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© Cambridge University Press, 1993 This article was originally
published in The Journal of Child Psychology and Psychiatry and Allied
Disciplines, Vol. 34, No. 8, pp. 1327-1350, November 1993. It is provided
to Asperger Syndrome Coalition of the United States, Inc. for use on
its website with the express written permission of Cambridge University
Press. It may be downloaded and printed for personal reference, but
not otherwise copied, altered in any way or transmitted to others (unless
explicitly stated otherwise) without the written permission of Cambridge
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Abstract:
| This paper describes a total population study of Asperger syndrome
using a two-stage procedure. All school children in an outer G–teborg
borough were screened. Final case selection based on clinical work-up
showed a minimum prevalence of 3.6 per 1,000 children (7-16 years
of age) using Gillberg and Gillberg's criteria and a male to female
ratio of 4:1. Including suspected and possible Asperger's syndrome
cases, the prevalence rose to 7.1 per 1,000 children and the male:female
ratio dropped to 2.3:1. These findings are discussed as they relate
to previously published results in the field and to findings obtained
using Szatmari et al.'s and ICD-10 draft criteria for the disorder.
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Introduction
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Since Lorna Wing's vivid clinical account of "Asperger's
syndrome" appeared in 1981 (Wing, 1981), about 50 articles have
been published on the subject. However, the epidemiology of Asperger
syndrome (AS) has never been specifically studied.
Considering that the 10th revision of the International
Classification of Diseases (ICD-10) (World Health Organization,
1992) includes "Asperger's syndrome" as a subcategory of Pervasive
Developmental Disorders, obtaining reliable prevalence estimates
becomes mandatory.
A review of indirect data from neighboring fields
revealed that a minimum prevalence in children who attend normal
schools would be 2.6 per 1,000 children, i.e. several times higher
than that reported for autism (Gillberg & Gillberg, 1989). The
rate among mentally retarded children appears to be similar (Gillberg,
Persson, Grufman & Themnr, 1986). Still, given the relatively
low prevalence of mental retardation, AS cases recruited from
this subpopulation add little to the total population prevalence.
The available data originate from surveys not particularly geared
to examining AS prevalence. The object of the present study was
to provide reliable epidemiological data on AS.
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Defining AS
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The definition of a "case" presents difficulties in all prevalence
studies on childhood psychiatric disorders (Schwartz Gould, Wunsch-Hitzig
& Dohrenwend, 1981), and AS is no exception. The main reason for
this is that we lack a true "gold standard" (Szatmari, 1989).
Furthermore, there is no universal agreement on diagnostic characteristics
(Szatmari, 1991). Asperger's own descriptions are penetrating
but not sufficiently systematic (Asperger, 1944). His frame of
reference was Bleuler's typology (i.e. "autistic psychopathy")
that is out of keeping with current diagnostic manuals. Wing,
in her pioneer paper, did not explicitly spell out which symptoms
had to be present for a diagnosis to be made, though more specified
characteristics are presented in a recently published paper (Wing,
1991). Other authors, such as Gillberg and Gillberg (1989), and
Szatmari, Brenner and Nagy (1989) from Asperger's and Wing's work,
clinical experience and comparative studies, have proposed operationalized
diagnostic criteria. In several other publications (Bosch, 1970;
Van Krevelen, 1971; Wolff & Barlow, 1979; Wolff & Chick, 1980;
Nagy & Szatmari, 1986; Kerbeshian & Burd, 1986; Rutter & Schopler,
1987; Kay & Kolvin, 1987; Bowman, 1988; Frith, 1989; Tantam, 1988a,
b, 1991; Goodman, 1989; Bishop, 1989; Baron-Cohen, 1988; Kerbeshian,
Burd & Fisher, 1990; Green, 1990; Howlin, 1991; Cox, 1991; Wolff,
1991a, b; Wolff, Townsend, McGuire & Weeks, 1991; Gillberg, 1992)
the delineation of the syndrome vis-ý-vis autism, schizoid personality
and schizotypal personality disorder, Tourette syndrome, semantic-pragmatic
language disorder and obsessive-compulsive disorder has been discussed.
The criteria of the Gillbergs (1989, 1991) and Szatmari et al.
(1989) have been elaborated with the purpose of making them compatible
with current diagnostic manuals. The proposals of these two groups
have much in common, but differ in that the Gillbergs underline
the children's obsessional and narrow pattern of interest and
Szatmari et al. highlight their social isolation. In short, Szatmari
et al.'s criteria appear to be slightly more in line with "the
passive" and the Gillbergs' criteria with "the active but odd"
in Wing's typology of autism spectrum disorders (Wing & Gould,
1979; Wing & Attwood, 1987).
The ICD-10 draft criteria for AS (WHO, 1990) are similar to those
of the Gillbergs, but with one important exception. In the ICD-10
"the term Asperger's syndrome proposes that there is a group of
individuals who have a disorder of social development similar
to that found in infantile autism, but with a pattern of early
language development that appears grossly normal" (Cox, 1991).
Accordingly, criteria of abnormalities in verbal communication
are not included. However, by not including operationalised criteria
of abnormalities, such as odd prosody and semantic-pragmatic problems,
the ICD-10 criteria exclude features that other authors in the
field find important and characteristic of AS (Asperger, 1944;
Wing, 1981, 1991). Current diagnostic criteria for AS are outlined
in Tables 1-3.
In the present study we applied the diagnostic criteria for AS
outlined by Gillberg and Gillberg (1989) and elaborated in Gillberg
(1991), since, at the time of embarking on the study, these were
the only published criteria available to us. Also, we report the
result of applying Szatmari et al.'s (1989) and the ICD-10 (WHO,
1990) diagnostic criteria for the disorder.
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Table 1. Asperger syndrome: Gillberg and Gillberg's (1989)
diagnostic criteria elaborated
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- Social impairment (extreme egocentricity)
(at least two of the following):
- Inability to interact with peers
- Lack of desire to interact with peers
- Lack of appreciation of social cues
- Socially and emotionally inappropriate behavior
- Narrow interest (at least one of the following):
- Exclusion of other activities
- Repetitive adherence
- More rote than meaning
- Repetitive routines (at least one of the following):
- On self, in aspects of life
- On others
- Speech and language peculiarities (at least three of
the following):
- Delayed development
- Superficially perfect expressive language
- Formal pedantic language
- Odd prosody, peculiar voice characteristics
- Impairment of comprehension including misinterpretation
of literal/implied meanings
- Nonverbal communication problems (at least one of the
following):
- Limited use of gestures
- Clumsy/gauche body language
- Limited facial expression
- Inappropriate expression
- Peculiar, stiff gaze
- Motor clumsiness:
Poor performance on neuro-developmental examination
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Table 2. Asperger's syndrome: Szatmari et al.'s (1989)
diagnostic criteria
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- Solitary - two of:
- No close friends
- Avoids others
- No interest in making friends
- A loner
- Impaired Social Interaction - one of:
- Approaches others only to have own needs met
- A clumsy social approach
- One-sided responses to peers
- Difficulty sensing feelings of others
- Detached from feelings of others
- Impaired Nonverbal Communication - one of:
- Limited facial expression
- Unable to read emotion from facial expressions of child
- Unable to give messages with eyes
- Does not look at others
- Does not use hands to express oneself
- Gestures are large and clumsy
- Comes too close to others
- Odd Speech - two of:
- Abnormalities in inflection
- Talks too much
- Talks too little
- Lack of cohesion to conversation
- Idiosyncratic use of words
- Repetitive patterns of speech
- Does not meet DSM-III-R criteria for:
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Table 3. Asperger's syndrome: ICD-10 (1990)
diagnostic criteria
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- A lack of any clinically significant general delay
in language or cognitive development. Diagnosis requires that
single words should have developed by two years of age or earlier
and that communicative phrases be used by three years of age
or earlier. Self-help skills, adaptive behavior and curiosity
about the environment during the first three years should be
at a level consistent with normal intellectual development.
However, motor milestones may be somewhat delayed and motor
clumsiness is usual (although not a necessary feature). Isolated
special skills, often related to abnormal preoccupations, are
common, but are not required for diagnosis.
- Qualitative impairments in reciprocal social interaction (criteria
as for autism). Diagnosis requires demonstrable abnormalities
in at least three out of the following five areas:
- failure adequately to use eye-to-eye gaze, facial
expression, body posture and gesture to regulate social interaction;
- failure to develop (in a manner appropriate to mental age,
and despite ample opportunities) peer relationships that involve
a mutual sharing of interests, activities and emotions;
- rarely seeking and using other people for comfort and affection
at times of stress or distress and/or offering comfort and
affection to others when they are showing distress or unhappiness;
- lack of shared enjoyment in terms of vicarious pleasure
in other people's happiness and/or a spontaneous seeking to
share their own enjoyment through joint involvement with others;
- a lack of socio-emotional reciprocity as shown by an impaired
or deviant response to other people's emotions; and/or lack
of modulation of behavior according to social context, and/or
a weak integration of social, emotional and communicative
behaviors.
- Restricted, repetitive, and stereotyped patterns of behavior,
interests and activities (criteria as for autism; however it
would be less usual for these to include either motor mannerisms
or preoccupations with part-objects or nonfunctional elements
of play materials). Diagnosis requires demonstrable abnormalities
in at least two out of the following six areas:
- an encompassing preoccupation with stereotyped and restricted
patterns of interest;
- specific attachments to unusual objects;
- apparently compulsive adherence to specific, nonfunctional,
routines or rituals;
- stereotyped and repetitive motor mannerisms that involve
either hand/finger flapping or twisting, or complex whole
body movements;
- preoccupation with part-objects or nonfunctional elements
of play materials (such as their odor, the feel of their surface,
or the noise/vibration that they generate);
- distress over changes in small, nonfunctional, details of
the environment
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