished by a pattern of symptoms rather than a single symptom It is characterized by qualitative impairment in social interaction by stereotyped and restricted patterns of behavior activities and interests and by no clinically significant delay in cognitive development or general delay in language 47 Intense preoccupation with a narrow subject one sided verbosity restricted prosody and physical clumsiness are typical of the condition but are not required for diagnosis 33 Suicidal behavior appears to occur at rates similar to those without ASD 48 Social interaction Further information Asperger syndrome and interpersonal relationships A lack of demonstrated empathy affects aspects of social relatability for persons with Asperger syndrome 49 Individuals with Asperger syndrome experience difficulties in basic elements of social interaction which may include a failure to develop friendships or to seek shared enjoyments or achievements with others e g showing others objects of interest a lack of social or emotional reciprocity and impaired nonverbal behaviors in areas such as eye contact facial expression posture and gesture 21 People with Asperger syndrome may not be as withdrawn around others compared with those with other forms of autism they approach others even if awkwardly For example a person with Asperger syndrome may engage in a one sided long winded speech about a favorite topic while misunderstanding or not recognizing the listener s feelings or reactions such as a wish to change the topic of talk or end the interaction 33 This social awkwardness has been called active but odd 21 Such failures to react appropriately to social interaction may appear as disregard for other people s feelings and may come across as rude or insensitive 33 However not all individuals with Asperger syndrome will approach others Some may even display selective mutism not speaking at all to most people and excessively to specific others Some may choose only to talk to people they like 50 The cognitive ability of children with AS often allows them to articulate social norms in a laboratory context 21 where they may be able to show a theoretical understanding of other people s emotions however they typically have difficulty acting on this knowledge in fluid real life situations 33 People with AS may analyze and distill their observations of social interaction into rigid behavioral guidelines and apply these rules in awkward ways such as forced eye contact resulting in a demeanor that appears rigid or socially naive Childhood desire for companionship can become numbed through a history of failed social encounters 21 Violent or criminal behavior The hypothesis that individuals with AS are predisposed to violent or criminal behavior has been investigated but is not supported by data 51 52 More evidence suggests that children diagnosed with Asperger syndrome are more likely to be victims rather than offenders 53 A 2008 review found that an overwhelming number of reported violent criminals with Asperger syndrome also had other coexisting psychotic psychiatric disorders such as schizoaffective disorder 54 This coexistence of psychotic disorders is referred to as comorbid disorders Comorbid disorders can be completely independent of one another or can have overlap in symptoms and how they express themselves Empathy People with an Asperger profile might not be recognized for their empathetic qualities due to variation in the ways empathy is felt and expressed Some people feel deep empathy but do not outwardly communicate these sentiments through facial expressions or language Some people come to empathy through intellectual processes using logic and reasoning to arrive at the feelings It is also important to keep in mind that many people with Asperger profiles have been bullied or excluded by peers in the past and might therefore be guarded around people which could appear as lack of empathy People with Asperger profiles can be and are extremely caring individuals in fact it is particularly common for those with the profile to feel and exhibit deep concern for human welfare animal rights environmental protection and other global and humanitarian causes 55 Evidence suggests that in the double empathy problem model autistic people have a unique interaction style which is significantly more readable by other autistic people compared to non autistic people 56 57 58 59 Restricted and repetitive interests and behavior People with Asperger syndrome can display behavior interests and activities that are restricted and repetitive and are sometimes abnormally intense or focused They may stick to inflexible routines move in stereotyped and repetitive ways preoccupy themselves with parts of objects or engage in compulsive behaviors like lining objects up to form patterns 47 The pursuit of specific and narrow areas of interest is one of the most striking among possible features of AS 21 Individuals with AS may collect volumes of detailed information on a relatively narrow topic such as weather data or star names without necessarily having a genuine understanding of the broader topic 21 33 For example a child might memorize camera model numbers while caring little about photography 21 This behavior is usually apparent by age five or six 21 Although these special interests may change from time to time they typically become more unusual and narrowly focused and often dominate social interaction so much that the entire family may become immersed Because narrow topics often capture the interest of children this symptom may go unrecognized 33 Stereotyped and repetitive motor behaviors are a core part of the diagnosis of AS and other ASDs 60 They include hand movements such as flapping or twisting and complex whole body movements 47 These are typically repeated in longer bursts and look more voluntary or ritualistic than tics which are usually faster less rhythmical and less often symmetrical 61 However in addition to this various studies have reported a consistent comorbidity between AS and Tourette syndrome in the range of 8 20 61 62 63 64 with one figure as high as 80 for tics of some kind or another 64 for which several explanations have been put forward including common genetic factors and dopamine glutamate or serotonin abnormalities 65 According to the Adult Asperger Assessment AAA diagnostic test a lack of interest in fiction and a positive preference towards non fiction is common among adults with AS 66 Speech and language Although individuals with Asperger syndrome acquire language skills without significant general delay and their speech typically lacks significant abnormalities language acquisition and use is often atypical 33 Abnormalities include verbosity abrupt transitions literal interpretations and miscomprehension of nuance use of metaphor meaningful only to the speaker auditory perception deficits unusually pedantic formal or idiosyncratic speech and oddities in loudness pitch intonation prosody and rhythm 21 Echolalia has also been observed in individuals with AS 67 Three aspects of communication patterns are of clinical interest poor prosody tangential and circumstantial speech and marked verbosity Although inflection and intonation may be less rigid or monotonic than in classic autism people with AS often have a limited range of intonation speech may be unusually fast jerky or loud Speech may convey a sense of incoherence the conversational style often includes monologues about topics that bore the listener fails to provide context for comments or fails to suppress internal thoughts Individuals with AS may fail to detect whether the listener is interested or engaged in the conversation The speaker s conclusion or point may never be made and attempts by the listener to elaborate on the speech s content or logic or to shift to related topics are often unsuccessful 33 Children with AS may have a sophisticated vocabulary at a young age and such children have often been colloquially called little professors 68 but have difficulty understanding figurative language and tend to use language literally 21 Children with AS appear to have particular weaknesses in areas of nonliteral language that include humor irony teasing and sarcasm Although individuals with AS usually understand the cognitive basis of humor they seem to lack understanding of the intent of humor to share the enjoyment with others 34 Despite strong evidence of impaired humor appreciation anecdotal reports of humor in individuals with AS seem to challenge some psychological theories of AS and autism 69 Motor and sensory perception Individuals with Asperger syndrome may have signs or symptoms that are independent of the diagnosis but can affect the individual or the family 70 These include differences in perception and problems with motor skills sleep and emotions Individuals with AS often have excellent auditory and visual perception 71 Children with ASD often demonstrate enhanced perception of small changes in patterns such as arrangements of objects or well known images typically this is domain specific and involves processing of fine grained features 72 Conversely compared with individuals with high functioning autism individuals with AS have deficits in some tasks involving visual spatial perception auditory perception or visual memory 21 Many accounts of individuals with AS and ASD report other unusual sensory and perceptual skills and experiences They may be unusually sensitive or insensitive to sound light and other stimuli 73 these sensory responses are found in other developmental disorders and are not specific to AS or to ASD There is little support for increased fight or flight response or failure of habituation in autism there is more evidence of decreased responsiveness to sensory stimuli although several studies show no differences 74 Hans Asperger s initial accounts 21 and other diagnostic schemes 75 include descriptions of physical clumsiness Children with AS may be delayed in acquiring skills requiring dexterity such as riding a bicycle or opening a jar and may seem to move awkwardly or feel uncomfortable in their own skin They may be poorly coordinated or have an odd or bouncy gait or posture poor handwriting or problems with motor coordination 21 33 They may show problems with proprioception sensation of body position on measures of developmental coordination disorder motor planning disorder balance tandem gait and finger thumb apposition There is no evidence that these motor skills problems differentiate AS from other high functioning ASDs 21 Children with AS are more likely to have sleep problems including difficulty in falling asleep frequent nocturnal awakenings and early morning awakenings 76 77 AS is also associated with high levels of alexithymia which is difficulty in identifying and describing one s emotions 78 Although AS lower sleep quality and alexithymia are associated with each other their causal relationship is unclear 77 CausesFurther information Causes of autism Hans Asperger described common traits among his patients family members especially fathers and research supports this observation and suggests a genetic contribution to Asperger syndrome Although no specific genetic factor has yet been identified multiple factors are believed to play a role in the expression of autism given the variability in symptoms seen in children 21 79 Evidence for a genetic link is that AS tends to run in families where more family members have limited behavioral symptoms similar to AS for example some problems with social interaction or with language and reading skills 10 Most behavioral genetic research suggests that all autism spectrum disorders have shared genetic mechanisms 21 There may be shared genes in which particular alleles make an individual vulnerable and varying combinations result in differing severity and symptoms in each person with AS 10 A few ASD cases have been linked to exposure to teratogens agents that cause birth defects during the first eight weeks from conception Although this does not exclude the possibility that ASD can be initiated or affected later it is strong evidence that ASD arises very early in development 80 Many environmental factors have been hypothesized to act after birth but none has been confirmed by scientific investigation 81 MechanismFurther information Autism Mechanism Functional magnetic resonance imaging provides some evidence for mirror neuron theory 82 Asperger syndrome appears to result from developmental factors that affect many or all functional brain systems as opposed to localized effects 83 Although the specific underpinnings of AS or factors that distinguish it from other ASDs are unknown and no clear pathology common to individuals with AS has emerged 21 it is still possible that AS s mechanism is separate from other ASDs 84 Neuroanatomical studies and the associations with teratogens strongly suggest that the mechanism includes alteration of brain development soon after conception 80 Abnormal fetal development may affect the final structure and connectivity of the brain resulting in altered neural circuits controlling thought and behavior 85 Several theories of mechanism are available none are likely to provide a complete explanation 86 General processing theories One general processing theory is weak central coherence theory which hypothesizes that a limited ability to see the big picture underlies the central disturbance in ASD 87 A related theory enhanced perceptual functioning focuses more on the superiority of locally oriented and perceptual operations in autistic individuals 88 Mirror neuron system MNS theory This section s factual accuracy may be compromised due to out of date information The reason given is There have been almost 4 decades since some of the material cited here was published and current consensus in ASD is less straightforward than depicted here Please help update this article to reflect recent events or newly available information January 2022 The mirror neuron system MNS theory hypothesizes that alterations to the development of the MNS interfere with imitation and lead to Asperger s core feature of social impairment 82 89 One study found that activation is delayed in the core circuit for imitation in individuals with AS 90 This theory maps well to social cognition theories like the theory of mind which hypothesizes that autistic behavior arises from impairments in ascribing mental states to oneself and others 91 or hyper systemizing which hypothesizes that autistic individuals can systematize internal operation to handle internal events but are less effective at empathizing when handling events generated by other agents 92 DiagnosisMain article Diagnosis of Asperger syndrome Standard diagnostic criteria require impairment in social interaction and repetitive and stereotyped patterns of behavior activities and interests without significant delay in language or cognitive development Unlike the international standard 46 the DSM IV TR criteria also required significant impairment in day to day functioning 47 DSM 5 eliminated AS as a separate diagnosis in 2013 and folded it into the umbrella of autism spectrum disorders 41 Other sets of diagnostic criteria have been proposed by Szatmari et al 93 and by Gillberg and Gillberg 94 Diagnosis is most commonly made between the ages of four and eleven 21 A comprehensive assessment involves a multidisciplinary team 10 49 95 that observes across multiple settings 21 and includes neurological and genetic assessment as well as tests for cognition psychomotor function verbal and nonverbal strengths and weaknesses style of learning and skills for independent living 10 The gold standard in diagnosing ASDs combines clinical judgment with the Autism Diagnostic Interview Revised ADI R a semistructured parent interview and the Autism Diagnostic Observation Schedule ADOS a conversation and play based interview with the child 25 Delayed or mistaken diagnosis can be traumatic for individuals and families for example misdiagnosis can lead to medications that worsen behavior 95 96 Underdiagnosis and overdiagnosis may be problems The cost and difficulty of screening and assessment can delay diagnosis Conversely the increasing popularity of drug treatment options and the expansion of benefits has motivated providers to overdiagnose ASD 97 There are indications AS has been diagnosed more frequently in recent years partly as a residual diagnosis for children of normal intelligence who are not autistic but have social difficulties 98 There are questions about the external validity of the AS diagnosis That is it is unclear whether there is a practical benefit in distinguishing AS from HFA or PDD NOS 98 different screening tools may render different diagnoses for the same person 10 Differential diagnosis Many children with AS are initially misdiagnosed with attention deficit hyperactivity disorder ADHD 21 Diagnosing adults is more challenging as standard diagnostic criteria are designed for children and the expression of AS changes with age 99 100 Adult diagnosis requires painstaking clinical examination and thorough medical history gained from both the individual and other people who know the person focusing on childhood behavior 66 Conditions that must be considered in a differential diagnosis along with ADHD include other ASDs the schizophrenia spectrum personality disorders obsessive compulsive disorder major depressive disorder semantic pragmatic disorder nonverbal learning disorder social anxiety disorder 95 99 Tourette syndrome 61 stereotypic movement disorder bipolar disorder 79 social cognitive deficits due to brain damage from alcohol use disorder 101 and obsessive compulsive personality disorder OCPD 64 102 ScreeningParents of children with Asperger syndrome can typically trace differences in their children s development to as early as 30 months of age 79 Developmental screening during a routine check up by a general practitioner or pediatrician may identify signs that warrant further investigation 10 21 The United States Preventive Services Task Force in 2016 found it was unclear if screening was beneficial or harmful among children in whom there are no concerns 103 Different screening instruments are used to diagnose AS 10 75 including the Asperger Syndrome Diagnostic Scale ASDS Autism Spectrum Screening Questionnaire ASSQ Childhood Autism Spectrum Test CAST previously called the Childhood Asperger Syndrome Test 104 Gilliam Asperger s disorder scale GADS Krug Asperger s Disorder Index KADI 105 and the autism spectrum quotient AQ with versions for children 106 adolescents 107 and adults 108 None have been shown to reliably differentiate between AS and other ASDs 21 ManagementFurther information Autism therapies Treatment attempts to manage distressing symptoms and to teach age appropriate social communication and vocational skills that are not naturally acquired during development 21 Intervention is tailored to the needs of the individual based on multidisciplinary assessment 109 Although progress has been made data supporting the efficacy of particular interventions are limited 21 110 Therapies Managing AS ideally involves multiple therapies that address core symptoms of the disorder While most professionals agree that the earlier the intervention the better there is no treatment combination that is recommended above others 10 AS treatment resembles that of other high functioning ASDs except that it takes into account the linguistic capabilities verbal strengths and nonverbal vulnerabilities of individuals with AS 21 A typical program generally includes 10 Applied behavior analysis ABA procedures including positive behavior support PBS or training and support of parents and school faculty in behavior management strategies to use in the home and school and social skills training for more effective interpersonal interactions 111 Cognitive behavioral therapy to improve stress management relating to anxiety or explosive emotions 112 and to help reduce obsessive interests and repetitive routines Medication for coexisting conditions such as major depressive disorder and anxiety disorders 113 Occupational or physical therapy to assist with poor sensory processing and motor coordination and Social communication intervention which is specialized speech therapy to help with the pragmatics and give and take of normal conversation 114 Of the many studies on behavior based early intervention programs most are case reports of up to five participants and typically examine a few problem behaviors such as self injury aggression noncompliance stereotypies or spontaneous language unintended side effects are largely ignored 115 Despite the popularity of social skills training its effectiveness is not firmly established 116 A randomized controlled study of a model for training parents in problem behaviors in their children with AS showed that parents attending a one day workshop or six individual lessons reported fewer behavioral problems while parents receiving the individual lessons reported less intense behavioral problems in their AS children 117 Vocational training is important to teach job interview etiquette and workplace behavior to older children and adults with AS and organization software and personal data assistants can improve the work and life management of people with AS 21 Medications No medications directly treat the core symptoms of AS 113 Although research into the efficacy of pharmaceutical intervention for AS is limited 21 it is essential to diagnose and treat comorbid conditions 49 Deficits in self identifying emotions or in observing effects of one s behavior on others can make it difficult for individuals with AS to see why medication may be appropriate 113 Medication can be effective in combination with behavioral interventions and environmental accommodations in treating comorbid symptoms such as anxiety disorders major depressive disorder inattention and aggression 21 The atypical antipsychotic medications risperidone olanzapine and aripiprazole have been shown to reduce the associated symptoms of AS 21 118 119 risperidone can reduce repetitive and self injurious behaviors aggressive outbursts and impulsivity and improve stereotypical patterns of behavior and social relatedness The selective serotonin reuptake inhibitors SSRIs fluoxetine fluvoxamine and sertraline have been effective in treating restricted and repetitive interests and behaviors 21 49 79 while stimulant medication such as methylphenidate can reduce inattention 120 Care must be taken with medications as side effects may be more common and harder to evaluate in individuals with AS and tests of drugs effectiveness against comorbid conditions routinely exclude individuals from the autism spectrum 113 Abnormalities in metabolism cardiac conduction times and an increased risk of type 2 diabetes have been raised as concerns with antipsychotic medications 121 122 along with serious long term neurological side effects 115 SSRIs can lead to manifestations of behavioral activation such as increased impulsivity aggression and sleep disturbance 79 Weight gain and fatigue are commonly reported side effects of risperidone which may also lead to increased risk for extrapyramidal symptoms such as restlessness and dystonia 79 and increased serum prolactin levels 123 Sedation and weight gain are more common with olanzapine 122 which has also been linked with diabetes 121 Sedative side effects in school age children 124 have ramifications for classroom learning Individuals with AS may be unable to identify and communicate their internal moods and emotions or to tolerate side effects that for most people would not be problematic 125 PrognosisThere is some evidence that children with AS may see a lessening of symptoms up to 20 of children may no longer meet the diagnostic criteria as adults although social and communication difficulties may persist 25 As of 2006 update no studies addressing the long term outcome of individuals with Asperger syndrome are available and there are no systematic long term follow up studies of children with AS 33 Individuals with AS appear to have normal life expectancy but have an increased prevalence of comorbid psychiatric conditions such as major depressive disorder and anxiety disorders that may significantly affect prognosis 21 25 Although social impairment may be lifelong the outcome is generally more positive than with individuals with lower functioning autism spectrum disorders 21 for example ASD symptoms are more likely to diminish with time in children with AS or HFA 126 Most students with AS and HFA have average mathematical ability and test slightly worse in mathematics than in general intelligence 127 However mathematicians are at least three times more likely to have autism spectrum traits than the general population and are more likely to have family members with autism 128 Although many attend regular education classes some children with AS may attend special education classes such as separate classroom and resource room because of their social and behavioral difficulties 33 Adolescents with AS may exhibit ongoing difficulty with self care or organization and disturbances in social and romantic relationships Despite high cognitive potential most young adults with AS remain at home yet some do marry and work independently 21 The different ness adolescents experience can be traumatic 129 Anxiety may stem from preoccupation over possible violations of routines and rituals from being placed in a situation without a clear schedule or expectations or from concern with failing in social encounters 21 the resulting stress may manifest as inattention withdrawal reliance on obsessions hyperactivity or aggressive or oppositional behavior 112 Depression is often the result of chronic frustration from repeated failure to engage others socially and mood disorders requiring treatment may develop 21 Clinical experience suggests the rate of suicide may be higher among those with AS but this has not been confirmed by systematic empirical studies 130 Education of families is critical in developing strategies for understanding strengths and weaknesses 49 helping the family to cope improves outcomes in children 53 Prognosis may be improved by diagnosis at a younger age that allows for early interventions while interventions in adulthood are valuable but less beneficial 49 There are legal implications for individuals with AS as they run the risk of exploitation by others and may be unable to comprehend the societal implications of their actions 49 EpidemiologyMain article Epidemiology of autism Frequency estimates vary enormously In 2015 it was estimated that 37 2 million people globally are affected 11 A 2003 review of epidemiological studies of children found autism rates ranging from 0 03 to 4 84 per 1 000 with the ratio of autism to Asperger syndrome ranging from 1 5 1 to 16 1 131 combining the geometric mean ratio of 5 1 with a conservative prevalence estimate for autism of 1 3 per 1 000 suggests indirectly that the prevalence of AS might be around 0 26 per 1 000 132 Part of the variance in estimates arises from differences in diagnostic criteria For example a relatively small 2007 study of 5 484 eight year old children in Finland found 2 9 children per 1 000 met the ICD 10 criteria for an AS diagnosis 2 7 per 1 000 for Gillberg and Gillberg criteria 2 5 for DSM IV 1 6 for Szatmari et al and 4 3 per 1 000 for the union of the four criteria Boys seem to be more likely to have AS than girls estimates of the sex ratio range from 1 6 1 to 4 1 using the Gillberg and Gillberg criteria 133 Females with autism spectrum disorders may be underdiagnosed 134 Comorbidities Main article Conditions comorbid to autism spectrum disorders Anxiety disorders and major depressive disorder are the most common conditions seen at the same time comorbidity of these in persons with AS is estimated at 65 21 Reports have associated AS with medical conditions such as aminoaciduria and ligamentous laxity but these have been case reports or small studies and no factors have been associated with AS across studies 21 One study of males with AS found an increased rate of epilepsy and a high rate 51 of nonverbal learning disorder 135 AS is associated with tics Tourette syndrome and bipolar disorder The repetitive behaviors of AS have many similarities with the symptoms of obsessive compulsive disorder and obsessive compulsive personality disorder 64 and 26 of a sample of young adults with AS were found to meet the criteria for schizoid personality disorder which is characterised by severe social seclusion and emotional detachment more than any other personality disorder in the sample 136 137 138 However many of these studies are based on clinical samples or lack standardized measures nonetheless comorbid conditions are relatively common 25 HistoryMain article History of Asperger syndrome Named after the Austrian pediatrician Hans Asperger 1906 1980 Asperger syndrome is a relatively new diagnosis in the field of autism 139 though a syndrome like it was described as early as 1925 by Soviet child psychiatrist Grunya Sukhareva 1891 1981 140 leading some of those diagnosed with Asperger s Syndrome to instead refer to their condition as Sukhareva s Syndrome in opposition to Hans Asperger s association with Nazism 1 As a child Asperger appears to have exhibited some features of the very condition named after him such as remoteness and talent in language 141 142 In 1944 Asperger described four children in his practice 49 who had difficulty in integrating themselves socially and showing empathy towards peers They also lacked nonverbal communication skills and were physically clumsy Asperger described this autistic psychopathy as social isolation 10 Fifty years later several standardizations of AS as a medical diagnosis were tentatively proposed many of which diverge significantly from Asperger s original work 143 Unlike today s AS autistic psychopathy could be found in people of all levels of intelligence including those with intellectual disability 144 Asperger defended the value of so called high functioning autistic individuals writing We are convinced then that autistic people have their place in the organism of the social community They fulfill their role well perhaps better than anyone else could and we are talking of people who as children had the greatest difficulties and caused untold worries to their care givers 17 Asperger also believed some would be capable of exceptional achievement and original thought later in life 49 Asperger s paper was published during World War II and in German so it was not widely read elsewhere Lorna Wing used the term Asperger syndrome in 1976 145 and popularized it to the English speaking medical community in her February 1981 publication 146 147 148 of case studies of children showing the symptoms described by Asperger 139 and Uta Frith translated his paper to English in 1991 17 Sets of diagnostic criteria were outlined by Gillberg and Gillberg in 1989 and by Szatmari et al in the same year 133 In 1992 AS became a standard diagnosis when it was included in the tenth edition of the World Health Organization s diagnostic manual International Classification of Diseases ICD 10 It was added to the fourth edition of the American Psychiatric Association s diagnostic reference Diagnostic and Statistical Manual of Mental Disorders DSM IV published in 1994 10 Hundreds of books articles and websites now describe AS and prevalence estimates have increased dramatically for ASD with AS recognized as an important subgroup 139 Whether it should be seen as distinct from high functioning autism is a fundamental issue requiring further study 49 and there are questions about the empirical validation of the DSM IV and ICD 10 criteria 33 In 2013 DSM 5 eliminated AS as a separate diagnosis folding it into the autism spectrum on a severity scale 41 Society and cultureSee also Societal and cultural aspects of autism and Disability rights movement Students and families walk to support Autism Awareness Month People identifying with Asperger syndrome may refer to themselves in casual conversation as aspies a term first used in print in the Boston Globe in 1998 149 150 Some autistic people have advocated 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 promote tolerance of neurodiversity 151 These views are the basis for the autistic rights and autistic pride movements 152 There is a contrast between the attitude of people with AS who typically do not want to be cured and are proud of their identity and parents of children with AS who typically seek assistance and a cure for their children 153 Some researchers have argued that AS can be viewed as a different cognitive style not a disorder 154 and that it should be removed from the standard Diagnostic and Statistical Manual much as homosexuality was removed 155 In a 2002 paper Simon Baron Cohen wrote of those with AS In the social world there is no great benefit to a precise eye for detail but in the worlds of maths computing cataloging music linguistics engineering and science such an eye for detail can lead to success rather than failure Baron Cohen cited two reasons why it might still be useful to consider AS to be a disability to ensure provision for legally required special support and to recognize emotional difficulties from reduced empathy 156 Baron Cohen argues that the genes for ASD s combination of abilities have operated throughout recent human evolution and have made remarkable contributions to human history 157 By contrast Pier Jaarsma and Welin wrote in 2011 that the broad version of the neurodiversity claim covering low functioning as well as high functioning autism is problematic Only a narrow conception of neurodiversity referring exclusively to high functioning autists is reasonable 158 They say that higher functioning individuals with autism may not be benefited with such a psychiatric defect based diagnosis some of them are being harmed by it because of the disrespect the diagnosis displays for their 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ISBN 978 1 85302 749 9 Aspie Oxford English Dictionary Online ed Oxford University Press Retrieved 29 May 2021 Subscription or participating institution membership required Williams CC 2005 In search of an Asperger In Stoddart KP ed Children Youth and Adults with Asperger Syndrome Integrating Multiple Perspectives Jessica Kingsley pp 242 52 ISBN 978 1 84310 319 6 The life prospects of people with AS would change if we shifted from viewing AS as a set of dysfunctions to viewing it as a set of differences that have merit Dakin CJ 2005 Life on the outside A personal perspective of Asperger syndrome In Stoddart KP ed Children Youth and Adults with Asperger Syndrome Integrating Multiple Perspectives Jessica Kingsley pp 352 61 ISBN 978 1 84310 319 6 Clarke J van Amerom G 2008 Asperger s syndrome differences between parents understanding and those diagnosed Social Work in Health Care 46 3 85 106 doi 10 1300 J010v46n03 05 PMID 18551831 S2CID 10181053 Clarke J van Amerom G 2007 Surplus 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using the AQ a preliminary study of its diagnostic validity in clinical practice in References Hus Vanessa Lord Catherine 2014 The Autism Diagnostic Observation Schedule Module 4 Revised Algorithm and Standardized Severity Scores Journal of Autism and Developmental Disorders 44 8 1996 2012 doi 10 1007 s10803 014 2080 3 PMC 4104252 PMID 24590409 A public paper re calibrating the Autism Diagnostic Observation Schedule for appropriate assessment of autistic adults who typically score lower on measures of impairment than autistic children due to compensatory strategies Royal College of Psychiatrists 2017 Interview Guide for the Diagnostic Assessment of Able Adults with Autistic Spectrum Disorder based on the Autism Diagnostic Interview Revised ADI R Retrieved from https en wikipedia org w index php title Asperger syndrome amp oldid 1143521652, wikipedia, wiki, book, books, library,

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