Asperger Syndrome
Information about Asperger Syndrome
| ICD-10 | F84.5 |
|---|---|
| ICD-9 | 299.8 |
| OMIM | 608638 |
| DiseasesDB | 31268 |
| MedlinePlus | 001549 |
| eMedicine | ped/147 |
Asperger syndrome (also Asperger's syndrome, Asperger's disorder, Asperger's, AS, or AD) is one of several autism spectrum disorders (ASD) characterized by difficulties in social interaction and by restricted and stereotyped interests and activities. AS is distinguished from the other ASDs in having no general delay in language or cognitive development. Although not mentioned in standard diagnostic criteria, motor clumsiness and atypical use of language are frequently reported.[][]
Asperger syndrome was named after Hans Asperger who, in 1944, described children in his practice who appeared to have normal intelligence but lacked nonverbal communication skills, failed to demonstrate empathy with their peers, and were physically clumsy. Fifty years later, AS was recognized in the International Statistical Classification of Diseases and Related Health Problems (ICD-10), and in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) as Asperger's Disorder. Questions about many aspects of AS remain: for example, there is lingering doubt about the distinction between AS and high-functioning autism (HFA);[] partly due to this, the prevalence of AS is not firmly established. The exact cause of AS is unknown, although research supports the likelihood of a genetic contribution, and brain imaging techniques have identified structural and functional differences in specific regions of the brain.
There is no single treatment for Asperger syndrome, and the effectiveness of particular interventions is supported by only limited data. Intervention is aimed at improving symptoms and function. The mainstay of treatment is behavioral therapy, focusing on specific deficits to address poor communication skills, obsessive or repetitive routines, and clumsiness. Most individuals with AS can learn to cope with their differences, but may continue to need moral support and encouragement to maintain an independent life.[1] Adults with AS have reached the highest levels of achievement in fields such as mathematics, physics and computer science.[1] Researchers and people with AS have contributed to a shift in attitudes away from the notion that AS is a deviation from the norm that must be treated or cured, and towards the view that AS is a difference rather than a disability.[1]
Classification
Asperger syndrome is 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 psychological development 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.[2] ASD, in turn, is a subset of the broader autism phenotype (BAP), which describes individuals who may not have ASD but do have autistic-like traits, such as social deficits.[3] Of the other four ASD forms, autism is the most similar to AS in signs and likely causes but its diagnosis requires impaired communication and allows delay in cognitive development; Rett syndrome and childhood disintegrative disorder share several signs with autism, but may have unrelated causes; and pervasive developmental disorder not otherwise specified (PDD-NOS) is diagnosed when the criteria for a more specific disorder are unmet.[4] The extent of the overlap between AS and high-functioning autism (HFA—autism unaccompanied by mental retardation) is unclear.[0][5][6] The current ASD classification may not reflect the true nature of the conditions.[7]Characteristics
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 activities and interests, and by absence of delay in cognitive development and of general delay in language.[8] Intense preoccupation with a narrow subject, one-sided verbosity, restricted prosody and intonation, and motor clumsiness are typical of the condition, but are not required for diagnosis.[0]Social interaction
The lack of demonstrated empathy is possibly the most dysfunctional aspect of Asperger syndrome.[0] Individuals with AS experience difficulties in basic elements of social interaction, which may include a failure to develop friendships or enjoy spontaneous interests or achievements with others, a lack of social or emotional reciprocity, and impaired nonverbal behaviors such as eye contact, facial expression, posture, and gesture.[0]Unlike those with autism, people with AS are not usually withdrawn around others; they approach others, even if awkwardly, for example by engaging in a one-sided, long-winded speech about an unusual topic while being oblivious to the listener's feelings or reactions, such as signs of boredom or wanting to leave.[0] This social awkwardness has been called "active, but odd".[0] This failure to react appropriately to social interaction may appear as disregard for other people's feelings, and may come across as insensitive. The cognitive ability of children with AS often lets them articulate social norms in a laboratory context,[0] 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.[0] People with AS may analyze and distill their observation of social interaction into rigid behavioral guidelines and apply these rules in awkward ways—such as forced eye contact—resulting in demeanor that appears rigid or socially naive. Childhood desires for companionship can be numbed through a history of failed social encounters.[0]
The hypothesis that individuals with AS are predisposed to violent or criminal behavior has been investigated and found to be unsupported by data.[0][11] More evidence suggests children with AS are victims rather than victimizers.[12]
Restricted and repetitive interests and behavior
People with Asperger syndrome display behavior, interests, and activities that are restricted and repetitive and are sometimes abnormally intense or focused. They may stick to inflexible routines or rituals, move in stereotyped and repetitive ways, or preoccupy themselves with parts of objects.[8]Pursuit of specific and narrow areas of interest is one of the most striking features of AS.[0] Individuals with AS may collect volumes of detailed information on a relatively narrow topic such as dinosaurs or deep fat fryers, without necessarily having genuine understanding of the broader topic.[0][0] For example, a child might memorize camera model numbers while caring little about photography.[0] This behavior is typically apparent by grade school, typically age 5 or 6 in the US.[0] The entire family may become immersed in the narrow topic. Because topics such as dinosaurs often capture the interest of children, this symptom may go unrecognized, and may not be apparent until the interests become more unusual and focused over time.[0]
Stereotyped and repetitive motor behaviors are a core part of the diagnosis of AS and other ASDs.[13] They include hand movements such as flapping or twisting, and complex whole-body movements.[8] 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.[14]
Speech and language
Although children with Asperger syndrome acquire language skills without significant general delay, and the speech of those with AS typically lacks significant abnormalities, language acquisition and use is often atypical.[0] 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.[0]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 autism, people with AS often have a limited range of intonation; speech may be overly 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 monitor 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.[0]
Children with AS may have an unusually sophisticated vocabulary at a young age and have been colloquially called "little professors", but have difficulty understanding metaphorical language and tend to use language literally.[0] Individuals with AS appear to have particular weaknesses in areas of nonliteral language that include humor, irony, and teasing. They usually understand the cognitive basis of humor but may not enjoy it due to lack of understanding of its intent.[6]
Other symptoms
Individuals with Asperger syndrome may have symptoms that are independent of the diagnosis, but can affect the individual or the family. These symptoms include atypical perception and problems with motor skills, sleep, and emotions.Asperger’s initial accounts[0] and other diagnostic schemes[15] include descriptions of motor clumsiness. Children with AS may be delayed in acquiring motor skills that require motor dexterity, such as bicycle riding or opening a jar, and may appear awkward or "uncomfortable in their own skin". They may be poorly coordinated, or have an odd or bouncy gait or posture, poor handwriting, or problems with visual-motor integration, visual-perceptual skills, and conceptual learning.[0][0] They may show problems with proprioception (sensation of body position) on measures of apraxia (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.[0]
Many accounts of individuals with AS and ASD report unusual sensory and perceptual skills and experiences. They may have superior performance in tasks like visual search problems that require processing of fine-grained features rather than entire configurations.[16] They may be unusually sensitive or insensitive to sound, light, touch, texture, taste, smell, pain, temperature, and other stimuli, and they may exhibit synesthesia, for example, a smell may trigger perception of color;[17] 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.[18]
Children with AS are more likely to have sleep problems, including difficulty in falling asleep, frequent nocturnal awakenings, and early morning awakenings.[19][19] AS is also associated with high levels of alexithymia, which is difficulty in identifying and describing one's emotions.[20] Although AS, lower sleep quality, and alexithymia are associated, their causal relationship is unclear.[19]
Causes
- Further information: Causes of autism
A few ASD cases have been linked to exposure to teratogens (agents that cause birth defects) during the first eight weeks from conception. Alhough this does not exclude the possibility that ASD can be initiated or affected later, it is strong evidence that it arises very early in development.[22] Many environmental factors have been hypothesized to act after birth, but none has been confirmed by scientific investigation.[23]
Mechanism
- Further information: Mechanism of autism

Functional magnetic resonance imaging provides some evidence for both underconnectivity and mirror neuron theories.[27][27]
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.[27][31] For example, one study found that activation is delayed in the core circuit for imitation in individuals with AS.[32] 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,[33] or hyper-systemizing, which hypothesizes that autistic individuals can systematize internal operation to handle internal events but are less effective at empathizing by handling events generated by other agents.[34]
Other possible mechanisms include serotonin dysfunction[35] and cerebellar dysfunction.[36]
Screening
Parents of children with Asperger syndrome can typically trace differences in their children's development to as early as 30 months of age.[21] Developmental screening during a routine check-up by a general practitioner or pediatrician may identify signs that warrant further investigation.[0][1] The diagnosis of AS is complicated by the use of several different screening instruments.[1][15] None have been shown to reliably differentiate between AS and other ASDs. The current "gold standard" in diagnosing ASDs uses 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.[0]Diagnosis
Diagnosis is most commonly made between the ages of four and eleven.[0] A comprehensive assessment involves a multidisciplinary team[0][1][1] that observes across multiple settings,[0] 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.[1] Delayed or mistaken diagnosis can be traumatic for individuals and families; for example, misdiagnosis can lead to medications that worsen behavior.[38] Many children with AS are initially misdiagnosed with attention-deficit hyperactivity disorder (ADHD).[0] Diagnosing adults is more challenging, as standard diagnostic criteria are designed for children and the expression of AS changes with age.[39] Conditions that must be considered in a differential diagnosis include other ASDs, the schizophrenia spectrum, ADHD, obsessive compulsive disorder, depression, semantic pragmatic disorder, nonverbal learning disorder,[38] Tourette syndrome,[14] stereotypic movement disorder and bipolar disorder.[21]
Underdiagnosis and overdiagnosis are problems in marginal cases. The cost of screening and diagnosis and the challenge of obtaining payment can inhibit or delay diagnosis. Conversely, the increasing popularity of drug treatment options and the expansion of benefits has motivated providers to overdiagnose ASD.[42] There are indications AS has been diagnosed more frequently in recent years, partly as a residual diagnosis for children of normal intelligence who do not have autism but have social difficulties. 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 and from PDD-NOS;[43] the same child can receive different diagnoses depending on the screening tool.[1]
Treatment
- Further information: Autism therapies
The ideal treatment for AS coordinates therapies that address core symptoms of the disorder, including poor communication skills and obsessive or repetitive routines. While most professionals agree that the earlier the intervention, the better, there is no single best treatment package.[1] 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.[0] A typical treatment program generally includes:[1]
- the training of social skills for more effective interpersonal interactions;[46]
- cognitive behavioral therapy to improve stress management relating to anxiety or explosive emotions,[47] and to cut back on obsessive interests and repetitive routines;
- medication, for coexisting conditions such as depression and anxiety;[47]
- occupational or physical therapy to assist with poor sensory integration and motor coordination;
- social communication intervention, which is specialized speech therapy to help with the pragmatics of the give and take of normal conversation;[48]
- the training and support of parents, particularly in behavioral techniques to use in the home.
No medications directly treat the core symptoms of AS.[47] Although research into the efficacy of pharmaceutical intervention for AS is limited,[0] it is essential to diagnose and treat comorbid conditions.[0] Deficits in self-identifying emotions or in observing effects of one's behavior's on others can make it difficult for an individual with AS to see why they should take medication.[47] Medication can be effective in combination with behavioral interventions and environmental accommodations in treating comorbid symptoms such as anxiety, depression, inattention and aggression.[0] The atypical neuroleptic medications risperidone and olanzapine have been shown to reduce the associated symptoms of AS;[0] 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.[0][0][21]
Care must be taken in the management of pharmacotherapy; abnormalities in metabolism, cardiac conduction times, and an increased risk of type 2 diabetes have been raised as concerns with these medications,[53][54] along with serious long-term neurological side effects.[49] SSRIs can lead to manifestations of behavioral activation such as increased impulsivity, aggression and sleep disturbance.[21] 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[21] and increased serum prolactin levels.[55] Sedation and weight gain are more common with olanzapine,[54] which has also been linked with diabetes.[53] Sedative side-effects in school-age children[56] 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.[57]
Prognosis
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.[0] Individuals with AS appear to have normal life expectancy but have an increased prevalence of comorbid psychiatric conditions such as depression, mood disorders, and obsessive-compulsive disorder that may significantly affect prognosis. Although social impairment is lifelong, outcome is generally more positive than with individuals with lower functioning autism spectrum disorders;[0] for example, ASD symptoms are more likely to diminish with time in children with AS or HFA.[58] Although most individuals with AS/HFA have average mathematical ability and test slightly worse in math than in general intelligence,[59] AS has been linked to high achieving mathematicians, physicists, computer scientists, and engineers, and the condition need not be an obstacle to achievement at the highest levels in these fields.[1]Children with AS may require special education services because of their social and behavioral difficulties although many attend regular education classes.[0] Adolescents with AS may exhibit ongoing difficulty with self-care, organization and disturbances in social and romantic relationships; despite high cognitive potential, most remain at home, although some do marry and work independently.[0] The "different-ness" adolescents experience can be traumatic.[61] 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;[0] the resulting stress may manifest as inattention, withdrawal, reliance on obsessions, hyperactivity, or aggressive or oppositional behavior.[47] Depression is often the result of chronic frustration from repeated failure to engage others socially, and mood disorders requiring treatment may develop.[0]
Education of families is critical in developing strategies for understanding strengths and weaknesses;[0] helping the family to cope improves outcome in children.[12] Prognosis may be improved by diagnosis at a younger age that allows for early interventions, while interventions in adulthood are valuable but less beneficial.[0] 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.[0]
Epidemiology
- Further information: Conditions comorbid to autism spectrum disorders
Prevalence estimates vary enormously. A 2003 review of epidemiological studies found prevalence rates ranging from 0.03 to 4.84 per 1,000, with the ratio of autism to Asperger syndrome averaging 5:1;[62] combining this 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.[63] 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 at higher risk for AS than girls; estimates of the sex ratio range from 1.6:1 to 4:1, using the Gillberg and Gillberg criteria.[63]
Anxiety and depression are the most common other conditions seen at the same time; comorbidity of these in persons with AS is estimated at 65%.[0] Depression is common in adolescents and adults; children are likely to present with ADHD.[64] 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.[0] One study of males with AS found an increased rate of epilepsy and a high rate (51%) of nonverbal learning disability.[65] AS is associated with tics, Tourette syndrome, and bipolar disorder, and the repetitive behaviors of AS have many similarities with the symptoms of obsessive compulsive disorder and obsessive-compulsive personality disorder.[66]
History
Lorna Wing popularized the term Asperger syndrome in the English-speaking medical community in her 1981 publication[69] of a series of case studies of children showing similar symptoms,<ref name="What'sSpecial"/> and Uta Frith translated his paper to English in 1991.[68] Sets of diagnostic criteria were outlined by Gillberg and Gillberg in 1989 and by Szatmari et al. in the same year.[63] AS became a standard diagnosis in 1992, when it was included in the tenth edition of the World Health Organization’s diagnostic manual, International Classification of Diseases (ICD-10); in 1994, it was added to the fourth edition of the American Psychological Association's diagnostic reference, Diagnostic and Statistical Manual of Mental Disorders (DSM-IV).[1]
Hundreds of books, articles and websites now describe AS, and prevalence estimates have increased dramatically for ASD, with AS recognized as an important subgroup.<ref name="What'sSpecial"/> Whether it should be seen as distinct from high-functioning autism is a fundamental issue requiring further study.[0] There is little consensus among clinical researchers about the use of the term Asperger's syndrome, and there are questions about the empirical validation of the DSM-IV and ICD-10 criteria.[0]
Cultural aspects
- Further information: Autistic culture
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 neurodiversity.[73] These views are the basis for the autistic rights and autistic pride movements.[74]
Simon Baron-Cohen has argued that AS and high-functioning autism are different cognitive styles, not disabilities, and that a diagnosis of AS/HFA should not be received as a family tragedy, but as interesting information, such as learning that a child is left-handed. According to Baron-Cohen, "people with AS/HFA might not necessarily be disabled in an environment in which an exact mind, attracted to detecting small details, is an advantage."[1] Tony Attwood argues, "the unusual profile of abilities that we define as Asperger's syndrome has probably been an important and valuable characteristic of our species throughout evolution."[76]
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Autism spectrum disorders (ASD), also called autism spectrum conditions (ASC) or the autism spectrum, with the word autistic sometimes replacing autism
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A stereotypy is a continuous, repetitive, purposeless or ritualistic movement, posture, or utterance, found in patients with mental retardation, autism spectrum disorders, tardive dyskinesia, and stereotypic movement disorder.
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Language delay is a failure to develop language abilities on the usual developmental timetable. Language delay is distinct from speech delay, in which the speech mechanism itself is the focus of delay.
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The Theory of Cognitive Development, one of the most historically influential theories was developed by Jean Piaget, a Swiss psychologist (1896–1980). His theory provided many central concepts in the field of developmental psychology and concerned the growth of intelligence,
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Motor skills disorder (also known as motor coordination disorder or motor dyspraxia) is a human developmental disorder that impairs motor coordination in daily activities. It is neurological in origin.
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Hans Asperger (February 18 1906 – October 21 1980) was the Austrian pediatrician after whom Asperger syndrome is named.
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Born on a farm outside Vienna, Asperger displayed an early talent for languages. He was a member in the youth movements of the 1920s...... Click the link for more information.
intelligence quotient or IQ is a score derived from one of several different standardized tests attempting to measure intelligence. IQ tests are used as predictors of educational achievement. People with low IQ scores are sometimes placed in special-needs education.
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Nonverbal communication (NVC) is the process of transmitting messages without spoken words, sometimes called body language, messages can be communicated through facial expressions; gestures; gaze; and posture; Many include the space we use around us; object communication
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Nonverbal communication (NVC) is the process of transmitting messages without spoken words, sometimes called body language, messages can be communicated through facial expressions; gestures; gaze; and posture; Many include the space we use around us; object communication
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Empathy (from the Greek εμπάθεια, "physical affection, partiality") is commonly defined as one's ability to recognize, perceive and feel directly the emotion of another.
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High-functioning autism (HFA) is an informal term applied to individuals with autism, an IQ of 85 or above, and the ability to speak, read, and write.[1] HFA may simply refer to autistic people who have normal overall intelligence; that is, are not cognitively challenged.
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In epidemiology, the prevalence of a disease in a statistical population is defined as the total number of cases of the disease in the population at a given time, or the total number of cases in the population, divided by the number of individuals in the population.
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Etiology (alternately aetiology, aitiology) is the study of causation. Derived from the Greek αίτιολογία, "giving a reason for" (
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Genetics is the science of heredity and variation in living organisms.[1][2] Knowledge of the inheritance of characteristics has been implicitly used since prehistoric times for improving crop plants and animals through selective breeding.
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Neuroimaging includes the use of various techniques to either directly or indirectly image the structure, function/pharmacology of the brain. It is a relatively new discipline within medicine and neuroscience.
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Autism spectrum disorders (ASD), also called autism spectrum conditions (ASC) or the autism spectrum, with the word autistic sometimes replacing autism
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MeSH D002659 The diagnostic category pervasive developmental disorders (PDD), as opposed to specific developmental disorders (SDD), refers to a group of five disorders characterized by delays in the development of multiple basic functions including socialization and
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A spectrum disorder in psychiatry is hard to define precisely but is a mental disorder having something to do with a spectrum of subtypes or closely related disorders. The spectrum model is proposed as a more coherent way of understanding psychiatric symptomatology.
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Social interaction is a dynamic, changing sequence of social actions between individuals (or groups) who modify their actions and reactions according to the actions by their interaction partner(s).
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phenotype describes the total physical appearance of an organism, as opposed to its genotype. This genotype-phenotype distinction was proposed by Wilhelm Johannsen in 1911 to make clear the difference between an organism's heredity and what that heredity produces.
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character is an attribute of an organism that allows it to be compared with another. In genetics this refers to heritable features which can exist in more than one state.[1] A trait
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