Abstract
First described in 1944 by Hans , it was not before 1994 that Asperger Syndrome (AS) was included in the fourth edition of the Diagnostic and Statistical Manual of Mental Disorders, only to disappear in the Manual’s fifth edition in 2013. During its brief existence as a diagnostic entity, AS aroused immense interest and controversy. Similar to patients with autism, AS patients show deficits in social interaction, inappropriate communication skills, and interest restriction, but also display a rich variety of subtle clinical characteristics that for many distinguish AS from autism. However, difficulties operationalising diagnostic criteria and differentiating AS from autism ultimately led to its merging into the unifying category of Autistic Spectrum Disorders. Here we briefly review the short history of this fascinating condition.
Introduction: Refrigerator Mothers and Fine-Boned Aristocrats
The entry of autism and Asperger syndrome (AS) into the history of psychopathology was marked by extraordinary coincidences. Both disorders were first described by and , respectively. Both were Austrian-born physicians and, though unaware of each other’s writings, both used the term “autistic” to describe a unique group of children who shared features of impaired social interaction and restricted, repetitive behaviors and interests. Both and borrowed the term “autistic” from Eugen Bleuler, who used it in his “Dementia Praecox or the Group of Schizophrenias” to describe extreme social withdrawal and self-centeredness in patients with schizophrenia. Moreover, both authors emphasized that the syndrome they were describing differed from infantile (e.g., De Sancti’s dementia praecocissima) and juvenile schizophrenia, namely by manifesting from birth and improving (in terms of social interaction) with growth, in contrast to the usual course of schizophrenia (). Significantly, although initially considered language abnormalities (varying from sheer absence of language to atypical, socially ineffective use of well-developed language) to be a defining feature of his “Autistic Disturbances of Affective Contact,” he later hypothesized that they could be secondary to the two nuclear features of the disorder: “extreme self-isolation” and “obsessive insistence on sameness” (). Furthermore, also noted that many of his patients possessed “good cognitive potential.” Linguistic and cognitive ability would later sit in the eye of the storm unleashed by the appearance of AS as a discrete diagnostic entity and the relentless polemic that accompanied it. While Kanner’s syndrome eventually made its way to the third edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-III), Asperger’s work, published in German, remained virtually unknown to the international scientific community for almost half a century. Indeed, the first English translation of Asperger’s article “Die Autistischen Psychopathen im Kindesalter” first appeared in 1991 in Uta Frith’s textbook “autism and AS.” AS had already been described in 1981 by Lorna Wing, who first proposed the term to refer to a special subgroup of children who, according to Asperger’s original description, were characterized by: social isolation and lack of reciprocity in social interactions; normal or precocious language acquisition, with above-average linguistic skills but subtle abnormalities of verbal and non-verbal communication (e.g., atypical syntax, pedantic vocabulary and absent or stereotyped prosody); a narrow focus of interests, often restricted to unpragmatic and highly original themes; overachievement in specific cognitive domains; and motor clumsiness (). Unlike , did not attempt to define diagnostic criteria for the disorder he was describing. Moreover, Asperger greatly emphasized subtle positive features in his patients: they often had extremely original thought, they tended to cultivate abstract and intellectualized interests, often had, in Asperger’s own words, “a rare maturity of taste in art,” and even a peculiar, fascinating physical appearance, with “finely boned features,” of “almost aristocratic appearance” (). Asperger’s captivating descriptions of his subjects certainly played a decisive role in the history of the syndrome that bore his name, especially as they contrasted sharply with Kanner’s later recriminatory writings on “refrigerator-mothers” and the origin of autism (). Indeed, although Lorna Wing in her initial account of Asperger’s work clearly stated her belief that AS and Kanner’s autism were both part of an autistic spectrum, the idea of AS as an autonomous disorder, distinct from autism, quickly got hold of the opinion of many authors in the field, and certainly of the general public’s curiosity for autism and related disorders (). It is important to note here that Asperger himself referred to Kanner’s paper, concluding that his subjects were clearly different from those described by . The idea quickly made its way that Kanner’s autism and AS were different disorders, distinguished mainly by the fact that AS children had good cognitive and linguistic skills and a normal development in the first 2–3 years of life (). Moreover, for many authors the impairment in social interaction differed qualitatively between AS and Kanner’s autism: while in the latter children seemed completely uninterested in others, AS children tried to relate with others but approached them in a dysfunctional and inconvenient way (; ). Asperger himself contributed to this view that autism and AS subjects might be distinguished on the basis of cognitive ability and language development by emphasizing his patients’ high intelligence and their acquisition of grammatical speech before they could walk (). The number of publications on AS grew exponentially in the years following paper, and in 1994 AS was finally included in DSM – IV (Figure 1).
FIGURE 1
Defining Asperger Syndrome: A Tower of Babel
DSM-IV broadened the diagnostic boundaries of Autism, conceived for the first time as a spectrum of disorders that included Autistic Disorder, AS and Pervasive Developmental Disorder Not Otherwise Specified (). Also new was the inclusion of Childhood Disintegrative Disorder (Heller Syndrome) and Rett Syndrome, both characterized by developmental regression with severe autistic features (). A diagnosis of Autism required only six symptoms (in contrast with the minimum of eight required in DSM-IIIR), including at least two social interaction deficits, two communication deficits, and one symptom of interest restriction/repetitive behavior. Functional impairment had to be obvious before age three. The newly created category of AS required at least two symptoms of social interaction deficits and one symptom of behavioral and interest restriction, normal cognitive, and linguistic development before age 3, and age-adequate adaptive functioning in areas other than social interaction. Onset before age three was not mandatory. Importantly, the subject should not meet diagnostic criteria for Autistic Disorder – in which case the latter diagnosis should be given precedence, implying a differential diagnosis between AS and autism without cognitive delay, also called high-functioning autism (HFA; ). Meanwhile, other sets of diagnostic criteria for AS had appeared (Figure 2). In 1988 Carina and Christopher proposed six criteria based on Asperger’s original case-reports: socially impairing egocentricity, narrow interest patterns, compulsive routine adherence, peculiarities of speech and language, deficits in non-verbal communication, and motor clumsiness (). Diagnosis required all six. There was no clause precluding a diagnosis of autism, and no mention of a minimum age limit or periods of normal development. 1 year later, proposed four mandatory criteria, comprising 22 symptoms: social isolation, impaired social functioning, deficits in non-verbal communication, and peculiarities of speech and language. As in DSM-IV, Autism was given diagnostic precedence over AS. Finally, WHO’s 1993 International Classification of Diseases and Disorders (ICD-10) also suggested diagnostic criteria for AS, essentially similar to DSM-IV’s (). Although not exactly contradictory, these several diagnostic schemes nevertheless produced a bewildering semiologic cacophony. Gillberg’s criteria are too restrictive, and the only mentioning clumsiness as a mandatory symptom. Szatmari’s criteria do not include interest restriction – a major criterion in the remaining diagnostic systems. DSM-IV and ICD-10 do not require abnormal non-verbal communication, mandatory in Gillberg’s and Szatmari’s sets. However, the most problematic clause, present in ICD-10, DSM-IV and Szatmari’s criteria, was the exclusion of a diagnosis of AS if criteria for autism were met.
FIGURE 2
The End in the Beginning
The consecration of AS as a distinct diagnosis was surrounded by controversy from the outset. Contradictions in the syndrome’s definition soon became evident that would ultimately doom AS to extinction in DSM-5. The main problem was the precedence given to a diagnosis of autism. It soon became clear that most patients with significant impairments in social interaction and restriction of interests and activities also fulfill criteria for autistic disorder, thus precluding a diagnosis of AS. The requirement of normal cognitive and linguistic development failed to rescue a diagnosis of AS for the simple reason that cognitive and linguistic delay are not mandatory for diagnosing autistic disorder (
Is as Different from HFA?
Eventually, the controversy gradually converged onto knowing if AS and HFA can be distinguished qualitatively (suggesting different etiological and neurobiological mechanisms for each syndrome), or if they merely differ quantitatively and should therefore be regarded as variants of a single disorder (
Although many authors consider clumsiness as typical of AS, studies on motor control and gait have only found subtle differences in comparisons with HFA (
An obvious approach to the question of whether or not AS and HFA are distinct entities is to look for biological differences between them. Despite the accumulated evidence on neurophysiological abnormalities in autism spectrum disorders (ASD) as a group, few data are available on possible differences between AS and HFA, apart from subtle differences in EEG connectivity patterns and left-hemisphere intra-cortical inhibition (abnormally decreased in HFA but not in AS;
Structural MRI studies comparing AS and HFA have produced contradictory results, with two recent meta-analyses and a recent systematic review reaching three different conclusions (
On the whole, data on the distinctiveness between AS and HFA remain difficult to interpret. Many differences are quantitative rather than qualitative, and are distinctly more pronounced at younger ages, although the same might be said of many differences that discriminate HFA from typically developing subjects (
A Foretold Resurrection?
Notwithstanding some clinical and biological differences between AS and HFA, the DSM-5 Neurodevelopmental Disorders Workgroup finally decided that there is insufficient evidence to support a meaningful distinction between them (
Statements
Funding
. This work was supported by an unrestricted grant from Novartis Pharma.
Conflict of interest
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer João Gama Marques and handling Editor Diogo Telles-Correia declared their shared affiliation, and the handling Editor states that the process nevertheless met the standards of a fair and objective review.
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Summary
Keywords
Asperger Syndrome, autism spectrum disorders, DSM-5, psychopathology, nosology
Citation
Barahona-Corrêa JB and Filipe CN (2016) A Concise History of Asperger Syndrome: The Short Reign of a Troublesome Diagnosis. Front. Psychol. 6:2024. doi: 10.3389/fpsyg.2015.02024
Received
30 July 2015
Accepted
18 December 2015
Published
25 January 2016
Volume
6 - 2015
Edited by
Diogo Telles-Correia, University of Lisbon, Portugal
Reviewed by
Andrea Raballo, University of Oslo, Norway; João Gama Marques, University of Lisbon, Portugal
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© 2016 Barahona-Corrêa and Filipe.
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*Correspondence: J. B. Barahona-Corrêa, bernardo.correa@cadin.net
This article was submitted to Psychopathology, a section of the journal Frontiers in Psychology
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