Asperger's Syndrome and Autism: Neurocognitive Aspects

PETER SZATMARI, M.D., LAWRENCE TUFF, PH.D., M. ALLEN J. FINLAYSON, PH.D., GIAMPIERRO BARTOLUCCI, M.D.
AND

Abstract. The objectives of this study were to see: (1) whether children with Asperger's Syndrome (AS) have similar neurocognitive deficits compared to nonretarded, or high. functioning autistic (HFA) children; and (2) whether the essential cognitive deficit among these children is in language or abstract problem solving . Subjects with AS, HFA , and a control group of socially impaired child psychiatric outpatient controls (OPe) were compared on a battery of neuropsychological tests. The results indicated that the AS and HFA groups differed little but that large differences from the OPC were observed on all tests. When the AS and HFA with FSIQ above 85 were compared to the OPC, outstanding deficits on motor coordination , language comprehension, and facial recognition were observed. Finally, some evidence is presented to suggest that the pattern of deficits of AS and HFA subjects varied by developmental level. The implications of these results for a neurological theory of autism are discussed. J. Am. Acad. ChildAdolesc. Psych iatry, 1990,29, 1:130-136. Key Words: autism, Asperger's syndrome, cognitive
The term Asperger's Syndrome (AS) has appeared with increasing frequency in published reports, case studies, and commentaries over the past several years (Wing and Gould, 1979; Wolff and Barlow , 1979; Wolff and Chick, 1980; Wing, 1981; Burgoine and Wing, 1983; Gillberg , 1985; Mawson et al. , 1985; Schopler, 1985; Nagy and Szatmari , 1986; Tantum, 1988). The disorder , first described by Asperger in 1944 (Asperger, 1944), is characterized by social isolation and odd or eccentric behavior in childhood. These children are often described by their parents as affectionate during infancy but show extreme social isolation from their peers. They are socially clumsy and engage in essentially one-sided social interactions. While their speech is not noticeably deviant, children with AS have difficulty with the pragmatics of communication (i.e. , problems in initiating and sustaining a conversation). They often show a paucity of gestures and facial expression or have difficulty judging "social distance." Frequently, they develop intense interests in often obscure subjects (i.e., floor plans to buildings , sharks, washing machines, etc.), but sometimes these preoccupations can be difficult to differentiate from more normal interests (i.e. , wrestling, horror movies, sport statistics). In view of the subtlety and mildness of many of the symptoms, children with AS are often given other diagnoses and, up to fairly recently, were rarely considered to have a form of pervasive developmental disorder (PDD). Although these children have also been associated with other diagnostic labels (i. e., atypical, pseudo-neurotic schizophrenic, autistic psychopathy , borderline , schizotypal, and schizoid), there is emerging agreement on the essential clinical features of the syndrome (Nagy and SzatAccepted May 12, 1989. Dr . Szatmari is Associate Professor, Dr . Tuffis Assistant Professor, Dr. Finlayson is Professor and Dr. Bartolucci is Professor, Department of Psychiatry. McMaster University. Hamilton. Ontario. The authors wish to thank the staff of the West End Creche for their help in data collection. This research was supported by a grant from the Medical Research Council of Canada . 0890-8567/90/290I-0130$2 .0010 1990 by the American Academy of Child and Adolescent Psychiatry .

mari, 1986). These include social isolation and abnormal interactions with peers , impairments in verbal and nonverbal communication, and early developmental delays (Szatmari et al., 1989). While published case reports (Burgoine and Wing, 1983; Gillberg , 1985,; Mawson et al. , 1985; Volkmar et al. , 1985) suggest AS may be considered a mild variant of autism, it is clear that children with the disorder differ from the majority of autistic children who are without communicative language, are mentally retarded, and have a poor prognosis. It is less clear whether subjects with AS are distinguishable from high-functioning (HFA) or nonretarded autistic children. Recent reports (Wing, 1981; Szatmari et al., 1990) do suggest that the two groups can be discriminated on the basis of their clinical features (particularly social responsiveness, imaginative play , bizarre preoccupations) and outcome, although these differences probably reflect severity rather than a different etiology. Whether AS and HFA are distinguishable neurocognitively , however, has not been directly addressed. The diagnostic validity of a psychiatric disorder such as AS can be strengthened if a specific deficit, or pattern of deficits, is evident on psychological tests. Although the authors were not able to identify any papers on AS, two .studies were found that examined the neurocognitive profiles of children with " nonautistic" forms of PDD (a group that includes children with AS). Wolff and Barlow (1979) compared schizoid (i.e. , AS-like children), high-functioning autistic, and controls on a variety of tests and concluded that those with schizoid disorders had similar deficits in language and visual association to autistic children but less perseverative responses. Pomeroy and Friedman (1987), on the other hand, compared 15 boys (4 to 13 years of age) diagnosed as either "atypical" or "childhood onset" PDD, with psychiatric outpatient controls on the Kaufman Assessment Battery (Kaufman et al. , 1983). Significant differences between the groups were found only among the " holistic" processing (visual-perceptual) subtests rather than the verbal subtests, suggesting a pattern quite different than that seen in autism. These two studies, then, have produced somewhat contradictory findings. Much more work has been done on neurocognitive pro130

ASPERGER'S SYNDROME AND AUTISM

files of autistic children. Until recently, the main conclusion has been that these children show relative deficits on tests of language, particularly comprehension, and relative .strengths on visual-perceptual tasks. This finding has been extended to include other left-hemisphere tasks and, as a result, a left-hemisphere dysfunction hypothesis has been proposed for autism (Hoffman and Prior, 1982; Dawson , 1983). More recently, Rumsey and Hamburger (1988) have shown, in a sample of 10 nonretarded autistic males (18 to 39 years of age), outstanding deficits on tests measuring abstract problem solving. Using the Wisconsin Card Sorting Test (WCST) and subtests from the Stanford-Binet , the autistic men obtained very poor scores compared to 'normal volunteers. No outstanding deficits were noted on either language or visual-perceptual tests, nor were there lateralized sensory or motor weaknesses . Rumsey and Hamburger's (1988) results support a dysfunction in frontal-subcortical systems rather than a left-hemisphere deficit. Finally, Fein et al. (1985) argue that there are distinct subtypes of neurocognitive profiles among autistic children and have reported an elegant cluster analysis to support their hypothesis. There appear, then, to be two outstanding issues with respect to neuro-cognitive impairments in PDD children: (1) do children with AS have a different cognitive profile from autistic children , and (2) is the core cognitive deficit in language or problem solving? The present study was designed to address these issues by comparing AS and autistic children and a consecutive series of cases referred to a mental health outpatient clinic for a variety of social problems. The latter comparison group was chosen to determine if the neurocognitive correlates of AS are different from those found in socially impaired children who are not PDD (Rourke, 1982). If no differences are found on this comparison , these cognitive deficits cannot be used to explain the essential nature of the PDD syndrome. The subjects for this study were part of a larger project designed to investigate the diagnostic validity of AS as a disorder distinct from autism. Previous reports document comparisons across early history and outcome (Szatmari et aI., 1990), perception of social information (Szatmari et aI., submitted), and linguistic functioning (Fine et aI., 1989).

TABLE

1. Sample Characteristics Autistic 17 22.8 (7-32) 4/13 82.2 Outpatient Control 36 13.7a (7- 18) 6/30 101.5b

Asperger's Syndrome
N Age (yr) Range Sex (F/M) Mean IQ 26 14.3 (8- 18) 5121 86.6

aHFA > AS = OPC. b OPC > AS = HFA .

Method
Sample

Child psychiatrists and developmental pediatricians working at a children's mental health center were sent descriptions of odd and eccentric children and were asked to refer any such child between the ages of 8 and 18 (Table 1). A standard assessment by the senior author (P. S.) was carried out to determine whether or not the child had AS. This assessment, which was unstructured, included intensive interviews with the parents and child and discussions with the child's teacher . The inclusion criteria for AS, adapted from Wing (1981), stipulated that a child show all of: (1) isolated behavior; (2) impaired social interaction; (3) one of odd speech, impaired nonverbal communication, or bizarre preoccupations; and (4) onset prior to 6 years of age (Szatl.Am.Acad. Child Adolesc .Psychiatry, 29:1 .Jan.1990

mari et aI. , 1989). The AS sample consisted of28 children, five females and 23 males: one child did not complete the testing protocol. The average age was 14 years with a range from 8 to 18 years. Previous chart diagnoses for the AS group included, among others, hyperactivity, anxiety disorders, learning disability , schizoid disorder, borderline schizophrenia , obsessive-compulsive disorder, and adjustment reaction. All children had consistently proved to be serious diagnostic puzzles to previous clinicians . No AS ' child had ever been diagnosed as autistic . Since high-functioning autistic (HFA) children are quite rare, they had to be recruited from two sources. The autistic service at our Centre had a small number (N = 5) of nonretarded autistic children on its caseload. These children have been independently diagnosed by two physicians (a pediatrician and child psychiatrist) as autistic according to DSM-III criteria . All these children were enrolled in the study. The second source of HFA children was from the West End Creche (WEC) in Toronto, a center specializing in the treatment of autistic children since 1950. The charts of all children who had been seen at the Creche were reviewed. Any child who received a diagnosis of autism, childhood schizophrenia or childhood psychosis was identified (these diagnoses were used interchangeably at the WEC over the years) . The diagnostic criteria for these three diagnoses were identical and included: (1) qualitative impairments in social relationships, (2) deficits in communicative abilities, and (3) various abnormal behaviors (such as stereotypies or insistence on sameness) . Any child with this diagnosis whose most recent IQ test score was above 65 (usually according to the Stanford-Binet) was included. Children without an IQ score were also included to avoid missing any nonretarded autistic children . The authors were able to identify 84 children who met these criteria. The parents of 41 children were traced and agreed to participate in this study. On subsequent testing , the diagnosis of autism was confirmed in 20 subjects who had an IQ score over 70. The autistic sample then, consisted of 25 individuals (five from Chedoke, 20 from WEC). The average age was 23 years but ranged from 7 to 32 years. Seventeen (17) HFA students completed the neuropsychological protocol; the other eight were either unavailable (i.e., out of town) or else did not wish to participate in this component of the project. A second comparison group consisted of children with a variety of peer problems. A consecutive series of children referred to the child and adolescent psychiatric outpatient teams presenting with some type of social impairment noted

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TABLE

2. Clinical Differences between AS and HFA Group (%) Asperger's (N = 27)

Variable Social impairments Lack of social responsiveness to mother Enjoys other adults Shows gaze avoidance Shares interests with parents Affectionate baby Complete lack of interest in peers Language and communication Babbled less than siblings Echolalia/pronoun reversal Repetitive speech Seldom starts a conversation Lack of imaginative play Range of interests Stereotypies Rituals Insistence on routines Bizarre preoccupations

(N

Autistic = 24)

p Value

25 80 36 68 67 38 24 37 22 59 12 48 13 30 37

72 34 50 33 33 72 65 75 48 65 48 86 25 50 86

0 .000 0.004 0.48 0.03 0.04 0.02 0.009 0.007 0.04 0.88 0.02 0.003 0.46 0.23 0.000

history and behaviors seen in PDD. Details on the interview are available elsewhere, as well as results on clinical comparisons between the AS and HFA subjects (Szatmari et al., 1990). For purposes of illustration, data from a number of questions that reflect DSM -III criteria are reported here to demonstrate differences between the AS and HFA groups on early history (see Table 2). It is clear that the HFA subjects demonstrated greater impairment on all measures of social responsiveness except gaze avoidance. Similarly, the groups were different on all language and communication measures except one item reflecting pragmatics (i.e., "seldom starts a conversation") . On the other hand, the groups had similar rates of "rituals" and "insistence on routines." Rates of "stereotypies" and "bizarre preoccupation" were strikingly different. These results support the notion that while AS can be considered a type of PDD, there are significant clinical differences between AS and HFA on clinical presentation.
Measures

on the intake referral sheet were eligible for inclusion. This could include comments such as "being a loner," "shys away from other children," "social withdrawal," "fights with friends," etc. These children had the usual psychiatric disorders of childhood and adolescence with the added provision that there was a problem in getting along with other children of the same age, for whatever reason. The children also had to be age- (within 2 years) and sex-matched to the AS group and could not have a history of brain damage (such as epilepsy or head injury). The authors were able to enroll 42 such children as out-patient controls (OPC), 34 males and eight females; 36 of these completed the neuropsychological testing. The average age was 14, ranging from seven to 17. Twenty-five (25) of the 36 OPC were given a psychiatric diagnosis on the parent version of the Diagnostic Interview for Children and Adolescents (Reich et al., 1982). The largest group (10) were those with both attention deficit disorder with hyperactivity (ADDH) and conduct disorder (CD). An additional three children had ADDH alone; four had CD alone; five had a pure anxiety disorder; and the final three had both CD and an anxiety disorder. The age, sex, and IQ characteristics of the AS, autistic, and control subjects who completed the testing are shown in Table 1. One-way analysis of variance indicated significant effects for age and FSIQ with the autistic subjects older than the AS and control subjects (p < 0.05). Controls had significantly higher IQs than either AS or autistic subjects (p < 0.05). Before testing, the subject's mother was interviewed by a research assistant, blind to diagnosis, using a structured interview. This instrument contains questions about early
132

Neurocognitive tests were selected to sample a wide range of abilities and included several tests previously reported to measure deficits in autistic children. Accordingly, subjects were assessed on measures of: (1) intelligence (i.e., WISC-R and WAIS-R) (Wechsler, 1974, 1981); (2) school achievement, (i.e. , WRAT-R) (Jastak and Jastak, 1984); (3) auditory comprehension and memory , (i.e., Children's Token Test) (Di Simoni, 1977); (4) verbal problem solving, (i.e., Children's Word Finding Test~WFT) (Pajurkova et al., 1976); (5) facial recognition (i.e ., Benton Test of Facial Recognition-BTFR) (Benton et al., 1983); (6) graphomotor construction (i.e., Developmental Test of Visual Motor Integration-DTVMI) .(Beery, 1982); (7) cognitive flexibility (i.e., Wisconsin Card Sorting Test-WCST) (Heaton, 1981); and (8) manual speed and dexterity (Grooved Pegboard Test) (Knights and Norwood, 1979). All tests were administered according to the standard instructions prescribed in the published manuals or reports. / 2 hours and were Testing sessions lasted approximately 31 usually completed in a single sitting. Raw scores were converted to standard scores using published age norms. In the case of several tests, normative information was not available for older children or adults (i.e., CWFT, DTVMI). In . these instances, standard scores for the DTVMI were therefore based on 13- to 14-year norms. Because the performance of older children on the CWFT approaches adult levels, this was not considered to be a problem. Total scores were used for all tests except the WRAT-R and WCST. On the WRAT-R, the reading and math subtests were used, while on the WCST, the number of correct categories achieved, the total number of errors, and the percentage of perseverative errors committed were used.
Analysis

First, a one-way analysis of variance was carried out on all tests to see whether mean scores differed between the three groups. A discriminant function analysis was then performed to see which measures differentiated the AS and HFA groups. An analysis of covariance could not be perJ .Am.Acad. Child Adolesc. Psychiatry, 29:1 ,Jan. 1990

ASPERGER'S SYNDROME AND AUTISM

TABLE

3. Group Task Performance (Total Sample)

TABLE

4. Group Task Performance (Total Sample)

p Value Asperger's Autistic OPC for (N = 26) (N = 17) eN = 36) ANOVA

ope p Value Asperger's Autistic eN = 26) eN = 17) (N = 36) ANOVA

VIQ Information Similarities Arithmetic Vocabulary Comprehension Digit Span PIQ Picture Completion Picture Arrangement Block Design Object Assembly Coding

85.8 7.93 8.50 7.66 8.14 6.31 8.4 1 87.8 7.69 8.44 8.92 8.14 7.30

84.5 7.66 6.76 7.72 7.75 6.88 8.59 81.4 7.36 7.57 8.41 7.81 5.86

101.6 9.52 10.48 10.12 10.24 11.35 9.37 101.4 9.79 10.81 10.72 10.96 9.19

0.000" 0.024" 0.00()b 0.011" 0.002" 0.000" 0.53Q< 0.000" 0.007" 0.001" 0.043" 0.000" 0.005"

Note: Standard scores are presented. a = OPC > AS = HFA . b = OPC > AS > HFA . c Nonsignificant.

WRAT Reading Arithmetic Read-FSIQ Math-FSIQ Token Test CWFT Pegs-dominant Pegs-nondominant DTVMI BTFR WSCT % Perseverative error Categories Errors

98.8 85.1 9.1 -1.8 40.3 31.4 27.1 25.9 34.7 37.3 33.6 40.2 42.0

105.5 86.8 10.6 1.4 43.9 34.2 29.6 41.0 32.6 39.1 24.4 34.7 33.8

101.4 88.8 0.6 -8.7 53.1 41.3 49.4 46.3 42.5 45.2 44.8 4i6 45.4

0.63' 0.27' 0.000" 0.000" 0.002" 0.016' 0.000" O.OOl d 0.0060.023'

0.003b 0.0060.006b

formed on the test battery since the assumption of parallel lines was violated for several of the variables (Le., the relationship between IQ and several tests varied by group) . To deal with this problem , the ANOVAs were rerun only in PDD and ope subjects with FSIQ above 85. To control for the effects of multiple testing, a discriminant function analysis was then carried out to see which variables distinguished the PDD and ope groups. Finally, to see whether PDD children were heterogeneous in terms of neurocognitive impairment, the PDD children were divided into those with FSIQ above and below 85. Their score on each test was subtracted from their own IQ scores to get an idea of cognitive deficits relative to IQ. These discrepancy scores among the low- and high-IQ PDD subjects were compared to see whether the cognitive profile of PDD children varies by IQ. Results The mean scores obtained by the AS, autistic, and control groups on all of the neurocognitive measures are shown in Tables 3 and 4. Table 3 provides information on the Wechsler subtests. The groups differed on all subtests, except for digit span. Appropriate post-hoc tests indicated that (other than for similarities) the out-patient controls performed better than the AS and autistic groups (who were not different from each other). In terms of level of performance, the AS and HFA groups showed significant impairments on both verbal and performance subtests and were quite similar to each other. Table 4 summarizes the neuropsychological and achievement test results. Looking first at the absolute WRAT-R scores, there were no differences between the groups. If, however, one calculates a discrepancy score between the standard score on reading and arithmetic, minus their FSIQ, significant differences emerge. Both the AS and the autistic groups were reading roughly 10 points above their IQ, whereas on arithmetic, the control group was performing roughly 10 points below their IQ. On the Token Test , Pegl.Am.Acad. Child Adolesc. Psychiatry, 29:1 . Ja n . 1990

Note : Except for standard scores on the WRAT, all other scoresare transformed to T-scores with a mean of 50 and SD of 10. " OPC> AS = HFA . b OPC > HFA. ' OPC > AS. dHFA = OPC > AS. , Nonsignificant.

board (dominant), and the DTVMI, both PDD subtypes performed worse than the ope (but not different from each other). On the eWFT, Pegboard (nondominant), and the BTFR, only the ope and AS groups were different. Finally , on the 3 subjects ofthe weST, the autistic group was worse than ope. In general, one or the other of the PDD subtypes was significantly different from the ope on all tests , although whether the AS or HFA group obtained lower scores varied. It was apparent, on these univariate analyses, that there were small differences between the two PDD subtypes. A discriminant function analysis, specifically comparing AS and HFA subjects, was carried out to explore this in more detail (not shown). Four variables discriminated the two groups: (1) Pegboard performance with the nondominant hand; (2) percent perseverative errors from the WeST; (3) Pegboard performance with the dominant hand; and (4) the number of errors committed on the WeST. The HFA did not show the expected dominant hand advantage, in contrast to the AS group, and did worse on the WeST subtests. However, the multivariate Wilks was barely significant (p = 0.048) and the classification matrix indicated that only two-thirds of each group were correctly classified. Thus, while some differences exist between AS and HFA, it seemed appropriate to combine these groups into one category (PDD subjects) for further analyses. The next issue investigated was whether PDD and ope subjects differed on their test results , taking account of the large differences in IQ between the groups. The comparison was carried out only on those subjects with FSIQ above 85, since an analysis of covariance could not be run (see Analy s is ) . There were now 22 PDD children with FSIQ above
133

SZATMARI ET AL.
TABLE

5. PDD" vs. OPC (Only in those with FSIQ >85)

PDD'
(N = 22)
(N

TABLE

OPC

6. Discriminant Function Analysis (PDD vs. OPC) Neuropsychological Tests

Variables Pegs-Dominant Comprehension Pegs -Nondominant BTFR . Wilks

= 36)

p Value

Step . No .

p Value

VIQ PIQ FSIQ Information Similarities Arithmetic Vocabulary Comprehension Digit Span Picture Completion Picture Arrangement Block Design Object Assembly Coding BTFR DTVMI Pegs-dominant Pegs-nondominant WCST-% Perseverative errors WCST -Categories WCST-no. of errors Token Test CWFT Read ing Math Read-FSIQ Math-FSIQ PDD
(n
b

48.6 47.1 48.7 49.4 48.9 51.3 50.0 44.7 52.8 44.5 48.2 52.9 47.6 45.1 38.7 39.9 31.4 36.1 39.6 44.8 43 .3 51.8 42.4 57.1 46.1 8.5 - 2.4

51.7 51.5 51.6 48.7 51.8 50.9 51.4 55.3 48.3 49.5 53.2 52.8 53.5 47.4 45.2 42.5 49.4 46.3 44.8 48.4 45.7 53.1 41.3 52.4 43.2 0.6 -8.7

NSb
NS NS NS NS NS NS

1 2 3 4

0.78288 0.70154 0.67295 0.65803

0.0003 0.0001 0.0001 0.0002

Note: FSIQ > 85.

0.0003
NS

0.04 0.04
NS 0.02 NS 0.04 NS 0.002 0.04 NS NS NS NS NS 0.09 NS 0.002 0.01

Asperger's Syndrome (n

14) and Autistic subjects

8)

NS

= nonsignificant.

85 (14 AS and 8 HFA). These were compared to the 36 OPC with similar FSIQ. Table 5 shows these results (to facilitate comparison with later tables, all tests are now reported as T-scores with a mean of 50 and a standard deviation of 10). The first point to note is that there were no significant differences between these groups on either verbal, performance, or FSIQ. The WISC-R subtests that significantly differentiated the groups were comprehension, picture completion, picture arrangement, and object assembly. Thus, deficits in both higher-order verbal and performance skills were apparent. On the neuropsychological tests, significant differences were found on the BTFR, Pegboard , and the discrepancy between academic achievement and IQ . It appears that PDD children , with IQ above 85, show outstanding deficits in motor coordination , higher-order nonverbal problem solving, and language comprehension, but have excellent reading recognition skills. Tests of acquired knowledge (i.e., Information, Arithmetic, Vocabulary), immediate attention and memory (Digit Span), and concrete match-to-sample tasks (Block Design , Coding, DTVMI) , were performed at levels indistinguishable from OPC . When the significant variables (i.e . , Comprehension, Picture Completion, Picture Arrangement, Object Assembly , BTFR, Pegs) were entered into a discriminant function analysis (Tables 6 and 7), four measures significantly differ134

entiated the groups: (1) Pegboard performance, both dominant and (2) nondominant; (3) comprehension from the WISC-R; and (4) the BTFR (see Table 6). The classification matrix correctly classified 82% of the children (73% of the PDD group and 88% of the out-patient controls) and the Wilks Lambda was highly significant (p = 0.002) (Table 7). This suggests that the pattern of neuropsychological deficits is quite specific to PDD children with FSIQ above 85, but that some PDD children (6 of 22) are free of this pattern . The issue of subtypes of cognitive profiles among PDD children was investigated by noting the extent to which the pattern of deficits differed according to the IQ of the subject. The PDD sample was , therefore , divided into two groups ; low IQ (i.e. , IQ between 70 and 85), and high IQ (i.e., IQ above 86). Each child's T-score (on each test) was subtracted from his/her own FSIQ T-score . This discrepancy score provided an estimate of the child's cognitive profile, relative to his/her own IQ. The discrepancy scores of the high and low IQ PDD groups were then compared using a simple ttest. Table 8 presents the results from this analysis. Individual verbal subtests were slightly better than FSIQ for both low-IQ and high-IQ groups (i.e., the scores were negative), but discrepancy scores for these two groups were not different from each other. On the performance subtest scores, however, the pattern was quite different. For the high IQ PDD group these subtests (except for block design) were relative deficits, whereas they were relative strengths for the low IQ group. The discrepancy scores were significantly different for object assembly, picture arrangement, and picture completion. Similarly, on the BTFR and the DTVMI, the high-IQ subtests performed quite badly, relative to IQ. In contrast , for the low-IQ group, there was little discrepancy from their own IQ . There was also a trend (p = 0.06) for the high-IQ group to have better verbal than performance IQ scores than the low-IQ group. It appears, then , that among high-IQ PDD subjects, relative cognitive deficits exist in both verbal and nonverbal tasks that measure higher order abstract processes. As IQ decreases , relative deficits in verbal skills become apparent and tests of visual-perceptual ability are a strength . Conclusion The main objectives of this study were to see: (1) whether AS and HFA had different neurocognitive profiles; and (2) whether the essential deficit among PDD children is found in language or abstract problem solving. The univariate analyses demonstrated that the AS and HFA groups were
,,/ l.Am.Acad. ChildAdolesc. Psychiatry, 29:1, lan.1990

ASPERGER'S SYNDROME AND AUTISM

TABLE

7. Discriminant Function Analysis (PDD vs OPC): Classification Matrix Cases Classified into Group

TABLE

8. Pattern Difference by PDD IQ Subtype PDD

Actual Group PDD OPC Totals

% Correct

AS

OPC

Variable Discrepancy Scores (FSIQ-subtests) Information Similarities Vocabulary Comprehension Digit Span Arithmetic Coding Object Assembly Block Design Picture Arrangement Picture Completion BTFR DTVMI Token Test Pegs-dominant Pegs-nondominant CWFT WCST-categories WCST-% Perseverance errors WCST-No. of errors VIQ-PIQ
a

IQ <85 (N = 21)

IQ =85 (N = 22)

p Value

73 88 82

16 4 20

6 30 36

Note: FSIQ > 85.

impaired on all tests, relative to the OPC, but were not really different from each other. The differences with the OPC were quite robust and indicate that some measurable neurocognitive impairment is almost always found in PDD children. Few statistically significant differences were found between the HFA and AS groups. These differences were relatively small and probably reflect severity rather than a separate etiology. Why the AS group should do so poorly with their nondominant hand is unclear. This could represent a measurement problem or greater evidence of mixed dominance among younger subjects. Other tests commonly thought to be associated with right-hemisphere function did not differentiate these two groups, so the notion that AS is a disorder of the nondominant hemisphere is not supported by these data. On the basis of these results, the authors feel it is justified to combine the AS and HFA groups into a more general PDD category. As a way of investigating the core cognitive deficit of PDD children, the AS and HFA groups were combined and compared to the OPC. The authors expected to find deficits in language and no differences on the performance subtests and other measures of visual-perceptual function; this did not tum out to be the case. Instead, measures of language comprehension, motor coordination, and nonverbal problem solving (i.e., Picture Completion, Picture Arrangement, Object Assembly, and Facial Recognition) significantly differed between the groups, and the resulting classification matrix indicated that this pattern is both sensitive and specific to PDD children but not unique. Previous studies reporting deficits in language and strengths in visual-perceptual function were done on lowerfunctioning, or younger, PDD children. The authors wondered about the extent to which their findings could be explained by the developmental level of the child. Many of the tests from the present battery assess differed cognitive abilities at different developmental levels. Performance subtests from the Wechsler Scales, for instance, require more independent problem solving and less simple perceptual matching at older age levels and/or higher IQ. Indeed, the results on pattern differences by IQ subtype (Table 8) indicated that many of these performance subtests were deficits only for the PDD children with FSIQ above 85 (i.e., at higher developmental levels). Thus, the pattern of neurocognitive deficit appears to vary by IQ. Among low-IQ PDD children, the typical pattern of low verbal/high performance scores is evident. Among high-IQ PDD probands, the pattern is more complex and suggests selective impairments in' higher-order problem solving, irrespective of
l.Am.Acad. ChildAdolesc.Psychiatry, 29:1 ,Jan. 1990

-4.5 -5.8 -5.1 -0.2 -5.2 -1.8 -1.2 -7.3 -6.7 -7.6 -6.1 -3.4 3.0 -0.2 11.0 9.6 8.5 1.2 13.9 4.6 -1.0

0.9 -0.9 -1.1 3.8 -4.8 -2.7 2.5 1.3 -3.0 2.8 4.8 11.9 10.0 -1.9 19.6 17.3 9.2 5.8 9.8 6.7 4.0

NS NS NS NS NS NS NS

0.002
NS

0.001 0.000 0.000 0.03

NS NS NS NS NS NS NS

0.06

NS, nonsignificant.

whether these are in language or visual-perceptual areas. In essence, the authors agree with Rumsey and Hamburger (1988) that the essential deficit among PDD children is abstract flexibility, implicating frontal-subcortical systems in the brain. One difference from Rumsey and Hamburger's (1988) findings is that outstanding deficits in motor coordination were observed in this study. Indeed, many of the PDD subjects were scoring 3 or 4 SD below the mean on the Pegboard tests. Rumsey and Hamburger (1988) did not observe similar motor deficits, although they found the same trend (i.e., p < 0.10). It is worth noting that their sample was, on average, older and brighter than the subjects in the present study. This conjunction of deficits on motor coordination and abstract thinking is similar to the neuropsychological deficits seen in Parkinson's disease and other forms of subcortical dementia (Cummings, 1986). The authors think that a useful paradigm for future studies might be to consider autism a developmental form of subcortical dementia. Indeed, the similarities with Parkinson's disease has been commented on previously (Vilensky et al., 1981), although not systematically pursued. The results of this study must be considered cautiously for a number of reasons. First of all, there are no wellestablished diagnostic criteria for AS that ensure complete separation of these children from autism. One reason, therefore, that more differences were not found between the AS and HFA may have had to do with this lack of clinical separation. The PDD probands were rediagnosed into the

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DSM-llI-R criteria of autistic disorder and PDD-not-otherwise-specified, but the results were substantially the same. Second, there was considerable disparity between the groups in age and IQ. This was necessary because the intent was to collect as large a sample size as possible, but it has meant that not all tests were equally sensitive across all age groups, nor do they necessarily assess similar cognitive abilities. In future studies, it would be worthwhile to test more specific hypotheses which better matched samples of more clearly defined PDD probands. The authors also think it would be important to see how the cognitive deficits of PDD children differ from those seen in learning disabled groups. This would control for the presence of cognitive impairments. One of the other implications of the present results is the usefulness of including AS subjects when studying the pervasive developmental disorders. AS children are not all that uncommon and are of normal IQ. Thus, including them avoids two of the major difficulties in studying autism (i.e., small numbers and the complicating effect of mental retardation). Many investigators have commented on the importance of using PDD individuals of normal IQ for biological and cognitive studies. Since nonretarded autistic children are quite rare, this can be difficult. The present results support the inclusion of AS as a very mild form of PDD where the cognitive profiles are virtually identical to autism, suggesting similar deficits in brain organization.

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