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Home , Asperger syndrome, Developmental disorder

Archives ofDisease in Childhood 1991; 66: 259-262 259

PERSONAL VIEW Arch Dis Child: first published as 10.1136/adc.66.2.259 on 1 February 1991. Downloaded from

Is Asperger's a useful diagnosis?

A D Cox

The first published account of early infantile is adopting the term, pervasive developmental was in 1943 and Asperger's original disorders, and includes subcategories not only paper appeared the following year.1 Childhood of childhood autism but also Asperger's syn- autism and Asperger's syndrome are in many drome (see table 2)."1 How is it proposed that ways similar and there is continuing argument this diagnostic term is to be used? It is useful? It as to whether it is useful or appropriate to con- appears that Gillberg uses Asperger's syndrome sider them as separate entities.23 as the equivalent of 'high functioning autism'.9 In the 9th revision of the International Classi- If that is really how the term is to be used, then fication ofDiseases (ICD-9) infantile autism is a one may indeed wonder whether it is appropri- subcategory of psychoses with origin specific to ate to have a separate subcategory. In his origi- childhood and Asperger's syndrome is not a nal description, Asperger emphasised a number listed subcategory (see table 1).4 The revised of features.'1 1 Firstly, the child usually begins American classification in the Diagnostic and to speak at the age expected in normal children Statistical Manual of Mental Disorders (DSM- and a full command of grammar is sooner or III), considered that infantile autism was no later acquired. However, the manner of speak- longer appropriately classified with psychoses ing is often pedantic and subjects engage in and included it as a subcategory under the gen- monologues rather than conversations. Use of eral classification of pervasive developmental gestures tends to be restricted and comprehen- disorders."7 Again there is no separate sub- sion of others' facial expressions and gestures is category for Asperger's syndrome. Pervasive poor. Secondly, there is an impairment of two developmental disorders are seen as charac- way social interaction with a lack of ability to terised by a qualitative impairment in the understand and use the rules governing social development of reciprocal social interaction and behaviour. It appears that there is not a primary

of verbal and non-verbal skills, desire to withdraw from social contact, rather a http://adc.bmj.com/ autistic disorder being the one acknowledged lack of ability to comprehend social situations specific subcategory. Field trials have shown and respond appropriately. Thirdly, as in infan- that there are two mandatory criteria for autistic tile autism, there is commonly in early child- disorder: an appreciable lack of awareness of the hood a preoccupation with particular activities existence or of others, and persistent and resistance to change. Later there are preoccupations with parts of objects.8 Gillberg all absorbing circumscribed interests and en- has criticised the use of the term pervasive gagement in routines. Fourthly, gross motor on September 30, 2021 by guest. Protected copyright. preferring 'autism spec- movements are characteristically clumsy and trum disorders' and he has recently suggested uncoordinated. that a separate diagnostic designation of infan- It will be seen that the description of tile autism doesn't seem to make a 'lot of sense Asperger's syndrome is substantially similar to any more'.9 Although he argues that the term the picture in some relatively intelligent indi- 'pervasive developmental disorder' is too loosely viduals with a diagnosis of infantile autism. defined and that the disorders included in the Indeed, Asperger originally believed that people category are 'specific' and/or 'partial' rather with his syndrome were of high intelligence. than pervasive, it seems likely that there is no What stands out as different from infantile aut- intended difference in the range of disorders ism is the superficially normal early language covered by the term 'pervasive developmental development. The term, Asperger's syndrome, disorder' and ' disorder'. therefore proposes that there are a group of Despite Gillberg's criticisms and sceptism about subclassification, the 10th revision of the United Medical and International Classification of (ICD-10) Table 2 International Classification of Diseases, 10th Dental Schools of reviston Guy's and St Thomas's Hospitals, F84 Pervasive developmental disorders: London F84-0 Childhood autism Table I International Classiftcation of Diseases, 9th F84 1 Atypical autism Correspondence to: revision F84-10 Atypicality in age of onset Professor A D Cox, F84-11 Atypicality in symptomatology Bloomfield Clinic, 299 Psychoses with origin specific to childhood: F84-12 Atypicality in both age of onset and Department of Child and 1-0 Infantile autism symptomatology Adolescent , 1-1 Disintegrative F84-2 Rett's syndrome Guy's Hospital, 1-8 Other F84-3 Other childhood disintegrative disorder St Thomas Street, 1-9 Unspecified F84-5 Asperger's syndrome London SEI 9RT. 260 Cox

individuals who have a disorder of social may be that there are subtle or not so subtle dif- development similar to that found in infantile ferences among the high functioning 'autistic' Arch Dis Child: first published as 10.1136/adc.66.2.259 on 1 February 1991. Downloaded from autism, but with a pattern of early language individuals with these discrepancies in early lan- development that appears grossly normal; at guage development. For example, it has been least as regards the milestones that are usually suggested that those with superficially nor- recorded. Tantam states that there is no diffi- mal early speak spon- culty with propositional language but 'marked taneously more often and demonstrate an pragmatic impairment'.3 The core features of active, if restricted fantasy life (S Wolff, per- autistic social development are often referred to sonal communication). as 'Wing's triad': deficits in social interaction, It is just this difference in early language communication, and imagination.12 development that is used as a basis for the estab- A major diagnostic difficulty in infantile aut- lishment of the subcategory of Asperger's syn- ism is that the classical criteria are most easily drome in ICD-10. It states that in Asperger's recognised among children aged between 3 and syndrome there is 'A lack of any clinically sig- 5 years. It is at this stage of development that nificant general delay in language or cognitive aloofness and indifference in social interactions development'. ' The criteria for qualitative may be most readily observed alongside a lack impairments in reciprocal social interaction are of language, including a lack of comprehension identical with those for childhood autism. The of speech and repetitive physical activities. The same is true for the restricted, repetitive and pattern of symptomatology changes, however, stereotyped patterns of behaviour, interest, and and changes most noticeably among more able activities. However, it is commented that it individuals. would be 'less usual for these to include either Wing has described what she calls an 'autistic motor-mannerisms or preoccupation with part continuum' (table 3) in which the most severely objects or non-functional elements of play disabled individuals are aloof and indifferent in materials'. The aim appears to be to draw atten- social interaction, the more able or least handi- tion to the existence of children with social capped characterised by bizarre one sided developmental disorders of a type found in social approaches. 13 More able autistic indi- childhood autism but without a major disorder viduals who develop language move from no of language development affecting comprehen- language at age 3 to quite extensive use of sion and expression. speech which, as in Asperger's syndrome, is There may be an empirical descriptive basis used in a grammatical but one sided and literal for a diagnosis of Asperger's syndrome, but fashion. The suggestion is that there are paral- there does not appear yet to be any definite lels in function in the areas of social interaction, psychopathological nor neuropathological dis- communication, and obsessional interests and tinction from high functioning childhood aut- activities. Those of low ability, or those of ism. A recent study of the use of intonation to higher ability at an earlier stage of development communicate, however, which compared sub- show aloofness, no language, and simple repeti- jects with Asperger's syndrome with high func-

tive activities. More able individuals, particu- tioning autistics concludes that 'an early history http://adc.bmj.com/ larly at a later stage of development, display a of deviant language' 'is associated with more more elaborate picture of social ineptness, cir- pronounced failure to use intonation to com- cumscribed interests, and concrete egocentric municate effectively later during childhood and use of language. This analysis could be seen to adolescence' (A J Fine et al, unpublished obser- reinforce the notion that Asperger's syndrome is vations). There were also differences in the abil- no more than high functioning autism. There ity to decode non-verbal social information.15 are individuals, however, with autistic social Considerable progress has been made in development who have appreciably abnormal trying to understand the nature of the psycholo- on September 30, 2021 by guest. Protected copyright. early language development including a signifi- gical processes which are dysfunctional in child- cant delay in comprehension of speech, who hood autism. Two main views are, on the one subsequently attain to a high functioning pic- hand that there is a deficit in the innate ability ture. There are others, also high functioning, to interact emotionally with others, and on the who do not have a history of abnormal early lan- other an impairment in the cognitive process of guage development, at least in a gross sense.14 metarepresentation.'6 17 Does this matter? If it is true that a similar The ability to form representations of repre- high functioning autistic picture can be sentations is considered necessary in order to obtained on the basis of quite different early 'mentalise'-that is, to think and reason about language development, what are the implica- one's own and others' minds. Attribution of tions for our understanding of the relationship beliefs and intentions to others is said to reflect between social and language development? It an individual's ''. Communica-

Table 3 The autistic continuum (features most often used in diagnosis)'3 Item Tend to be seen Tend to be seen in in the most severely least severely handicapped/retarded handicapped/retarded Social interaction (1) Aloof and indifferent (2) Approaches for (3) Passively accepts (4) Makes bizarre one physical needs only approaches sided approaches Social communication (1) No communication (2) Needs only (3) Replies if approached (4) Spontaneous, but (verbal and non-verbal) repetitive, one sided, odd IsAsperger's syndrome a useful diagnosis? 261

tion that is not modified by inferences about individuals with fragile X is more other's beliefs and intentions is literal and one characteristic than social indifference and that sided. 18 hyperactivity and attentional problems are Arch Dis Child: first published as 10.1136/adc.66.2.259 on 1 February 1991. Downloaded from The metarepresentational deficit theory has extremely common with frequent abnormalities been criticised on the ground that deficiencies in spoken language. It is nevertheless consi- in behaviour are a feature of dered that this genetic anomaly may be impor- autistic children and that these normally emerge tant in a small number of individuals with between 6 and 9 months of age, at least autism. Other evidence supports the overall six months before the capacity for significance of genetic factors in autism, metarepresentation. 19 although there is still some doubt as to the Further criticism comes from the other main extent to which any of them is specific for the theory that argues that the exclusively cognitive social developmental disorder as opposed to cer- formulation of the metarepresentational tain cognitive abnormalities.25 26 It is not evi- hypothesis misunderstands the connection dent what the position is with regard to between social and emotional development.20 21 Asperger's syndrome and this is partly because Hobson suggests that autistic individuals fail to it is unclear whether research into high level develop an adequate concept of persons because autistic children can be considered research into 'an impairment in biologically based capacities Asperger's syndrome. DeLong and Dwyer for engaging in (and representing) personal found that high functioning autistics have a relatedness, especially affective relatedness'. positive family history for Asperger's syndrome This impairment is present before the stage at and low functioning autistics have a high inci- which joint attention usually develops. dence of abnormal neurological findings.27 This second main view has in turn been Bipolar affective illness was also more common attacked on the ground that there is no empiri- among families of high function autistics. This cal support for the hypothesised affective dis- study claims that there was little distinction order in infancy.22 In addition, recent work sug- between high functioning autism and gests autistic children can develop selective Asperger's syndrome among its probands, but attachments and that they also pick up the feel- the criteria used for the diagnosis of Asperger's ings of others.'8 23 Sigman and Mundy regard syndrome did not include superficially normal the children's difficulties as one of social early language development. comprehension-the identification of the signi- With regard to the of Asperger's ficance and meaning of their experiences- syndrome, it has recently been reported to be of rather than one of social responsiveness.23 the order of 2S6-3 per 1000 children with a The various theories are concerned with a heavy male preponderance as in childhood social psychological deficit that is conceived as a autism.2' However, the Gillbergs used delayed core dysfunction in both autism and Asperger's language development and mild or moderate syndrome. If the underlying dysfunction is impairment of language comprehension as inclu- detectable before language develops then the sion criteria for Asperger's syndrome alongside

level of deficit could be comparable in the two 'superficially perfect expressive language'. http://adc.bmj.com/ conditions when individuals are matched for These criteria obscure the difference between intelligence quotient and language level. If the Asperger's syndrome and high functioning aut- core dysfunction lies in metarepresentation, ism. The problem appears to arise from a desire however, it could be expected to be alleviated in not to assign a diagnosis of autism to individuals those whose language development is relatively with 'superficially perfect expressive language' preserved. In other words it would be predicted in adolescence who met criteria for autism at age that those with Asperger's syndrome would 3 to 5 years. Nevertheless it must be recognised have better than those with autism. that there are considerable difficulties in draw- on September 30, 2021 by guest. Protected copyright. Whether this is so is not known. ing the line between and Although there is increasing evidence of for empathic aspects of social development. neuropathology among autistic children,9 and a wide variety of specific may under- lie the manifestation of the syndrome, there is Conclusion and comment still considerable debate about localisation. In clinical practice it can often appear a some- Neuropsychological studies do not assist in this what sterile academic exercise to decide which respect. 17 Recent investigation by elec- subcategory of pervasive development disorder troencephalography has supported the notion is appropriate for a given child. The phrase that there may be deficits in information proces- 'autistic spectrum' is problematic because the sing (H van Engeland, paper given at European abnormalities of development in the different Medical Research Councils workshop; Brussels, areas do not correlate very exactly. For ex- November 1989),24 but it has been proposed ample, knowledge of a child's general intellec- that the most consistent recent findings point to tual level does not predict whether they have brain stem pathology.9 an autistic social developmental abnormality, From the genetic point of view there is dis- although it is of course more common among agreement about the significance of the fragile those with low intelligence. Wing and Gould X anomaly, although a very recent authoritative reported that half of the severely mentally review states that it is 'now clear that the pro- retarded have the 'triad' of impairments in portion of autistic individuals with fragile X is social recognition, social communication, and no higher and may even be lower than the pro- social understanding.29 Similarly, a develop- portion of mentally retarded individuals with mental affecting comprehen- fragile X'.25 It is further suggested that among sion does not necessarily predict a major social 262 Cox

developmental disorder, although recent follow sigmal detection theory to assess DSM-III R criteria for up has suggested that such children do have autistic disorder. J Am Acad Child Adolesc Psychiatry 1989;28:542-8. Arch Dis Child: first published as 10.1136/adc.66.2.259 on 1 February 1991. Downloaded from some social difficulty, if not of severity that 9 Gillberg C. Autism and pervasive developmental disorders. J Child Psychol Psychiatry 1990;31:99-119. would make a diagnosis of pervasive develop- 10 World Health Organisation. Mental and behavioural dis- appropriate.30 orders (including disorders of psychological development), diagnostic criteria for research. International classification of Although there is a danger that children are diseases. 10th Revision. Geneva: WHO, 1990. assigned to a diagnostic slot because they do not 11 Van Krevelen DA. Early infantile autism and autistic psycho- pathy. J'ournal of Autism and Childhood fit, or that disagreement between experts leads 1971;1:82-6. to unnecessary confusion for the parents, 12 Wing L. Autism: possible clues to the underlying pathology-I: clinical facts. In: Wing L, ed. Aspects of Asperger's syndrome is, on balance, a useful autism:biological research. London: The National Autistic Society/Gaskell/Royal College of Psychiatrists, 1988:1-10. diagnosis. 13 Wing L. The autistic continuum. In: Wing L, ed. Aspects of There is a clear advantage in selecting specific autism:biological research. London: The National Autistic subgroups such as those with childhood autism Society/Gaskell/Royal College of Psychiatrists, 1988:v-viii. 14 Bishop DVM. Autism, Asperger's syndrome and semantic- or Asperger's syndrome as defined in ICD-10 pragmatic disorder: where are the boundaries? BrJ3 Disord for research purposes, because it may help dis- Commun 1989;24;107-2 1. 15 Szatmari P, Bremner R, Nagy J. Asperger's syndrome: a entangle the relationship between different review of clinical features. Can J Psychiatry 1989;34: aspects of development. Clinically the diagnosis 554-60. 16 Baron-Cohen S. Social and pragmatic deficit in autism:cogni- of Asperger's syndrome is valuable in that it tive or affective? J Autism Dev Disord 1988;18:379-402. draws attention to the fact that in young chil- 17 Frith U. Autism:possible clues to the underlying pathology- III: psychological facts 1988. In: Wing L, ed. Aspects of dren there may be significant abnormalities of autism:biological research. London: The National Autistic social development of autistic type even where Society/Gaskell/Royal College of Psychiatrists, 1988:19-30. 18 Frith U. A new look at language and communication in other aspects appear relatively normal. In addi- autism. Br J Disord Commun 1989;24:123-50. tion the specific diagnostic label can be of use to 19 Mundy P, Sigman M. The theoretical implications of joint- attention deficits in autism. Development and Psychopathol- some parents in helping them come to terms ogy 1989;1:173-83. with the nature of their children's disabilities. It 20 Hobson P. Concerning knowledge of mental states. BrJ Med Psychol 1990;63:199-214. can be helpful also in obtaining appropriate 21 Hobson RP. On sharing experiences, Development and resources. Because the different developmental 1989;1: 197-203. 22 Leslie AM, Happe F. Acute autism and ostensive communi- problems do not run in parallel, however, this cation: the relevance of metarepresentation. Development does not preclude the need to address the speci- and Psychopathology 1989;1:205-12. 23 Sigman M, Mundy P. Social attachments in autistic children. fic problems of individual children.'3 J Am Acad Child Adolesc Psychiatry 1989;28:74-81. 24 Courchesne E, Lincoln AJ, Yeung-Courchesne R, Elmasian R, Grillon C. Pathophysiological findings in non-retarded 1 Wing L. Asperger's syndrome: a clinical account. Psychol autism and receptive developmental language disorder. Med 1981;11:115-29. J Autism Dev Disord 1989;1:1-17. 2 Szatmari P, Bartolucci G, Bremner R. Asperger's syndrome 25 Rutter M, McDonald H, Le Couteur A, Harrington R, and autism: comparison offamily history and outcome. Dev Bolton P, Bailey A. Genetic factors in child psychiatric Med Child Neurol 1989;31:709. disorders-II empirical findings. J Child Psychol Psychiatry 3 Tantam D. Asperger's syndrome. J Child Psychol Psychiatry 1990;31:39-83. 1988;29:245-55. 26 Folstein SE, Rutter ML. Autism:familial aggregation and 4 Rutter M, Shaffer D, Sturge C. A guide to a multiaxial classifi- genetic implications. J Autism Dev Disord 1988;18:3-30. cation schemeforpsychiatric disorders in childhood and adolesc- 27 DeLong GR, Dwyer JT. Correlation of family history with ence. London: Institute of Psychiatry, 1975. specific autistic groups: Asperger's syndrome and bipolar 5 American affective . J Autism Dev Disord 1988;18:593-600. Psychiatric Association. Diagnostic and statistical http://adc.bmj.com/ manual of mental disorders: DSM-III. 3rd Ed. Washington 28 Gillberg C, Gillberg IC. Asperger's syndrome: some epide- DC: American Psychiatric Association, 1980. miological considerations: a research note. J Child Psychol 6 American Psychiatric Association. Diagnostic and statistical Psychiatry 1989;30:631-8. manual of mental disorders: DMS-III revised. 3rd Ed, 29 Wing L, Gould G. Severe impairments of social interaction revised. Washington DC: American Psychiatric Associa- and associated abnormalities in children: and tion, 1987. classifications. J Autism Dev Disord 1979;9:11-30. 7 Rutter M, Schopler E. Autism and pervasive developmental 30 Cantwell DP, Baker L, Rutter M, Mawhood L. Infantile aut- disorders: concepts of diagnostic issues. J Autism Dev ism and developmental receptive dysphasia: a comparative Disord 1987;17:159-86. follow-up into middle childhood. J Autism Dev Disord 8 Siegel B, Vukicevic J, Elliott GR, Kraemer HC. The use of 1989;19:19-31. on September 30, 2021 by guest. Protected copyright.