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Outcome in Autism and Autistic-like Conditions
Outcome in Autism and Autistic-like Conditions CHRISTOPHER GILLBERG, M.D.
Abstract. Aut ism carries a very variable prognosis; there might be a slight increase in mortality in the first 30 yea rs of life . A small, but not negligible , minority of people with autism lead productive, self-suppor ting adult lives , but about two-th irds will remain dependent on others throughout life. The risk of epilepsy is very high, both in earl y childhood and adolesce nce . An important mino rity deteriorate in adolescence . Outc ome in autistic-like conditions is eve n more variable , ranging from excellent in many cases of so-called Asperger syndrome to gloomy in most cases of so-ca lled disi ntegra tive diso rders. l. Am . Acad. Child Adolesc. Psychiatry, 1991 , 30 , 3:375-382 . Key Words: autism, Asperger syndro me, autistic- like conditio ns, pervasive deve lopmental disorders, outcom e .
Victor , the wild boy of Aveyron (Hard, 1801), was followed intensively for some years in later childhood. There can be little doubt that he suffered from childhood autism (Wing, 1980). Hard's meticulou s description of his progress has not been parallel ed in later follow-up studies of child ren with pervasive developm ental disorders. Victor made considerable gains in the activi ties of daily life , but it was clear that his outcome was severely restricted (Frith, 1989). Donald , the first case of autism described by Kanner (1943), had a better outcome than Victor in adult life. According to Kanner 's own description (Kanner, 1971), Donald, while still showing social deficit s, had a good job and was doing fairly well with regard to overall adjustment. In his first paper on "autistic psychopath y" (childhood personality disorde rs very similar to autism), Asperger suggested that outcome in high-level cases often was remarkably good (Asperger, 1944). Obviously, outcome in autism can vary considerably. Oddl y enough, in spite of these now historical descriptions that so clearl y illustrate this point , the systematic empirical evidence in the field is limited. The number of represent ative follow-up studies of autism and autistic-like conditi ons from childhood into late adolescence and adult life are easily counted . Lotter (1978) surveyed the follow-up studie s that had been published up until the late 1970s. He concluded that outcome was usuall y severely restr icted; more than half of all cases were totally depend ent on other adults in most aspects of everyday life . Th e few studies that have been published since then have supported this conclu sion. This paper review s the outcome of children who once suffered from autism and other disorders currentl y referred to as " pervasive developmental disorders, " with a special empha sis on the long-term follow-up studies from Goteborg,
Accepted November 8, 1990 . Prof essor Gil/berg is Medical Director at the Department of Pediatrics and Child and Adolescent Psychiatry. University ofGoteborg, Child Neuropsychiatry Centre, Goteborg, Sweden. Reprint requests to Professor Gil/berg. Department of Pediatrics and Child and Adolescent Psychiatry, University of Goteborg, Child Neuropsychiatry Centre. Box 17lJ 3, S-402 6/ Goteborg, Sweden 0890-85 67/9113003 -0375 $03.OO/0 © 1991 by the Ameri can Academy of Child and Adolescent Psychiatry. l.Am.A cad. Child Ado/esc. Psychiatry , 30 :3 , May 1991
Sweden. The paper is organized into the following sections: early autism outcome studies, recent autism outcome studies, outcome in autistic-like cond itions , autism in adulthood , and suggested avenues for future research.
Review of Early Outcome Studies of Autism In 1978, Victor Lotter reviewed the published studies in the field of autism outcome up to the mid 1970s. He found 21 studies describing some outcome aspects of children classified as psychotic in their early years. He excluded 13 of these either because diagnostic criteria were too uncertain, too few cases with clear infantile autism were included, or because the follow-up period was too short or not clearly specified. Less than a handful of outcome studies relating to autism have been published during the last 15 years (see below). The overall findings from the eight relevant studies reviewed by Lotter will be briefl y summarized here . Some of the data have been drawn directly from Lotter's review , but some data represent the result of computations made by the author on the basis of the " raw data " in the Lotter paper . Of the eight studie s considered by Lotter to be acceptable for the purpose of studying outcome in autism (Eisenberg, 1956; Creak, 1963; Mittler et aI. , 1966; Goldfarb , 1970; Rutter , 1970; Beeckrnan s-Balle, 1973; DeMyer et al. , 1973; Lotter, 1974a, b), four originated in the United Kingdom , three in the United States, and one in Belgium. The se studies included 474 cases of infantile autism diagnosed according to criteri a similar to those by Rutter ( 1978) or of ' 'childhood psychosis" /"childhood schizophrenia," which, in most cases (according to descriptions in the orig inal papers), tended to be very similar to the cases that had been diagnosed as suffering from autism . Even with in this set of reasonably acceptable autism outcome studies , there has probably been considerable diagno sic variability both within (a few) and across studies. In spite of the shortcomings of most of the studies reviewed by Lotter , and in spite of the fact that only one outcome study performed before 1975 report ed on a population-based series of autism cases, it seemed, at the time , that some relati vely firm conclu sion s could be drawn . First of all, outcome tended to be quite variable; many cases had extremely poor social and academic progress (e.g., 375
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Rutter, 1970), and others did very well, at least in the academic field (e.g., Kanner, 1971). Second, the overall trend was toward a poor prognosis. At least 313 of the 474 cases (66%) were placed in the poor outcome (severe handicap, no independent social progress) or very poor outcome (unable to lead any kind of independent existence) categories suggested by Lotter. Most acceptable outcome studies reported a similar percentage of poor or very poor outcomes . Third, apart from those with poor or very poor outcomes, at least seven were reported to have died between the ages of 2 and 30 years. This represents a proportion of 1.5% of the whole group and is higher than the corresponding frequency in the general population . In Sweden, the mortality rate in this age range in the late 1960s (a time when most of the studies surveyed by Lotter were completed) was 0.6% (data from the Swedish Central Bureau of Statistics). Fourth, there was a high rate of epilepsy, and new cases of seizures tended to accumulate at or after adolescence. However, age at onset of seizures was not reported by all authors. In those five acceptable outcome studies that reported on adolescent onset seizures, the mean rate was 11 % (30 out of 281 cases). This rate of adolescent onset epilepsy is likely to be an underestimate because quite a number of the cases with autism had not been followed through adolescence. In this connection , it is noteworthy that the overall rate of epilepsy in autism is much higher than 11 % (around one-third of all cases develop epilepsy in early childhood or adolescence). Fifth, a few studies reported dramatic adolescent changes in the mental/psychiatric domains in a subgroup of patients with autism. Rutter (1970) for instance, in his series of 63 clinical cases of "childhood psychosis," found seven who deteriorated (three of whom also had adolescent onset seizures). Other authors (most notably Kanner, 1971) claimed that several patients with autism began to improve considerably in the teenage period. Sixth, a small proportion of children diagnosed as suffering from autism in the 1940s, 1950s, and 1960s held jobs (mostly unskilled) or went to college in early adult life, seemingly regardless of treatment given. Altogether 16 of the 201 cases reported in Lotter 's overview (8%) who had reached the age of 16 years at the time of the follow-up were working, and one was a college student. This , too, is likely to be an underestimate since the length of the followup period usually did not extend well into adult age. However, there is also the risk that some of these relatively wellfunctioning people with autism might not in the long run be able to hold on to their jobs . Nevertheless, it is clear that cases with a relatively good prognosis are not quite so exceptional in the field of autism as is commonly believed . Finally, the only clear childhood predictor of outcome in almost all the studies is IQ. Children with very low IQ (in the severe mental retardation range) in early childhood are highly likely to remain low IQ scorers throughout their lives and hence have a "poor" prognosis . Outcome among those with higher IQ is much more variable and less predictable . Useful speech in early childhood (for instance at age 5 years) is also a good predictor of better outcome, but it is unclear 376
whether speech in itself predicts outcome or whether it is associated with good IQ and therefore with a generally better prognosis. The major problems with Lotter's useful review are associated with factors in the original studies that cannot be amended in retrospect: variable criteria for diagnosing autism, clinical rather than population-based samples, and a lack of standardized criteria for evaluating outcome.
Outcome in Typical Autism Cases-Recent Studies This section deals only with studies that report followup findings concerning children who have been diagnosed at one time in their lives (usually before the teenage period), as suffering from infantile autism or childhood autism, as defined by Rutter (1978), DSM-lll, Wing (1980), or Coleman and Gillberg (1985). Included among this group are children who have shown early onset concomitant social aloofness, severe abnormalities of communicative functioning (language, mime, and gesture), and elaborate repetitive behaviors (elaborate repetitive motor stereotypies or rituals and compulsions that are performed in a manner often described as "insistence on sameness "). This clinical picture is the one described in Kanner's original paper (Kanner 1943) and later condensed in the writings of Kanner and Eisenberg (1956). As has been pointed out already in the introduction , outcome in children with classic autism is variable, ranging from recovery or almost total recovery to extremes of social and communicative handicaps . The findings will be dealt with in subsections on: (I) those few who do well and those who make substantial but restricted progress, (2) the many who remain extremely handicapped, and (3) the minority who deteriorate and those who show exacerbation of problems in adolescence. An attempt will also be made to summarize predictors of good and poor outcome and to subdivide outcome categories according to etiological subgroups. Finally, some suggested avenues for future research will be discussed briefly. The Few Who Do Well and Those Who Make Substantial Progress Virtually all follow-up studies of autism report a small proportion of children who appear to do well or very well by adolescence or early adult life. Most experienced authors in the field have come across quite a number of children who appear to "grow out of autism." The majority of children with autism actually make some progress in their social functioning (in particular , there is often much less social aloofness) and hence may not be regarded as suffering from autism at a later age. However, the vast majority of these children remain socially severely restricted as adults . The number of "cures" or "extremely good outcomes ," i.e ., those who appear to be indistinguishable from "normal" is small, amounting to no more than a few percent in most studies (Lotter, 1978). The very few cases of children with autism who appear l.Am.Acad. Child Adolesc.Psychiatry, 30:3, May 1991
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"normal" by adult life usually indicate that the children have had some kind of treatment. Th is may lead to the erroneous conclusion that the treatment produced the cure. However , the variety of treatments given to such children and the fact that quite a number have not received any kind of treatment at all argues against the notion of a cause-effect link between treatment and cure. It seems much more likely that some, as yet unidentified , neurobiological phenomenon accounts for the "growing out of autism." A recent study by Szatmari and coworkers (1989) suggested that the number of very good outcome cases among high-functioning individuals with autism might be very high. In that study, nonverbal IQ at the time of diagnosis (under age 6 years) was more than 65 . The authors reported that at least half of a group of 16 children with high-level autism had obtained college degrees or were doing advanced college studies. The notion is conveyed that these patients had well-to-do parents who might have been exceptional in the way they managed to advocate forcefully for their child in terms of educational and recreation al resources. Surprisingly, the authors report that' ' the majority of the cases had a poor outcome." It is unclear from the results how the authors arrived at this conclusion. Nevertheless , those who may have had a very poor prognosis were excluded from the original group of 45 whom the authors planned to contact. One was excluded because his IQ went down , another one because he died, yet another one because of deafness, and a fourth one because as a child, there had been problems in assessing him. Twenty-five more cases were lost to follow-up for reasons that are not specified. In summary, it appears that this study by the Szatmari group may have included quite a number of cases with poor or very poor outcomes that were not reported in detail. Had they been included, the overall conclusions might have been very different. Even so, it appears that , at the very least , 15 to 20% of all high-functioning autistic children seen in a specialized center in Toronto before 1970 do well or very well in late adolescence to early adult life (age at follow-up was 17-34 years). Rumsey and her group (Rumsey et aI., 1985) recruited a group of adults with autism for follow-up after advertising in the National Society for Autistic Citizens Newsletter. Their study was also confined to the group with the best abilities and without complic ating handicaps in childhood , and their results were similar, though somewhat ' more pessimistic than those of Szatmari and coworkers (1989). The M an y Who Remain Extremely Handicapped
The author's own group (Gillberg and Steffenburg, 1987) recently reported follow-up results at age 16 to 23 years from a population-based group of children who had been diagnosed in early childhood as suffering from autism (N = 23)-and who retrospectively fulfilled DSM-llI. Rutter (1978), and Kanner and Eisenberg (1956) criteria for infantile autism-or " other childhood psychosis" (N = 23) . The results were very similar to the only previously published population-based series of autism cases (Lotter, 1974). l.Am .A cad. Child Adolesc. Psychiatry ,30 :3 , May 1991
Of the combined group with autism and autistic-like conditions ("other childhood psychosis" ) , 59% did poorly or extremely poorly in late adolescence and early adult life. Only 4% were almost indistingui shable from normal, and another 11 %, though still showing very odd behaviors , had made exceptional progress. There were no major differences in outcome between the two diagnostic groups. Two percent of the whole group died (at 8 years of age). Epilepsy occurrcd in 29% of the autism and 43% of the " other childhood psychosis" groups. Pubertal onset epilepsy was very common, and grand mal and psychomot or variants were the most frequent types of seizures. The findings with respect to epilepsy were very similar to those described almost 20 years ago by Rutter (1970) in a follow-up of some 60 clinically referred cases of autism and " other childhood psychosis ." However, they differ somewhat from those of two recent studies (one population-based by Olsson et al., 1988 and one large-scale clinical referral study by Volkmar and Nelson, 1990), both indicating that the greatest number of new epilepsy cases in autism occurred in early childhood. As in virtually all other studies in the field, IQ > 50 and communicative speech before the age of 6 years were the best predictors of a relatively good outcome. The vast majority of children with IQ < 50 and no communicative speech at the age of 6 years had a poor or very poor prognosis. Those Who Ha ve Severe Adolescent Problems
Perhaps the most surprising finding of this study was that .more than half of all cases in the follow-up study in adolescence showed either a temporary (30%) or a permanent (22%) aggravation of symptoms. There was no difference between " classic" autism and other childhood psychosis . The group with permanent deterioration was somewhat larger than in Rutter ' s (1970) follow-up study (12%). Deterioration was often, but by no means always, associated with pubertal onset epilepsy. The aggravation of symptoms was often seen as hyperactivity , aggressiveness , destructiveness-against self, rarely against others-and a return to insistence on sameness or unpredictability as often seen most clearly in the preschool years. The deterioration was often preceded by the same kind of symptoms that were then graduall y followed by inertia, loss of some language skills, and slow intellectual deterioration. There was a strong tendency for girls to be more often affected by deterioration than boys (in spite of the number of girls who were very small). Very often there was also a marked accentuation of periodicity with respect to problem severity around the time of puberty. Most of these children had shown periodicity already in early childhood. However , the problem s associated with great fluctuations of this kind became more conspicuous and more difficult to manage in adolescence. The reason for the problem s in puberty is unclear. In certain cases, it is obvious that they are the result of severe brain abnormalities , although not necessarily progres sive brain destruction or dysfunction. As demands on social adaptation and overall functioning increase with increasing 377
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age, the problems can become more difficult because of a lack of the availability of undamaged nerve tissue (which may well have resulted from damage suffered pre- or perinatally) rather than as a result of continuing loss of nerve capacity. Various kinds of neurohormonal changes might well be responsible also. A link with hereditary affective disorders has been suggested (Gillberg, 1984). It also seems clear that, in some instances, onset of epilepsy, whether associated with underlying progressive brain abnormalities or not, can trigger aggravation of symptoms and deterioration. This seemed to be the case in the present study and also in that of Rutter (1970). Sometimes pubertal problems result from the simple fact that the child grows bigger and stronger . Although he or she may still show the same level of overall functioning and behavioral problems, because of sheer body size and strength, things might become much more difficult to cope with for both family and staff. In some centers with very good educational treatment programs continuing throughout childhood, adolescence, and early adult life, pubertal problems are not thought to be so devastating (Mesibov, 1989, pers. commun.); perhaps coping strategies and useful everyday life skills have been taught from a very early age onward. Also , it is probably essential not to subject the child with autism to major changes in adolescence (such as change of school, teachers, and general support group). Unfortunately, in many countries at the moment, there is no coherent chain of services from childhood to adulthood for people with autism . The well-known problems with accepting sudden changes are often disregarded when "child services" and "adult services" draw the dividing line with regard to responsibilities. This state of affairs exists in education and psychiatry alike , at least in Sweden. It is likely that at least some of the major problems in adolescence can be attributed to such circumstances alone. In the Swedish study, it was sad to see how, around the time of puberty, the small minority who had received neuropsychopharmacological treatment in childhood had turned into a large majority in early adult life. In childhood , persons with autism were followed by professionals who were well informed about autism. In adult life, they were seen by psychiatrists who, knowing little about autism, thought that various kinds of bizarre behavior would probably respond well to neuroleptic treatment. Such treatment generally did not affect pubertal problems in autism positively, although in particular cases , they were sometimes very useful. Lithium treatment was sometimes tried in cases with marked periodicity, seemingly with good effect in several instances . The whole field of neuropsychopharmacology of autism (in particular, in adolescence and adult life) is in dire need of research (Campbell, 1989). With regard to high-functioning individuals with autism (and Asperger syndrome), case reports are now appearing that highlight the very special problems shown by some from adolescence onwards. It appears that a minority of individuals with autism in these cases , because of their inability to empathize with the perspectives of others and 378
because of their extreme repetitive interests, can get involved in severe "criminal" activities, such as violence (Baron-Cohen, 1988), fire setting (LeCouteur, 1989, pers . commun.), and even "murder"-such as in the case, investigated by the author, where there was an obsessional interest in poison . It is possible that in the area of "strange crime" one might find a disproportionate number of cases with high-functioning autism . Finally, in this section, it seems appropriate to point out again that there is overlap between the concept of autism and that of Asperger syndrome, such that a child diagnosed very early in life as suffering from autism might well grow up to fit the picture of Asperger syndrome. The reverse can also occur rarely.
Brief Summary of Outcome and Prognostic Factors Outcome in autism is quite variable (and in many cases clearly dependent on underlying causes). Slightly less than 10% do very well in adult life, hold jobs, and even have families in some cases . This proportion, of course, varies according to criteria used for diagnosing autism. Those children who have a tested performance IQ exceeding 70 and have some communicative speech around the age of 5 years and who have already at that age begun to show considerable improvement are the ones most likely to have a very favorable outcome in adult life, regardless of treatment given . Some 60% of the whole group of children with typical autism will grow up to be completely dependent on other adults in all aspects of life. IQ below 50 and lack of communicative speech at age 5 predict outcome in this category with some certainty, but one must keep in mind that a number of children with negative predictors do fairly well in adult life. It seems that early onset seizures, and perhaps adolescent onset seizures (Gillberg and Steffenburg, 1987), are also predictors of a relatively poor outcome. Between the poles of good and bad outcomes are a group of cases with clearly quite restricted outcome, but who nevertheless often do considerably better than had been expected on the basis of childhood evaluation. There is probably an increased mortality rate in autism, which, theoretically, may be associated with underlying severe progressive disorders, such as tuberous sclerosis . A large proportion of cases with autism have severe aggravation of symptoms in adolescence and an important minority actually deteriorate. There seems to be an association between adolescent deterioration on the one hand and severe mental retardation and epilepsy on the other. Perhaps there is also an increased risk of adolescent problems in girls over boys .
Outcome According to Etiology So far there is very little scientific evidence for a differential prognosis according to underlying causes in autism. However, a small proportion of cases of autism (3 to 5% according to Coleman and Gillberg, 1985) are causally related to severe tuberous sclerosis, which is known often to have a progressive downhill course with severe seizure disl.Am.Acad. Child Adolesc.Psychiatry, 30:3, May 1991
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order and kidney and heart failure. Premature mortality in this group is likely to be much increased . If the associated brain disorder in autism is the fragile X syndrome, then one must expect a rather different development through adolescence and adult life, even though the clinical picture in childhood may have been virtually identical to that seen in other cases of autism . It now seems that autism in the fragile X syndrome is characterized by hyperactivity, "nervousness," gaze avoidance and shyness (with characteristic turning-away-behavior upon greeting) and gradual development of clearer indications of a need for social interactions (Hagerman, 1989). If the underlying disorder is phenylketonuria (PKU) then dietary treatment (and the age at which it has been started) may change the course of the disorder completely. Cases with infantile spasms and rubella embryopathy have a very high incidence of autism and autistic behavior , but such behavior also appears to have a relatively fair prognosis in such cases (see Coleman and Gillberg , 1985 for a review). However, the empirical evidence supporting this notion of "transient autism" in infantile spasms and rubella embryopathy is weak. Girls with Rett syndrome are often classified as suffering from autism in the early stages of the disorder (Witt-Engerstrom and Gillberg, 1987). These girls usually grow out of the typical autism symptoms as they reach preadolescence. Obviously , in the longer perspective, it will become essential to pinpoint, as far as possible , the exact cause in each individual case of autism before making a prognostic decision . Autism will come to be seen as a label with similar implications as epilepsy and cerebral palsy: all three are unequivocal signs of underlying central nervous system dysfunction , and all three require extensive neurobiological work-up in order to determine underlying causes . Once the cause is known, outcome will be easier to predict.
Brief Overview of Studies Reporting Outcome in Autistic-like Conditions of Childhood At least two groups of disorders are relevant when discussing outcome in autistic-like conditions , as reported before 1980, namely, disintegrative psychosis and " schizoid personality. " These are now often referred to as childhood disintegrative disorders and Asperger syndrome, respectively (see below). There are also the categories of "non nuclear" autism cases (which have been included in the above section) and children with so-called' 'borderline psychosis" (which will be dealt with very briefly below) . Disintegrative psychosis (Corbett et aI., 1977; EvansJones and Rosenbloom, 1978), consists of autistic type symptomatology appearing after 2 to 3 years of normal development and a brief period of acute-subacute regression, confusion, and (usually) hyperactivity. This condition is very rare and has been the subject of very little systematic scientific enquiry. It seems that, except in very unusual cases, outcome is, if anything, poorer than in autism (Hill and Rosenbloom, 1986; Volkmar and Cohen, 1989). It is likely that degenerative or metabolic brain disorders are more commonly the cause of disintegrative psychosis than J. Am. Acad. Child Adolesc . Psychiatry, 30:3 , May 1991
of autism, which could account for prognostic differences . Nevertheless, both the causes and the long-term outcome of disintegrative psychosis are not well understood. It seems reasonable to assume that disintegrative psychosis is nothing but a conglomerate of psychiatric syndromes with a fairly typical phenotype but with a variety of underlying brain disorders . Some authors have highlighted the importance of possible associated "psychosocial stressors," such as hospitalization , travel abroad, and the birth of a sibling (EvansJones and Rosenbloom , 1978; Volkmar and Cohen, 1989). However , considering that such "events" are extremely common (at least as common in normality as in disintegrative psychosis) and that disintegrative disorders are extremely rare, it seems highly unlikely that they could be in any way causally related to the development of the disorder. Rett syndrome (Hagberg et aI., 1983) is now grouped among the childhood disintegrative disorders in the draft version of the ICD-1O (World Health Organization, 1988). This syndrome, before the late 1980s, was often labeled as autism, disintegrative psychosis, or degenerative brain disorder of unknown cause. Outcome in Rett syndrome is extremely poor with regard to motor and IQ development, but the outlook with regard to autistic-type symptoms (often extremely prominent in the initial stages of the disorder) is relatively better. Just recently, a number of publications (e.g., Gillberg, 1989; Burd et al. , 1989) have highlighted diagnostic difficulties in the field of Rett syndrome versus disintegrative disorders and autism . Burd and coworkers (1989) have suggested that disintegrative disorders should be subdivided into "Heller type" and "Rett type" and that they not be grouped among the developmental disorders (which is suggested in the latest draft version of the ICD10) for the simple reason that they do not show a developmental (but a disintegrative , deteriorating) course. Nevertheless, diagnostic difficulties in the field will remain since there are cases that show features of Heller syndrome, Rett syndrome, and Kanner syndrome, and, in which an argument could be made for diagnosing none, one, two, or three disorders in the same individual. Until diagnostic issues are settled, questions of prognosis will remain for children showing mixed features of Heller, Rett, and Kanner syndromes. Outcome in children with schizoid personality (Wolff and Chick, 1980) and in those who have received a diagnosis of "autistic psychopathy" (Asperger, 1944) or Asperger syndrome (Wing , 1981), on the whole, tends to be much better than that described for autism, but it is likely that superimposed psychiatric problems are common in adult life. However, Asperger noted that many had a good academic prognosis . The present author has seen a number of people with typical Asperger syndrome who not only hold qualified jobs but are also married and have children. Such people retain most or all of the oddities encountered in early childhood (social unawareness, lack of common sense , restricted , repetitive interests, and formal pedantic speech), but many of them do not come to the attention of psychiatrists. In Wolff and Chick's study (1980), one out of 22 people with Asperger-type problems developed schizophre379
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nia. Asperger (1944) stated that only one in 200 of his cases went on to develop symptoms of schizophrenia. Of the 18 patients in Wing's (1981) series, who were aged 16 and over at the time they were seen, one had an unconfirmed diagnosis of schizophrenia , one had psychosis with delusions and hallucination s that could not be classified , and one had an episode of catatonic stupor. In the present author' s experience , a wide variety of "bizarre" and so called " borderline" behaviors occur in late adolescence and adult life in Asperger syndrome, but classic schizophrenia is very rare. This is in line with Tantam 's (1986) experience. It is quite likely that classic schizophrenia may not occur at a higher than chance rate in Asperger syndrome, but that psychiatrists unacquainted with the clinical picture may find it difficult to evaluate " concrete ," " childish ," and "bizarre" symptoms in Asperger's syndrome and would be prone to diagnose "schizophreniform disorder" or a similar construct. There seems to be much more convincing evidence that there is an association between Asperger syndrome and affective illness/depression in adult life (Wing, 1981; Tantam , 1988). Among Wing's adult cases, more than 40% were considered to have affective illness or (possible) depression . Eleven percent had attempted suicide. Finally, in connection with schizoid personality disorder and Asperger syndrome, one must not forget that quite a number of children who were diagnosed as high-level autistic in early childhood conform to the "classical" picture of Asperger' s syndrome or are said to be typically schizoid in adult life. In this context, it is also appropriate to mention a group of children with early onset deficits in attention , motor control, and perception (Gillberg and Gillberg, 1989) who often have associated " autistic-like" or " Aspergertype" features. Although generally showing a much better outcome than children with autism, it seems that, at least throughout childhood and early adolescence, such children are severely restricted in their social relationships. The outcome in early onset schizophrenia is not known. The only available study detailing prognostic findings in a relatively large group of individuals, who appear to fulfill strict criteria for schizophrenia and with onset in the 7 to 13 years age range is that by Eggers (1978). He followed 57 such patients for an average of 15 years (6--40 years) after the clinical onset of the disorder. He found that 20% had recovered completely, that 50% had a relatively good or fair prognosis, and that only 30% had a poor or very poor prognosis. He also found , contrary to expectation , that in the good outcome group , more than half the cases had grown up in a disturbed family atmosphere . The proportion of disturbed family relationships was similar in the poor outcome group, which means that the family atmosphere did not predict outcome . Above average intelligenc e and a "syntonic premorbid personality " were associated with the best outcome. Eggers ( 1989) has also reported follow-up findings in a subgroup of the 57 schizophrenic cases. This subgroup met the Research Diagnostic Criteria for schizo-affective psychosis established by Spitzer et al. (1978). Altogether, 16 of the original cases met these criteria. It turned out that if 380
classified by this criterion, the schizo-affective group had a considerably better prognosis with only one in four showing a poor outcome as compared with the more " pure" schizophrenic cases, who showed a very restricted outcome in about 50%. Children with so called' 'borderline" conditions probably represent a group with a mixed bag of minor neurodevel opmental disorders (learning disorders , attention disorders , and specific developmental delays) and an amalgam of highly unspecific psychiatric problems (Gualtieri et aI., 1983). It is quite likely that such children receive a host of psychiatric diagnoses other than " borderline." A population-based study of deficits in attention, motor control , and perception from Goteborg, Sweden , has indicated that children diagnosed as suffering from a combination of such deficits (often referred to as minimal brain dysfunction type problems) often show " psychotic" or " autistic" features as well (Gillberg, 1983a). The long-term follow-up of such children (e.g., Gillberg, 1987) is very variable . A majo rity tend to have a somewhat restricted social prognosis (at least in the late teens), some become delinquent, and some have an excellent prognosis. Autism in Adulthood There is not enough room here for an exhaustive review of autism in adulthood . Suffice it to say that children diagnosed as suffering from autism do not all fit one "diagnostic shape" as they become adults. Wing (1983) has suggested that there are three major groups of behaviors typical of adults who were once diagnosed as suffering from early childhood autism. One group of individual s remains aloof and seemingly indifferent to the compan y of other people . Another group is passive and friendly and accepts the company and approaches made by others as long as there are no major upsets of routines. The third group is described as active but odd and reported to make socially inappropriate approaches. Results from the Goteborg group have supported Wing's categorie s (Gillberg, 1983b; Gillberg et al. , 1986). The study by Gillberg and Steffenburg (1987) suggested that about 50% of a population-based series of childhood autism cases belong to the first group in adult life , 25% to the second , and 25% to the third. For a recent good summary of the current state of knowledge in this field, the reader is referred to Wing (1989).
Avenues for Future Research There is now a great need for detailed longitudin al followup data from all the population-based groups of cases with autism and autistic-like conditions that have been reported in the literature over the last 25 years. Follow-up of clinical samples cannot contribute a great deal to our understanding of the course in autism, because problems with the representativeness of such samples will always arise. Follow-up studies should make use of stringent and generally accepted criteria for diagnosing autism and autistic-like conditions, standardized IQ-instruments (such as the WISC and the WAIS), standardized autism rating scales (such as the Child J . Am. A cad . Child Adolesc. Psychiatry, 30:3. May 1991
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hood Autism Rating Scale), standardized measures of social functioning (such as the Vineland Social Maturit y Scale), and currently availa ble neurobiological examination tools. New instruments will have to be developed , particularly for studying underl ying cognitive psychological problems (such as the possible lack of a theory of mind in autism [Frith, 1989]), if we are to acquire a bette r concept of the various subgroup s in adulth ood. Adolescence is a critical period in autism, and the longitudin al follow-u p studies should pay special attention to this age group. Finally, detailed scrutiny of development of autism-type problems in all the various etiological subgroups (tuberous scleros is, fragile X, PKU, etc.) , will probably prove enormously helpful for a better understanding of the true nature of the basic deficits in autism.
References Asperger, H. (1944) , Die autis tischen Psychopathe n im Kindesalter. Archiv fiir Psychiatrie und Nerve nkra nkheiten , 117:76-136. Baron -Cohen. S. (1988 ) . An assessme nt of violence in a young man with Aspe rger' s syndrome . J . Child Psychol. Psychiatry , 29:351360. Beeckmans-Balle , M . (197 3), Le syndrome d 'aut isme infantile. Acta Psychiatr. Belg . , 73 :537-555 . Burd , L. , Fishe r, W . & Kerbeshian , J. ( 1989), Pervasive disin tegrative disorder: are Rett syndrome and Heller dementia infantilis subtypes? Dev. Med . Child Neu rol., 3 1:609-6 16. Campbell , M . (1989 ), Pharmac othera py in autism: an overv iew. In: Diagnosis and Treatment of Autism , ed. C . Gill berg. New York : Plen um Press. Coleman, M . & Gill berg, C. (1985 ), The Biology of the A utistic Syndrom es . New York: Praeger. Corbett, J ., Harr is, R. , Taylor , E. & Tr imble , M. (1987) , Progressive disintegrative psychosis of childhoo d. J . Child Psy chol. Psychiatry, 18:211-219. Creak, M. (196 3) , Child hoo d psychosis: a review of 100 cases . Br . J. Psy chiatry , 109:84-89. DeMyer , M . K., Barton, S. , DeM yer, W . E . , Norton, J. A . , Allen, J . & Steele , R. (1973), Prognosis in autism : a follo w-up study. J. Autism and Childhood Schi zoph renia, 3:199-246. Eggers, C. (1978), Cou rse and prognosis of childhoo d schizo phre nia . Journal of A utism and Childhood Sch izophrenia, 8:2 1-36 . - - (1989 ), Sc hizo-affec tive psychoses in chil dhoo d: a follow-up study. J. Autism Dev , Disord .• 19 :327- 334. Eisenberg , L. (1956 ), The autis tic child in adolescence . Am . J . Psychiatry , 112:607-6 12. Evans-Jones, L. G . & Rosenbloom, L. (1978), Disintegrative psychosi s in childhood . Dev. M ed . Child N eurol ., 20:462-470 . Frith, V . ( 1989) , Au tism. Explaining the En igma . Oxford: Basil Blac kwell. Gillberg, C . (1983a), Perce ptua l, motor and atten tional defic its in Swedish primary schoo l children . Some child psychiatric aspects. J . Child Psycho l. Psychiatry, 24 :377--403. - - (l983b), Psychotic behaviour in children and young adults in a ment al handicap hostel. Acta Pedia tr . Scand. , 68 :351-358 . - - (1984), Auti stic children grow ing up . Problems of pube rty and adolescence . Dev . M ed . Chil d N eurol., 26 :125- 129. - - (1989), The borderland of autism and Rett syndrome: five case historie s to highli ght diag nostic dif ficult ies. J . A utism Dev. Di sord. , 19:545-559 . - - Steffenburg, S . ( 19 87) , Outcome and prognostic factors in infantile autis m and similar conditions: a populatio n-based study of 46 cases follow ed through puberty. J . Autism Dev . Disord . , 17 :273287 . J.Am .Aca d . Child A do/esc. P sy chiatry, 30:3 , May 19 9/
- Grufrnan, M. . Persson, E. & Themner, U. (1986), Psychiatric disorders in mildly and severely ment ally retarded children. Br . J. Psychiatry . 149 :68- 74. Gillber g, I. C. (1987) , Def icits in attention, motor control and p erception. Fo llow-up fro m pre schoo l to the early tee ns. Master ' s thesis, University of Uppsa la and Univer sity of Goteborg , Goteb org , Sweden. - - ( 1989), Childhoo d disintegrative dis order: a lon gitu dinal follow up study. - - Gillberg , C . (1989), Children with pres chool minor neur odevelopmental disorders. IV . Behaviou r and school achieve ment at age -
13 . Dev. Med . Child Neurology , 3 1:3- 13. Goldfarb , W. (1970). A follow-up inves tigatio n of schizophrenic children treated in residence . Psyc hoso cial Process, 1:9-64 . Gualtieri , C . T ., Koria th, U. & van Bourgond ien , M . E . (1983), " Border-line" children. J . Autism Dev , Disord ., 13:67- 7 3. Hagberg , B., Aica rdi , J., Dias , K. & Ramos, O. (1983 ), A progressive syndro me of autism , dementi a, ataxi a and loss of pur poseful hand use in girls: Rett 's synd rome . Report of 35 cases. Ann . Neu rol . 14:471--479 . Hagerman, R. ( 1989) , Ch romosomes and genes. In : Diag nosis and Treatment of Autism. ed . C . Gi llberg. New York: Plenum Press . Hill. A. E. & Rosenbl oom . L. (1986) , Disintegrative psychosis of childhood : teenage follow -up. Dev . Med . Child Neurol, 28 :34--40. Itard , J .M.G . (1964 ), Mernoire et rapp ort sur Victor de l'A veyron . In: Les Enfa nts Sa uvag es, ed . L. Malson. Par is: Union Generale d'Editions (Original wor k published 1801). Kanner , L. (1943 ), Autistic disturbances of affective co ntact. N er vous Child, 2:217-250 . - - (197 1), Follow-up study of eleven children originally repo rted in 1943. Journal of Autism and Chi ldh ood S chi zophrenia , 1:119145 . - - Eisenberg, L. (1956), Early infantile autis m, 1943-55, Am . J . Orthopsychiatry, 26 :55-65 . Lotter, V. (1974) , Fac tors related to outcome in autistic children . Journal of Autism and Childhood Schizophrenia , 4 :263-277 . - - ( 1978), Follow -up studi es. In: Autism. A Reappraisal ofCon cept s and Trea tment, eds . M. Rutter & E. Sc hopler. New York : Plenu m Press. Mittler, P., Gillies, S . & Jukes , E. (1966), Prognosis in psychotic children: report of a fo llow- up study . J . Me lli . D efic , Res . , 10:7 383 . Olsso n,!. , Gillberg , C . & Ste ffenburg , S . (1988), Epilepsy in autism and autisticlike conditions: a pop ulation-based study . Arch. Neuro l. , 45 :666-668 . Rumsey, J. M. , Rapoport, J . L. & Sceery , W. R. (1985) Aut istic children as adults : psyc hiatric and behavioral ou tco mes . J . A m . Acad . Child Psychiatry , 24 :465--473. Rutter , M. (1970 ), Autistic children: infancy to adulthoo d. Se mina rs ill Psychiatry , 2:435--450 . - - (1978), Diagnosis and Definition. In: A utism . A Reapp raisal of Concepts and Trea tment , eds . M . Rutter & E . Schopler. Ne w York : Plenum Press, pp. 1- 25. Spi tzer , R . L., Endicott, J . & Robins, E . (197 8), Research Diagn ostic Criteria: ration ale and reli abilit y. Arch . Ge n. Psychiatry , 35 :773782 . Swed ish Cen tral Burea u of Statis tics (199 1), Demographic Data . Orebro , Swe den: Swe dish Ce ntral Bur eau of St atistics . Szatrnari, P. , Bartolucci , G . , Brenn er , R., Bond , S . & Rich , S. (1989) , A fo llow-study of high-functioning autistic children. J. A utis m Del'. Disord . , 19:213-225 . Tantam , D . (1986), Ecc entricity and A utism . A doctoral dissertation , University of London, Lond on. - - (1988 ), Asperger's syndrome . J . Child Psy ch ol. Psych iatry , 29:245-255 . Volkmar, F. R . & Co hen, D. J. (1989), Disintegrative disord er or " late onset" autism . J . Child Psych ol. Psy ch iatry , 30 :717-724 . - - Nelson, D . S . (1990), Sei zure disorders in autism . J . Am . Acad . Child Adolesc. Psy chiatry , 29: 127- 129 . Wor ld Health Orga nization ( 1988) , Int ernat ional Classification ofDi s-
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eases and Disorders (lCD-lO), (Draft version). Geneva: World Health Org anization . Wing , L. (1980), Early Childhood Autism, 2nd editi on. Oxford : Pergam on Pre ss. - - (1981), Asperger's syndrome: a clini cal account. Psychol. Med ., 11:115-129 . - - (198 3), Social and interperso nal needs. In : Autism in Adolescents and Adults, eds . E. Sch opler & G . B . Mesibov. New York: Plenum
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Press , pp . 337- 354. - - (1989) , Autistic adults . In : Diagnosis and Treatment of Au tism , ed . C. Gillberg . Ne w York : Plenum Press. Witt-Eng erstrorn, I. & Gillberg , C. (1987), Rett sy ndrome in Sweden . J , Autism Dev. Disord., 17:149-150 . Wolff, S . & Chick , J. (198 0), Schizoid per son alit y in childhood : a con trolled follow-up study . Psychol . Med ., 10:85-1 00.
l .Am.Acad . Child Adolesc . Psychiatry, 30 :3, May 1991
Abstract. Aut ism carries a very variable prognosis; there might be a slight increase in mortality in the first 30 yea rs of life . A small, but not negligible , minority of people with autism lead productive, self-suppor ting adult lives , but about two-th irds will remain dependent on others throughout life. The risk of epilepsy is very high, both in earl y childhood and adolesce nce . An important mino rity deteriorate in adolescence . Outc ome in autistic-like conditions is eve n more variable , ranging from excellent in many cases of so-called Asperger syndrome to gloomy in most cases of so-ca lled disi ntegra tive diso rders. l. Am . Acad. Child Adolesc. Psychiatry, 1991 , 30 , 3:375-382 . Key Words: autism, Asperger syndro me, autistic- like conditio ns, pervasive deve lopmental disorders, outcom e .
Victor , the wild boy of Aveyron (Hard, 1801), was followed intensively for some years in later childhood. There can be little doubt that he suffered from childhood autism (Wing, 1980). Hard's meticulou s description of his progress has not been parallel ed in later follow-up studies of child ren with pervasive developm ental disorders. Victor made considerable gains in the activi ties of daily life , but it was clear that his outcome was severely restricted (Frith, 1989). Donald , the first case of autism described by Kanner (1943), had a better outcome than Victor in adult life. According to Kanner 's own description (Kanner, 1971), Donald, while still showing social deficit s, had a good job and was doing fairly well with regard to overall adjustment. In his first paper on "autistic psychopath y" (childhood personality disorde rs very similar to autism), Asperger suggested that outcome in high-level cases often was remarkably good (Asperger, 1944). Obviously, outcome in autism can vary considerably. Oddl y enough, in spite of these now historical descriptions that so clearl y illustrate this point , the systematic empirical evidence in the field is limited. The number of represent ative follow-up studies of autism and autistic-like conditi ons from childhood into late adolescence and adult life are easily counted . Lotter (1978) surveyed the follow-up studie s that had been published up until the late 1970s. He concluded that outcome was usuall y severely restr icted; more than half of all cases were totally depend ent on other adults in most aspects of everyday life . Th e few studies that have been published since then have supported this conclu sion. This paper review s the outcome of children who once suffered from autism and other disorders currentl y referred to as " pervasive developmental disorders, " with a special empha sis on the long-term follow-up studies from Goteborg,
Accepted November 8, 1990 . Prof essor Gil/berg is Medical Director at the Department of Pediatrics and Child and Adolescent Psychiatry. University ofGoteborg, Child Neuropsychiatry Centre, Goteborg, Sweden. Reprint requests to Professor Gil/berg. Department of Pediatrics and Child and Adolescent Psychiatry, University of Goteborg, Child Neuropsychiatry Centre. Box 17lJ 3, S-402 6/ Goteborg, Sweden 0890-85 67/9113003 -0375 $03.OO/0 © 1991 by the Ameri can Academy of Child and Adolescent Psychiatry. l.Am.A cad. Child Ado/esc. Psychiatry , 30 :3 , May 1991
Sweden. The paper is organized into the following sections: early autism outcome studies, recent autism outcome studies, outcome in autistic-like cond itions , autism in adulthood , and suggested avenues for future research.
Review of Early Outcome Studies of Autism In 1978, Victor Lotter reviewed the published studies in the field of autism outcome up to the mid 1970s. He found 21 studies describing some outcome aspects of children classified as psychotic in their early years. He excluded 13 of these either because diagnostic criteria were too uncertain, too few cases with clear infantile autism were included, or because the follow-up period was too short or not clearly specified. Less than a handful of outcome studies relating to autism have been published during the last 15 years (see below). The overall findings from the eight relevant studies reviewed by Lotter will be briefl y summarized here . Some of the data have been drawn directly from Lotter's review , but some data represent the result of computations made by the author on the basis of the " raw data " in the Lotter paper . Of the eight studie s considered by Lotter to be acceptable for the purpose of studying outcome in autism (Eisenberg, 1956; Creak, 1963; Mittler et aI. , 1966; Goldfarb , 1970; Rutter , 1970; Beeckrnan s-Balle, 1973; DeMyer et al. , 1973; Lotter, 1974a, b), four originated in the United Kingdom , three in the United States, and one in Belgium. The se studies included 474 cases of infantile autism diagnosed according to criteri a similar to those by Rutter ( 1978) or of ' 'childhood psychosis" /"childhood schizophrenia," which, in most cases (according to descriptions in the orig inal papers), tended to be very similar to the cases that had been diagnosed as suffering from autism . Even with in this set of reasonably acceptable autism outcome studies , there has probably been considerable diagno sic variability both within (a few) and across studies. In spite of the shortcomings of most of the studies reviewed by Lotter , and in spite of the fact that only one outcome study performed before 1975 report ed on a population-based series of autism cases, it seemed, at the time , that some relati vely firm conclu sion s could be drawn . First of all, outcome tended to be quite variable; many cases had extremely poor social and academic progress (e.g., 375
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Rutter, 1970), and others did very well, at least in the academic field (e.g., Kanner, 1971). Second, the overall trend was toward a poor prognosis. At least 313 of the 474 cases (66%) were placed in the poor outcome (severe handicap, no independent social progress) or very poor outcome (unable to lead any kind of independent existence) categories suggested by Lotter. Most acceptable outcome studies reported a similar percentage of poor or very poor outcomes . Third, apart from those with poor or very poor outcomes, at least seven were reported to have died between the ages of 2 and 30 years. This represents a proportion of 1.5% of the whole group and is higher than the corresponding frequency in the general population . In Sweden, the mortality rate in this age range in the late 1960s (a time when most of the studies surveyed by Lotter were completed) was 0.6% (data from the Swedish Central Bureau of Statistics). Fourth, there was a high rate of epilepsy, and new cases of seizures tended to accumulate at or after adolescence. However, age at onset of seizures was not reported by all authors. In those five acceptable outcome studies that reported on adolescent onset seizures, the mean rate was 11 % (30 out of 281 cases). This rate of adolescent onset epilepsy is likely to be an underestimate because quite a number of the cases with autism had not been followed through adolescence. In this connection , it is noteworthy that the overall rate of epilepsy in autism is much higher than 11 % (around one-third of all cases develop epilepsy in early childhood or adolescence). Fifth, a few studies reported dramatic adolescent changes in the mental/psychiatric domains in a subgroup of patients with autism. Rutter (1970) for instance, in his series of 63 clinical cases of "childhood psychosis," found seven who deteriorated (three of whom also had adolescent onset seizures). Other authors (most notably Kanner, 1971) claimed that several patients with autism began to improve considerably in the teenage period. Sixth, a small proportion of children diagnosed as suffering from autism in the 1940s, 1950s, and 1960s held jobs (mostly unskilled) or went to college in early adult life, seemingly regardless of treatment given. Altogether 16 of the 201 cases reported in Lotter 's overview (8%) who had reached the age of 16 years at the time of the follow-up were working, and one was a college student. This , too, is likely to be an underestimate since the length of the followup period usually did not extend well into adult age. However, there is also the risk that some of these relatively wellfunctioning people with autism might not in the long run be able to hold on to their jobs . Nevertheless, it is clear that cases with a relatively good prognosis are not quite so exceptional in the field of autism as is commonly believed . Finally, the only clear childhood predictor of outcome in almost all the studies is IQ. Children with very low IQ (in the severe mental retardation range) in early childhood are highly likely to remain low IQ scorers throughout their lives and hence have a "poor" prognosis . Outcome among those with higher IQ is much more variable and less predictable . Useful speech in early childhood (for instance at age 5 years) is also a good predictor of better outcome, but it is unclear 376
whether speech in itself predicts outcome or whether it is associated with good IQ and therefore with a generally better prognosis. The major problems with Lotter's useful review are associated with factors in the original studies that cannot be amended in retrospect: variable criteria for diagnosing autism, clinical rather than population-based samples, and a lack of standardized criteria for evaluating outcome.
Outcome in Typical Autism Cases-Recent Studies This section deals only with studies that report followup findings concerning children who have been diagnosed at one time in their lives (usually before the teenage period), as suffering from infantile autism or childhood autism, as defined by Rutter (1978), DSM-lll, Wing (1980), or Coleman and Gillberg (1985). Included among this group are children who have shown early onset concomitant social aloofness, severe abnormalities of communicative functioning (language, mime, and gesture), and elaborate repetitive behaviors (elaborate repetitive motor stereotypies or rituals and compulsions that are performed in a manner often described as "insistence on sameness "). This clinical picture is the one described in Kanner's original paper (Kanner 1943) and later condensed in the writings of Kanner and Eisenberg (1956). As has been pointed out already in the introduction , outcome in children with classic autism is variable, ranging from recovery or almost total recovery to extremes of social and communicative handicaps . The findings will be dealt with in subsections on: (I) those few who do well and those who make substantial but restricted progress, (2) the many who remain extremely handicapped, and (3) the minority who deteriorate and those who show exacerbation of problems in adolescence. An attempt will also be made to summarize predictors of good and poor outcome and to subdivide outcome categories according to etiological subgroups. Finally, some suggested avenues for future research will be discussed briefly. The Few Who Do Well and Those Who Make Substantial Progress Virtually all follow-up studies of autism report a small proportion of children who appear to do well or very well by adolescence or early adult life. Most experienced authors in the field have come across quite a number of children who appear to "grow out of autism." The majority of children with autism actually make some progress in their social functioning (in particular , there is often much less social aloofness) and hence may not be regarded as suffering from autism at a later age. However, the vast majority of these children remain socially severely restricted as adults . The number of "cures" or "extremely good outcomes ," i.e ., those who appear to be indistinguishable from "normal" is small, amounting to no more than a few percent in most studies (Lotter, 1978). The very few cases of children with autism who appear l.Am.Acad. Child Adolesc.Psychiatry, 30:3, May 1991
LONGIT UDIN AL RESEA RCH: O UTCOME IN AUTI SM
"normal" by adult life usually indicate that the children have had some kind of treatment. Th is may lead to the erroneous conclusion that the treatment produced the cure. However , the variety of treatments given to such children and the fact that quite a number have not received any kind of treatment at all argues against the notion of a cause-effect link between treatment and cure. It seems much more likely that some, as yet unidentified , neurobiological phenomenon accounts for the "growing out of autism." A recent study by Szatmari and coworkers (1989) suggested that the number of very good outcome cases among high-functioning individuals with autism might be very high. In that study, nonverbal IQ at the time of diagnosis (under age 6 years) was more than 65 . The authors reported that at least half of a group of 16 children with high-level autism had obtained college degrees or were doing advanced college studies. The notion is conveyed that these patients had well-to-do parents who might have been exceptional in the way they managed to advocate forcefully for their child in terms of educational and recreation al resources. Surprisingly, the authors report that' ' the majority of the cases had a poor outcome." It is unclear from the results how the authors arrived at this conclusion. Nevertheless , those who may have had a very poor prognosis were excluded from the original group of 45 whom the authors planned to contact. One was excluded because his IQ went down , another one because he died, yet another one because of deafness, and a fourth one because as a child, there had been problems in assessing him. Twenty-five more cases were lost to follow-up for reasons that are not specified. In summary, it appears that this study by the Szatmari group may have included quite a number of cases with poor or very poor outcomes that were not reported in detail. Had they been included, the overall conclusions might have been very different. Even so, it appears that , at the very least , 15 to 20% of all high-functioning autistic children seen in a specialized center in Toronto before 1970 do well or very well in late adolescence to early adult life (age at follow-up was 17-34 years). Rumsey and her group (Rumsey et aI., 1985) recruited a group of adults with autism for follow-up after advertising in the National Society for Autistic Citizens Newsletter. Their study was also confined to the group with the best abilities and without complic ating handicaps in childhood , and their results were similar, though somewhat ' more pessimistic than those of Szatmari and coworkers (1989). The M an y Who Remain Extremely Handicapped
The author's own group (Gillberg and Steffenburg, 1987) recently reported follow-up results at age 16 to 23 years from a population-based group of children who had been diagnosed in early childhood as suffering from autism (N = 23)-and who retrospectively fulfilled DSM-llI. Rutter (1978), and Kanner and Eisenberg (1956) criteria for infantile autism-or " other childhood psychosis" (N = 23) . The results were very similar to the only previously published population-based series of autism cases (Lotter, 1974). l.Am .A cad. Child Adolesc. Psychiatry ,30 :3 , May 1991
Of the combined group with autism and autistic-like conditions ("other childhood psychosis" ) , 59% did poorly or extremely poorly in late adolescence and early adult life. Only 4% were almost indistingui shable from normal, and another 11 %, though still showing very odd behaviors , had made exceptional progress. There were no major differences in outcome between the two diagnostic groups. Two percent of the whole group died (at 8 years of age). Epilepsy occurrcd in 29% of the autism and 43% of the " other childhood psychosis" groups. Pubertal onset epilepsy was very common, and grand mal and psychomot or variants were the most frequent types of seizures. The findings with respect to epilepsy were very similar to those described almost 20 years ago by Rutter (1970) in a follow-up of some 60 clinically referred cases of autism and " other childhood psychosis ." However, they differ somewhat from those of two recent studies (one population-based by Olsson et al., 1988 and one large-scale clinical referral study by Volkmar and Nelson, 1990), both indicating that the greatest number of new epilepsy cases in autism occurred in early childhood. As in virtually all other studies in the field, IQ > 50 and communicative speech before the age of 6 years were the best predictors of a relatively good outcome. The vast majority of children with IQ < 50 and no communicative speech at the age of 6 years had a poor or very poor prognosis. Those Who Ha ve Severe Adolescent Problems
Perhaps the most surprising finding of this study was that .more than half of all cases in the follow-up study in adolescence showed either a temporary (30%) or a permanent (22%) aggravation of symptoms. There was no difference between " classic" autism and other childhood psychosis . The group with permanent deterioration was somewhat larger than in Rutter ' s (1970) follow-up study (12%). Deterioration was often, but by no means always, associated with pubertal onset epilepsy. The aggravation of symptoms was often seen as hyperactivity , aggressiveness , destructiveness-against self, rarely against others-and a return to insistence on sameness or unpredictability as often seen most clearly in the preschool years. The deterioration was often preceded by the same kind of symptoms that were then graduall y followed by inertia, loss of some language skills, and slow intellectual deterioration. There was a strong tendency for girls to be more often affected by deterioration than boys (in spite of the number of girls who were very small). Very often there was also a marked accentuation of periodicity with respect to problem severity around the time of puberty. Most of these children had shown periodicity already in early childhood. However , the problem s associated with great fluctuations of this kind became more conspicuous and more difficult to manage in adolescence. The reason for the problem s in puberty is unclear. In certain cases, it is obvious that they are the result of severe brain abnormalities , although not necessarily progres sive brain destruction or dysfunction. As demands on social adaptation and overall functioning increase with increasing 377
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age, the problems can become more difficult because of a lack of the availability of undamaged nerve tissue (which may well have resulted from damage suffered pre- or perinatally) rather than as a result of continuing loss of nerve capacity. Various kinds of neurohormonal changes might well be responsible also. A link with hereditary affective disorders has been suggested (Gillberg, 1984). It also seems clear that, in some instances, onset of epilepsy, whether associated with underlying progressive brain abnormalities or not, can trigger aggravation of symptoms and deterioration. This seemed to be the case in the present study and also in that of Rutter (1970). Sometimes pubertal problems result from the simple fact that the child grows bigger and stronger . Although he or she may still show the same level of overall functioning and behavioral problems, because of sheer body size and strength, things might become much more difficult to cope with for both family and staff. In some centers with very good educational treatment programs continuing throughout childhood, adolescence, and early adult life, pubertal problems are not thought to be so devastating (Mesibov, 1989, pers. commun.); perhaps coping strategies and useful everyday life skills have been taught from a very early age onward. Also , it is probably essential not to subject the child with autism to major changes in adolescence (such as change of school, teachers, and general support group). Unfortunately, in many countries at the moment, there is no coherent chain of services from childhood to adulthood for people with autism . The well-known problems with accepting sudden changes are often disregarded when "child services" and "adult services" draw the dividing line with regard to responsibilities. This state of affairs exists in education and psychiatry alike , at least in Sweden. It is likely that at least some of the major problems in adolescence can be attributed to such circumstances alone. In the Swedish study, it was sad to see how, around the time of puberty, the small minority who had received neuropsychopharmacological treatment in childhood had turned into a large majority in early adult life. In childhood , persons with autism were followed by professionals who were well informed about autism. In adult life, they were seen by psychiatrists who, knowing little about autism, thought that various kinds of bizarre behavior would probably respond well to neuroleptic treatment. Such treatment generally did not affect pubertal problems in autism positively, although in particular cases , they were sometimes very useful. Lithium treatment was sometimes tried in cases with marked periodicity, seemingly with good effect in several instances . The whole field of neuropsychopharmacology of autism (in particular, in adolescence and adult life) is in dire need of research (Campbell, 1989). With regard to high-functioning individuals with autism (and Asperger syndrome), case reports are now appearing that highlight the very special problems shown by some from adolescence onwards. It appears that a minority of individuals with autism in these cases , because of their inability to empathize with the perspectives of others and 378
because of their extreme repetitive interests, can get involved in severe "criminal" activities, such as violence (Baron-Cohen, 1988), fire setting (LeCouteur, 1989, pers . commun.), and even "murder"-such as in the case, investigated by the author, where there was an obsessional interest in poison . It is possible that in the area of "strange crime" one might find a disproportionate number of cases with high-functioning autism . Finally, in this section, it seems appropriate to point out again that there is overlap between the concept of autism and that of Asperger syndrome, such that a child diagnosed very early in life as suffering from autism might well grow up to fit the picture of Asperger syndrome. The reverse can also occur rarely.
Brief Summary of Outcome and Prognostic Factors Outcome in autism is quite variable (and in many cases clearly dependent on underlying causes). Slightly less than 10% do very well in adult life, hold jobs, and even have families in some cases . This proportion, of course, varies according to criteria used for diagnosing autism. Those children who have a tested performance IQ exceeding 70 and have some communicative speech around the age of 5 years and who have already at that age begun to show considerable improvement are the ones most likely to have a very favorable outcome in adult life, regardless of treatment given . Some 60% of the whole group of children with typical autism will grow up to be completely dependent on other adults in all aspects of life. IQ below 50 and lack of communicative speech at age 5 predict outcome in this category with some certainty, but one must keep in mind that a number of children with negative predictors do fairly well in adult life. It seems that early onset seizures, and perhaps adolescent onset seizures (Gillberg and Steffenburg, 1987), are also predictors of a relatively poor outcome. Between the poles of good and bad outcomes are a group of cases with clearly quite restricted outcome, but who nevertheless often do considerably better than had been expected on the basis of childhood evaluation. There is probably an increased mortality rate in autism, which, theoretically, may be associated with underlying severe progressive disorders, such as tuberous sclerosis . A large proportion of cases with autism have severe aggravation of symptoms in adolescence and an important minority actually deteriorate. There seems to be an association between adolescent deterioration on the one hand and severe mental retardation and epilepsy on the other. Perhaps there is also an increased risk of adolescent problems in girls over boys .
Outcome According to Etiology So far there is very little scientific evidence for a differential prognosis according to underlying causes in autism. However, a small proportion of cases of autism (3 to 5% according to Coleman and Gillberg, 1985) are causally related to severe tuberous sclerosis, which is known often to have a progressive downhill course with severe seizure disl.Am.Acad. Child Adolesc.Psychiatry, 30:3, May 1991
LONGITUDINAL RESEARCH : OUTCOME IN AUTISM
order and kidney and heart failure. Premature mortality in this group is likely to be much increased . If the associated brain disorder in autism is the fragile X syndrome, then one must expect a rather different development through adolescence and adult life, even though the clinical picture in childhood may have been virtually identical to that seen in other cases of autism . It now seems that autism in the fragile X syndrome is characterized by hyperactivity, "nervousness," gaze avoidance and shyness (with characteristic turning-away-behavior upon greeting) and gradual development of clearer indications of a need for social interactions (Hagerman, 1989). If the underlying disorder is phenylketonuria (PKU) then dietary treatment (and the age at which it has been started) may change the course of the disorder completely. Cases with infantile spasms and rubella embryopathy have a very high incidence of autism and autistic behavior , but such behavior also appears to have a relatively fair prognosis in such cases (see Coleman and Gillberg , 1985 for a review). However, the empirical evidence supporting this notion of "transient autism" in infantile spasms and rubella embryopathy is weak. Girls with Rett syndrome are often classified as suffering from autism in the early stages of the disorder (Witt-Engerstrom and Gillberg, 1987). These girls usually grow out of the typical autism symptoms as they reach preadolescence. Obviously , in the longer perspective, it will become essential to pinpoint, as far as possible , the exact cause in each individual case of autism before making a prognostic decision . Autism will come to be seen as a label with similar implications as epilepsy and cerebral palsy: all three are unequivocal signs of underlying central nervous system dysfunction , and all three require extensive neurobiological work-up in order to determine underlying causes . Once the cause is known, outcome will be easier to predict.
Brief Overview of Studies Reporting Outcome in Autistic-like Conditions of Childhood At least two groups of disorders are relevant when discussing outcome in autistic-like conditions , as reported before 1980, namely, disintegrative psychosis and " schizoid personality. " These are now often referred to as childhood disintegrative disorders and Asperger syndrome, respectively (see below). There are also the categories of "non nuclear" autism cases (which have been included in the above section) and children with so-called' 'borderline psychosis" (which will be dealt with very briefly below) . Disintegrative psychosis (Corbett et aI., 1977; EvansJones and Rosenbloom, 1978), consists of autistic type symptomatology appearing after 2 to 3 years of normal development and a brief period of acute-subacute regression, confusion, and (usually) hyperactivity. This condition is very rare and has been the subject of very little systematic scientific enquiry. It seems that, except in very unusual cases, outcome is, if anything, poorer than in autism (Hill and Rosenbloom, 1986; Volkmar and Cohen, 1989). It is likely that degenerative or metabolic brain disorders are more commonly the cause of disintegrative psychosis than J. Am. Acad. Child Adolesc . Psychiatry, 30:3 , May 1991
of autism, which could account for prognostic differences . Nevertheless, both the causes and the long-term outcome of disintegrative psychosis are not well understood. It seems reasonable to assume that disintegrative psychosis is nothing but a conglomerate of psychiatric syndromes with a fairly typical phenotype but with a variety of underlying brain disorders . Some authors have highlighted the importance of possible associated "psychosocial stressors," such as hospitalization , travel abroad, and the birth of a sibling (EvansJones and Rosenbloom , 1978; Volkmar and Cohen, 1989). However , considering that such "events" are extremely common (at least as common in normality as in disintegrative psychosis) and that disintegrative disorders are extremely rare, it seems highly unlikely that they could be in any way causally related to the development of the disorder. Rett syndrome (Hagberg et aI., 1983) is now grouped among the childhood disintegrative disorders in the draft version of the ICD-1O (World Health Organization, 1988). This syndrome, before the late 1980s, was often labeled as autism, disintegrative psychosis, or degenerative brain disorder of unknown cause. Outcome in Rett syndrome is extremely poor with regard to motor and IQ development, but the outlook with regard to autistic-type symptoms (often extremely prominent in the initial stages of the disorder) is relatively better. Just recently, a number of publications (e.g., Gillberg, 1989; Burd et al. , 1989) have highlighted diagnostic difficulties in the field of Rett syndrome versus disintegrative disorders and autism . Burd and coworkers (1989) have suggested that disintegrative disorders should be subdivided into "Heller type" and "Rett type" and that they not be grouped among the developmental disorders (which is suggested in the latest draft version of the ICD10) for the simple reason that they do not show a developmental (but a disintegrative , deteriorating) course. Nevertheless, diagnostic difficulties in the field will remain since there are cases that show features of Heller syndrome, Rett syndrome, and Kanner syndrome, and, in which an argument could be made for diagnosing none, one, two, or three disorders in the same individual. Until diagnostic issues are settled, questions of prognosis will remain for children showing mixed features of Heller, Rett, and Kanner syndromes. Outcome in children with schizoid personality (Wolff and Chick, 1980) and in those who have received a diagnosis of "autistic psychopathy" (Asperger, 1944) or Asperger syndrome (Wing , 1981), on the whole, tends to be much better than that described for autism, but it is likely that superimposed psychiatric problems are common in adult life. However, Asperger noted that many had a good academic prognosis . The present author has seen a number of people with typical Asperger syndrome who not only hold qualified jobs but are also married and have children. Such people retain most or all of the oddities encountered in early childhood (social unawareness, lack of common sense , restricted , repetitive interests, and formal pedantic speech), but many of them do not come to the attention of psychiatrists. In Wolff and Chick's study (1980), one out of 22 people with Asperger-type problems developed schizophre379
GILLBERG
nia. Asperger (1944) stated that only one in 200 of his cases went on to develop symptoms of schizophrenia. Of the 18 patients in Wing's (1981) series, who were aged 16 and over at the time they were seen, one had an unconfirmed diagnosis of schizophrenia , one had psychosis with delusions and hallucination s that could not be classified , and one had an episode of catatonic stupor. In the present author' s experience , a wide variety of "bizarre" and so called " borderline" behaviors occur in late adolescence and adult life in Asperger syndrome, but classic schizophrenia is very rare. This is in line with Tantam 's (1986) experience. It is quite likely that classic schizophrenia may not occur at a higher than chance rate in Asperger syndrome, but that psychiatrists unacquainted with the clinical picture may find it difficult to evaluate " concrete ," " childish ," and "bizarre" symptoms in Asperger's syndrome and would be prone to diagnose "schizophreniform disorder" or a similar construct. There seems to be much more convincing evidence that there is an association between Asperger syndrome and affective illness/depression in adult life (Wing, 1981; Tantam , 1988). Among Wing's adult cases, more than 40% were considered to have affective illness or (possible) depression . Eleven percent had attempted suicide. Finally, in connection with schizoid personality disorder and Asperger syndrome, one must not forget that quite a number of children who were diagnosed as high-level autistic in early childhood conform to the "classical" picture of Asperger' s syndrome or are said to be typically schizoid in adult life. In this context, it is also appropriate to mention a group of children with early onset deficits in attention , motor control, and perception (Gillberg and Gillberg, 1989) who often have associated " autistic-like" or " Aspergertype" features. Although generally showing a much better outcome than children with autism, it seems that, at least throughout childhood and early adolescence, such children are severely restricted in their social relationships. The outcome in early onset schizophrenia is not known. The only available study detailing prognostic findings in a relatively large group of individuals, who appear to fulfill strict criteria for schizophrenia and with onset in the 7 to 13 years age range is that by Eggers (1978). He followed 57 such patients for an average of 15 years (6--40 years) after the clinical onset of the disorder. He found that 20% had recovered completely, that 50% had a relatively good or fair prognosis, and that only 30% had a poor or very poor prognosis. He also found , contrary to expectation , that in the good outcome group , more than half the cases had grown up in a disturbed family atmosphere . The proportion of disturbed family relationships was similar in the poor outcome group, which means that the family atmosphere did not predict outcome . Above average intelligenc e and a "syntonic premorbid personality " were associated with the best outcome. Eggers ( 1989) has also reported follow-up findings in a subgroup of the 57 schizophrenic cases. This subgroup met the Research Diagnostic Criteria for schizo-affective psychosis established by Spitzer et al. (1978). Altogether, 16 of the original cases met these criteria. It turned out that if 380
classified by this criterion, the schizo-affective group had a considerably better prognosis with only one in four showing a poor outcome as compared with the more " pure" schizophrenic cases, who showed a very restricted outcome in about 50%. Children with so called' 'borderline" conditions probably represent a group with a mixed bag of minor neurodevel opmental disorders (learning disorders , attention disorders , and specific developmental delays) and an amalgam of highly unspecific psychiatric problems (Gualtieri et aI., 1983). It is quite likely that such children receive a host of psychiatric diagnoses other than " borderline." A population-based study of deficits in attention, motor control , and perception from Goteborg, Sweden , has indicated that children diagnosed as suffering from a combination of such deficits (often referred to as minimal brain dysfunction type problems) often show " psychotic" or " autistic" features as well (Gillberg, 1983a). The long-term follow-up of such children (e.g., Gillberg, 1987) is very variable . A majo rity tend to have a somewhat restricted social prognosis (at least in the late teens), some become delinquent, and some have an excellent prognosis. Autism in Adulthood There is not enough room here for an exhaustive review of autism in adulthood . Suffice it to say that children diagnosed as suffering from autism do not all fit one "diagnostic shape" as they become adults. Wing (1983) has suggested that there are three major groups of behaviors typical of adults who were once diagnosed as suffering from early childhood autism. One group of individual s remains aloof and seemingly indifferent to the compan y of other people . Another group is passive and friendly and accepts the company and approaches made by others as long as there are no major upsets of routines. The third group is described as active but odd and reported to make socially inappropriate approaches. Results from the Goteborg group have supported Wing's categorie s (Gillberg, 1983b; Gillberg et al. , 1986). The study by Gillberg and Steffenburg (1987) suggested that about 50% of a population-based series of childhood autism cases belong to the first group in adult life , 25% to the second , and 25% to the third. For a recent good summary of the current state of knowledge in this field, the reader is referred to Wing (1989).
Avenues for Future Research There is now a great need for detailed longitudin al followup data from all the population-based groups of cases with autism and autistic-like conditions that have been reported in the literature over the last 25 years. Follow-up of clinical samples cannot contribute a great deal to our understanding of the course in autism, because problems with the representativeness of such samples will always arise. Follow-up studies should make use of stringent and generally accepted criteria for diagnosing autism and autistic-like conditions, standardized IQ-instruments (such as the WISC and the WAIS), standardized autism rating scales (such as the Child J . Am. A cad . Child Adolesc. Psychiatry, 30:3. May 1991
L ON GITUDI N A L RESEA RCH : OU TCOME IN A UTISM
hood Autism Rating Scale), standardized measures of social functioning (such as the Vineland Social Maturit y Scale), and currently availa ble neurobiological examination tools. New instruments will have to be developed , particularly for studying underl ying cognitive psychological problems (such as the possible lack of a theory of mind in autism [Frith, 1989]), if we are to acquire a bette r concept of the various subgroup s in adulth ood. Adolescence is a critical period in autism, and the longitudin al follow-u p studies should pay special attention to this age group. Finally, detailed scrutiny of development of autism-type problems in all the various etiological subgroups (tuberous scleros is, fragile X, PKU, etc.) , will probably prove enormously helpful for a better understanding of the true nature of the basic deficits in autism.
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