Nuuropsvchobguz, Vol. 25. No 6. pp. 881-891. 1987 Prmted in Great Bream

0028.3932187 S3.00+0.00 (” 1987 Pergamon Journals Ltd.

DEVELOPMENTAL GERSTMANN SYNDROME WITHOUT APHASIA IN FRAGILE X SYNDROME

JAMES P. GRIGSBY*, MELINDA B. KEMPER? and RANDI J. HAGERMANS *Psychology Service, Veterans Administration Medical Center, University of Colorado at Denver, Denver,

Colorado, U.S.A.; Khild Development Unit, Children’s Hospital, Denver, Colorado, U.S.A. and $Department of Pediatrics. University of Colorado Health Sciences Center, Child Development Unit, Children’s Hospital,

Denver, Colorado, U.S.A.

(Rechrd I1 April 1986; accepted 19 March 1987)

Abstract-Considerable controversy surrounds Gerstmann’s syndrome, with some authors ques- tioning its localizing significance. and others doubting its existence as a distinct entity. This literature is reviewed. and a case is presented of a young boy with Fragile X syndrome who showed all five symptoms of the developmental Gerstmann syndrome. He was one of a group of individuals found to be cytogenetically positive for Fragile X syndrome, who were observed to possess three or more of Gerstmann’s symptom in the complete absence of aphasic disorder.

INTRODUCTION

IN A SERIES of papers published over a period of 30 yr, Gerstmann outlined, then defended the existence of the syndrome which carries his name [bll]. Gerstmann’s syndrome is characterized by dysgraphia, dyscalculia, finger agnosia and right-left disorientation. The so-called developmental Gerstmann syndrome 120,211 also includes constructional dyspraxia. In recent years, this group of symptoms has increasingly come under attack as a questionable neuropsychological entity, and it is not clear that the matter has yet been satisfactorily settled.

The syndrome was first questioned by BENTON [2], who saw it as “an artifact of defective and biased observation”. In a study of 100 people with either focal or diffuse neurological lesions, he found no cases of a “pure” syndrome, and observed that its four components were no more highly correlated with one another than they were with three ostensibly unrelated symptoms. Benton concluded that the syndrome is a “fiction”, that careful observation demonstrates a large number of permutations of parietal lobe cognitive defects, and that the value of the syndrome as a localizing diagnostic indicator is overstated.

There were several problems with Benton’s paper. First, as GESCHWIND and STRUB note 1121, he did not choose the appropriate statistical tool for studying a syndrome. The correlation among elements of a syndrome is irrelevant, as is the correlation of those components with other deficits. The crucial point is the predictive validity of the syndrome.

Second, since the syndrome is hypothesized to occur as a function of left parietal lobe damage, it should be studied in patients with left parietal lesions, only 12 of whom were included in Benton’s analysis. In a large group of patients with mixed diffuse and focal pathology, intercorrelations may thus be lost. While Benton found similar results among his 12 patients with left parietal lesions, the nature, size and location of the lesions of these persons were unclear. Assuming that Gerstmann was correct in arguing that the angular

881

882 JAMES P. GRIGSBY, MELINDA B. KEM~ER and RANDI J. HAGERMAN

gyrus is the site of lesions which cause this syndrome, it may be that only a rather precisely circumscribed lesion could produce a “pure” Gerstmann’s syndrome. A small infarct along the posterior parietal artery might have such an effect, but the proximity of the angular gyrus to Wernicke’s area, for example, makes it likely that aphasic symptoms will also be seen.

Third, the scores for each function obtained by Benton’s patients do not measure unitary capacities. Five different tasks were used, for example, in assessing finger agnosia. The summary score which was computed may thus reflect disorders other than impaired finger gnosis. This same problem holds true for Benton’s test of right-left orientation.

KINSBOURNE and WARRINC;TON 1191 reported on nine nonaphasic patients with a complete Gerstmann’s syndrome, though their tests of finger agnosia were questionable [26, 271. Constructional dyspraxia was simultaneously present in several of their patients.

In 1964, HEIMBURC;ER et al. [16] examined the neuropsychological test scores of 456 consecutively tested individuals who were suspected of having some sort of cerebral dysfunction. Only 23 had Gerstmann’s symptoms, and these persons tended to have large, progressive, very destructive lesions. Ninety-two per cent were hemiparetic (83% on the right side), and all 23 were dysphasic. In patients with 3-4 of Gerstmann’s symptoms there were almost always additional deficits, which were generally gravely disabling.

They also found that as the number of Gerstmann symptoms increases, the likelihood of a left hemisphere lesion increases accordingly, though the occurrence ofall 4 did not guarantee a left sided focus. While there was a strong tendency for the left angular gyrus to be the site of a lesion. in several casts it was not, and as a rule, the lesions were “huge”, affecting surrounding areas as well. Of the 345 patients without Gerstmann symptoms, two were right-handed with lesions of the left angular gyrus. Heimburger c’t al. concluded that the syndrome “is only one of a number of concurrent deficits rather than an autonomous entity”. As in other studies. there were problems with the tests used by these authors.

CRITCHLEY [4] reviewed the literature on Gerstmann’s syndrome. and stated that he was not convinced of its validity, nor was he convinced of the existence of a developmental Gerstmann syndrome.

In the same year that Critchley published his review, POECK and OR<;ASS 1251 did a comprehensive statistical study of the problem. Wishing to avoid some of the methodological flaws of Benton’s work, they used simple screening tests, without continuous scores, which classified an individual’s ability as either “defective” or “normal”. Besides looking for Gerstmann’s deficits, they tested reading ability, constructional praxis, verbal memory and language ability. Aphasia was chosen because HEIMBUR(;ER rt d. [ 161 and BENTON [I] had noted a high incidence ofdysphasic disturbances amont their Gerstmann syndrome patients.

Only 4 of 50 patients, unselected for type, location or size of lesion, showed the complete syndrome. Each ofthese had at least three other concurrent neuropsychological deficits. Like Benton, they found that the correlations between these four symptoms and each other were no greater than the correlations between the other deficits investigated and the Gerstmann symptoms. Poeck and Orgass concluded that the syndrome is therefore not independent of other disorders.

All four individual symptoms were significantly associated with aphasia. In fact, whenever 3-4 of the classic symptoms occurred, it was always only in left hemisphere lesions which involved an aphasic disorder. They concluded that “the Gerstmann syndrome does not convey any information of practical importance about the localization” of a lesion, since aphasia was always present.

Reviewing Gerstmann’s early cases, Poeck and Orgass noted that as early as 1927 he could

FRAGILE X SYNDROME 883

have considered constructional dyspraxia to be a fifth component of the syndrome, because of its regular occurrence. Arguing that all of these symptoms are related to language, or are assessed by verbal tests, these authors stated that aphasia is the basis for the Gerstmann syndrome, and that these symptoms “are an arbitrary partial grouping of the numerous

neuropsychological disturbances resulting from lesions of the leading hemisphere” (p. 436). Although it makes some important points, this paper is unfortunately subject to some of

the same criticisms that hold for Benton’s work. These include the sample size and selection, and a lack of precise information on the location, type and extent of left parietal lobe lesions. In addition, it is not necessarily the case that all of these functions must be related to language

or assessed by verbal tests. Finger agnosia, for example, may be tested nonverbally (e.g. pointing to the finger that is touched). If constructional dyspraxia is considered part of the syndrome [21], it clearly may be tested nonverbally.

STRUB and GESCHWIND [33] responded with a case of Gerstmann’s syndrome without aphasia. Their patient was a 52-yr-old nonaphasic woman who had developed the syndrome over a period of 6 months. Laboratory testing revealed cortical atrophy, and she was diagnosed as demented. In their discussion, Strub and Geschwind agreed that there may be a

high correlation between Gerstmann’s syndrome and aphasia, but argued correctly that this does not demonstrate a causal relationship. In addition, they noted that “a large parietal lesion could interfere with several of these functions, but it does not follow that they all have a similar psychological basis”.

Strub and Geschwind’s paper brought an acrimonious response from POECK and ORGASS [28], who thought they had driven a stake through the heart of the syndrome. They took exception to the case presented by Strub and Geschwind, arguing that focal syndromes should not be studied in persons with diffuse neurological disorders (as this patient had). Furthermore, in this case finger gnosis was not impaired in the usual sense, and the patient

showed no right-left disorientation for herself or the examiner. Her right-left failures came only on more complex tasks, which may be affected by general mental deterioration. Poeck and Orgass agreed that the remaining problems exhibited by this woman were not aphasic in nature, but could be explained as resulting from constructional dyspraxia and “perceptual spatial deficit”. They suggested that her difficulties were secondary to right parietal lobe

dysfunction. In their reply to Poeck and Orgass, GESCHWIND and STRUB [12] contended that the

syndrome remains a good localizing sign. They respond to the suggestion that there is no evidence of left parietal pathology by pointing to POECK and ORGASS’S own work [25], which found no patients with all four components who had lesions of the right hemisphere alone. They fail, however, to address the fact that their patient’s right-left disorientation and finger

agnosia were questionable. In 1977, BENTON again entered the debate [3], reviewing the research since his earlier

paper. He concluded that the elements ofthe syndrome are not specific, independent entities. He acknowledged the vulnerability of his own earlier study [2] to the criticisms leveled by Strub and Geschwind regarding the nature of a syndrome and the irrelevance ofcorrelational analyses, but did not consider the conceptual and methodological problems of other studies supporting his perspective.

Benton found the evidence in support of the Gerstmann syndrome to be anemic, and

described it as “an arbitrary grouping of deficits without a distinctive neuropsychological significance”. Furthermore, he argued that aphasia and general mental impairment are

necessary but not sufficient conditions for the occurrence of finger agnosia and right left confusion.

ROELTGEN et al. 1291 presented the case of a 62-yr-old man who had suflered an ischemic infarct in the dominant superior angular gyrus, posterior supramarginal gyrus and superior parietal lobule. This patient showed all four symptoms of Gerstmann’s syndrome. with no evidence of aphasia, ideomotor apraxia. alexia, memory disorder or constructional dyspraxia. He could distinguish left from right on himself for simple commands, but made errors with two step commands. The authors concluded that Gerstmann’s syndrome is uncommon because the lesions usually extend into surrounding areas of the parietal lobe.

SOBOTA pt a/. 1321 published a study of a 52-yr-old right-handed male who had suffered an ischemic lesion of the left postcentral gyrus, with a consequent Gerstmann syndrome. Their patient was studied over a period of several days. He demonstrated a significant overall drop in intellectual functioning, severely impaired immediate recall and short term memory. bilateral motor impairment (especially the right hand). and exosomesthesia in response to bilateral simultaneous tactile stimulation. SHAPIRO ct N/. [3 I] discussed this symptom as a rare phenomenon. a pathological extension of the body image “secondary to bilateral cerebral disease”, which “except in children under special conditions-has been observed exclusively in patients with severe mental changes resulting from disease of the brain” (p. 487). It is frequently elicited by bilateral simultaneous stimulation. The patient showed all four of Gerstmann’s signs when first evaluated, though they decreased in severity with time. Sobota and his associates note a lack of agreement between CT scan, blood flow studies, autopsy findings and neuropsychological assessment, and invoke the concept of diaschisis 1361 to explain the generalized cognitive disturbance resulting from a rather small lesion of the postcentral gyrus, and the change in the symptom picture over time.

These authors concluded that Gerstmann’s syndrome is a”myth”, and that while it may be a useful lateralizing sign, its value as an indicator of angular gyrus lesions is suspect. While the case they present is interesting, it is perhaps susceptible to the same criticism POECK and ORCASS [28] made ofthe paper by STRUB and GESC‘HWIND [33]. that focal symptoms are best studied in persons without generalized disorders. It is also conceivable that Sobota’s patient had suffered more chronic. diffused but perhaps subtle impairment of function secondary to his numerous medical complaints.

The developmentul Grrstmann syndrome

Relevant to the findings presented in this paper is the work of KINSBOURM and WARRINGTON [20], who described a developmental version of the Gerstmann syndrome. They presented a series of seven children with symptoms of Gerstmann’s syndrome, including constructional dyspraxia. The etiologies were in some cases indeterminate. but most were considered to have suffered some form of trauma at birth. In all cases, Wechsler verbal IQ scores exceeded performance IQ scores by a considerable margin (a range of 2O- 35 points). Low scores tended to occur on Block Design, Picture Arrangement and especially Object Assembly subtests, with means of 6.0,5.8 and 2.0, respectively. Arithmetic scores were also relatively low (mean= 7.6). There was little evidence of heritability. Language disturbances were not noted in their subjects, though the first case showed “rapid and staccato” but otherwise normal speech, while the speech of the second was described as “somewhat explosive”. These were the only cases of non-traumatic etiology. KINSBOURNE [Zl] contended that cerebral damage is not necessarily the cause, but that the syndrome may

FKAGILE XSYNDKOME 885

represent a “developmental lag”, for which one may in time compensate, with possible continuing educational retardation.

The main thrust of disagreement with the existence of Gerstmann’s syndrome is thus that it is a function of aphasic disturbance, or possibly of general cognitive deterioration. Poeck and Orgass take this even further, and argue that two of the components of Gerstmann’s syndrome are themselves questionable: right-left disorientation and finger agnosia [26,27]. They assert that both symptoms are secondary either to aphasia or general impairment of intellect. While certain of the cases in the literature are indeed questionable, those of KINSBOURNE and WARRINGTON [20] may be valid, though their testing for finger agnosia is suspect. The following case material provides support for the existence of a developmental Gerstmann syndrome, of genetic transmission, without aphasia or general intellectual

impairment.

Fragile X syndrome

Fragile X syndrome [S, 14,17,24,30,34] is an X-linked disorder which frequently presents as mental retardation or as a pattern of learning disabilities, often with associated physical or behavioral symptoms. Among males these may include large or prominent ears, high or prominent forehead, elongated face, macroorchidism, hyperextensible finger joints, hyperactivity and autism [ 141, while no unusual physical signs have been found among retarded heterozygous females. In males, mild to moderate retardation is a likely outcome. In females there is a greater probability of normal or near normal intellect, with approximately two-thirds appearing normal [35], though there are often specific learning disabilities [lS]. Estimates of incidence have varied, though HAGERMAN et al. [ 141 suggest that Fragile X may be “second only to Down’s syndrome as an identifiable chromosomal cause of mental retardation”. The case presented here is the only learning disabled male subject whom we have so far assessed. Our remaining cases have been females. His case is thus significant in that the same pattern ofcognitive shortcomings may be found in both sexes, though it is seen more commonly in females. This itself is unusual, given that learning disabilities are often thought to occur more frequently in males. Because Fragile X is an X-linked disorder, however, and males have but one X chromosome, they may be more sensitive to the deleterious effects of the fragile site and thus more prone to be retarded than to have normal intelligence with learning difficulties.

CASE PRESENTATION The subject of the current report is M, a right-handed boy of St, in third grade, who has been diagnosed as having

Fragile X syndrome. He has been discussed elsewhere [ 131. M was the first of a series of nine consecutive individuals who had been found to be cytogenetically positive for this syndrome who were then evaluated neuropsychologically following diagnosis and administration of intelligence and achievement tests. All were in the immediate families of retarded individuals who had been found to have the Fragile X syndrome. All were right-handed, and none had any history of vascular, traumatic, neoplastic or other cerebral insults. Data are also presented on these other subjects (see Tables 1, 2 and 3).

METHODS All subjects were first administered the Wechsler Intelligence Scale for Children-Revised (WISC-R) or the

Wechsler Adult Intelligence Scale-Revised (WARS-R), and the Wide Range Achievement Test-Revised (WRAT-R). The pattern ofscores, which were especially low on spelling, arithmetic and block design, suggested the possibility of a focal neuropsychological deficit involving dysgraphia, dyscalculia and constructional dyspraxia. Further tests

XX6 JAMES P. GKIGSBY, MLLINI)A B. KI+II’LK and RANIX J. HAC;~.KMAN

were then devised to assess the presence of parietal, temporal and occipital lobe localizing signs. Descriptions of these tests. and of M’s performance. folIoA.

M obtalncd arithmetic score5 on the WISC-R and WRAT-R at the 2nd and 12th percentiles. respectively. Signs of spatial dyscalculla w’erc present. He found it difficult to read numbers with more than three digits, and had special difficulty with those containing zeros. For example, he read 1.041 as “one hundred and four, one”. He read 2.067 as “two. three hundred and Lxty-scvcn”. He had great difficulty in writing numbers from dictation. While he performed adequately with two-digit numbers. he incorrectly wrote 209 as 29, 124 as 1024, and 61 1 as 601 I, Asked which of two numbers was larger. he picked IX9 over 201. and 1967 over 3002, choosing the larger individual numerals in the smaller numbers. These dilticult~cs rcflcct what 1.1 KIA 1231 called “dismtcgration of the categorical structure of number” (p. 1X3).

Calculation skills were very poor, and M wa\ often unable to decide which operation wah intended by the various arithmetic signs. While he attempted to calculate using his fingers. he wab nevertheless wide of the mark on most simple problem\ (e.g.. 21 ~~ 14 = 12). misGnp II of I2 simple calculation problems. Carrying and borrowing wcrc particularly dlfticult.

M was asked to identify right and left on both hlmsclf and on the examiner. In response to both simple and complex commands. Among our subjects. the occurrence of three or more errors on oneselfwas taken as evidence of right left disorientation M’x performance w’as near the chance level for all types of instructions, though he was slightly better (but quite slow) in responding to simple commands on himself (e.g.. “show me your right hand”. six out of eight correct). Given two-part commands on his own body. he missed three of five.

M made dysgraphic errors which wcrc chnracterired by minor problems with spelling (his WRAT-R spelling score was relatively good. at the 42nd percentile ). but cspeciallg by problems in the formation ofletters and numbers (Fig. 1 I. Thex occurred both in copqing and in writing from dictation. He was unable to write m cursive. He could correctly identify lettt‘rh. and had no difficulty in determlning when a letter or number was presented as a mirror image of itself

M was tested both verbally and nonverbally, while blindfolded, for tinger agnosia. Thlj testing was simplified in light of the comments of POF-cx and OKGASS 1261, who consldered Kinshourne and Warrington’s procedures to be “inappropriate to assess finger agnosia” (p. 806). First. M was asked to point to the finger which had been touched, for a total ofthree trials with each linger. Hc was then asked to name the finger that had been touched. again for three trials on each finger. Fingers were named by number (thumb and I 4. with practice naming the lingers before using

the blindfold). His performance was equally impaired for both nonverbal and verbal identilication, with both hands. Though he uas accurate at identifying the thumb when touched. he was only 33 “4 accurate in identifying his other four lingers

Scccrc constructional dilticultics wcrc observed both in the reproduction ofgeomctric figures. and in his drawings ofa cloth and a hand. On the block design subtest of the WISC-R hedld somewhat poorlv (25th percentile). and his obJect a\semhlq score uas at the 5th pcrccntile. Samples of his work are presented in F’ig. 2.

M demonstrated no spontaneous indications of aphasic disorder. Hia speech was distinct and fluent. with no indications of word finding problems or paraphasic errors. He named objects and pictures (e.g. pen, watch, fork. hottlc. hodq par&) flawlessly. had no dilticulty in following directions (c.g., place the watch on the book and hand mc the pencil). and demonstrated an understanding of prepositions denoting location (e.g.. above, under) and complex logical and grammatical constructions. An earlier speech and language ecaluation by a rpeech pathologist had found no Indications ofaphasia. On the vocabulary suhtcst ofthe WISC-R. he obtained a scaled score of IO. at the 50th pcrccntlle. On the Peabody Picture Vocabulary Tc\t (PPVT), he obtained a score at the 93rd percentile. Absence of dqsphauia In this subject and in the Icarnlng disahled hetcroqgous females contrasted with findings reported for retarded Fragile X males [ 22 1.

M rcud Ictterh accurately, and scnrcd a~ the 90th pcrccntilc on the WRAT-R reading test. In the developmental (;er<tmann \yndromc rending ahilitk i\ also generally preserved. especially relative to arithmetic skill. He

FRAGILE X SYNDROME

Table 1. WISC-R and WRAT-R scores obtained by subject M

887

Scaled scorts and IQ scores on WISC-R

Information 11 Picture completion 10 Similarities 10 Picture arrangement 1 Arithmetic 4 Block design 8 Vocabulary 10 Object assembly 5 Comprehension 7 Coding 12 Verbal IQ 90 Performance IQ 81 Full-scale IQ - 84

Percentile ranks on WRAT-R Reading 90 percentile Spelling 42 percentile Arithmetic 12 percentile

Math problems written from dictation

MY (copied)

‘Ihe man was sick so he went to bed. (copied)

FIG. I. Subject M’s dysgraphic productions, numbers written from dictation and words copied from typed text.

88X JAMES P. GRIGSRY. MELINDA B. KEMPER and RANDI J. HAGERMAN

stirdus Subject ’ s Figure QPY

c ml

Subject’s drawing of ahand

FIG. 2. Samples of constructional dyspraxia. The drawing at bottom is the subject’s response to the request to draw a hand.

demonstrated no visual agnosia, and had little difficulty in seeing embedded figures, finding 8 of 10 and missing the largest in each case. On the Picture Completion subtest of the WISC-R. he scored at the 50th percentile.

According to Reed’s norms for the Reitan-Indiana neuropsychological battery for young children. M’s tinger tapping speed was at about the 90th percentile for his age bilaterally. Manual coordination was within normal limits. He demonstrated no ideomotor apraxia (i.e., simple gestures such as waving goodbye, using a key). though he was unable to correctly demonstrate how to pour and stir cocoa (ideational apraxia). On the Coding subtest of the WISC-R, he scored at the 75th percentile.

Memory and learning ability. apart from immediate recall for digits, were quite good. On a test which required him to learn a list of 10 unrelated words presented several times, he remembered 9 by the third presentation. and all 10 on the fifth trial. a performance which is in the everage range for adults. After a delay of 30 min. he remembered all IO words. On the associate learning subtest of the Wechsler Memory Scale. his score of 16.5 w’as above the mean for adults between the ages of 20 and 29 (mean = 15.72). On a second paired associates task, in which he was asked to remember the words that went with a series of seven pictures, he demonstrated perfect recall.

The subject described above was one of nine consecutively evjaluated individuals who had previously been found

FRAGILE X SYNDROME 889

cytogenetically positive for Fragile X syndrome. Most of them showed strikingly similar cognitive defects. In no case was there any evidence of aphasia. Data on these subjects is presented in Table 2. Of the nine subjects, none showed the “pure” Gerstmann syndrome. Four, including the subject presented above, demonstrated constructional dyspraxia in addition to the syndrome (two of the four were sisters). One showed none of the symptoms, while the remainder had either three or four Gerstmann symptoms. KEMPER et al. [18] have demonstrated that heterozygous Fragile X females have a relatively typical pattern of scores on the Wechsler Intelligence Scale subtests, with depressed scores on Block Design, Arithmetic and Digit Span. While the overall profile differs somewhat from that observed by KINSBOURNE and WARRINGTON 1201, both studies found low scores on Arithmetic and Block Design subtests. Presumably these scores are a function of dyscalculia and constructional dyspraxia.

DISCUSSION

The current study found that eight of nine individuals evaluated showed three or more symptoms of developmental Gerstmann syndrome. This provides confirmation for the contention [25] that Gerstmann could have added this symptom to the syndrome, and supports the findings of KINSBOURNE and WARRINGTON [20]. The present data seem to make it clear that Gerstmann’s syndrome is in fact a clinical entity, though it may be rare. Among individuals with Fragile X syndrome, however, it appears to be relatively common, though an “incomplete” syndrome is often observed. Among the subjects reported on here, there were five different combinations of Gerstmann signs.

There was no evidence of aphasic disorder in any of these subjects. Though the patient of STRUB and GESCHWIND [33] may have been somewhat questionable because of her concurrent dementia, the cases presented by KINSBOURNE and WARRINGTON [20], ROELTC;EN

et al. [29] and SOBOTA et al. [32], along with the data from the present study, appear to be conclusive in demonstrating that aphasia is not the cause of these symptoms. Furthermore, other focal neuropsychological deficits were not seen in any of the present group of patients. Of further interest is the fact that dyscalculia was observed in all eight of our symptomatic subjects. This is in contrast with the observation of HECAEN and ALBERT [ 151 that dyscalculia is the least common symptom of the syndrome. Unlike the findings of POECK and ORGASS [27], subjects who did poorly on right-left discrimination had no no difficulty distinguishing above from below.

The relative homogeneity of cognitive pathology in these individuals with Fragile X suggests the presence of a regularly occurring, discrete developmental lesion. Although a congenital anomaly may not have the same structural and functional appearance as an acquired deficit, the literature on acquired Gerstmann’s syndrome suggests at least the possibility that Fragile X syndrome is often characterized in part by an anomaly of the dominant parietal lobe. While the location of any presumed lesion in the present subjects is unknown, the use of appropriate procedures (e.g. PET scan) may shed more light on the matter.

This pattern of deficits in people with Fragile X is essentially the same as the developmental Gerstmann syndrome described by KINSBOURNE and WARRINGTON [20], and it may be that those authors had in fact encountered some cases of persons with Fragile X. Besides the similarities noted above, it is interested that Kinsbourne and Warrington’s first two subjects showed unusual speech, one described as “rapid and staccato”, and the other as “somewhat explosive”. The speech pattern in Fragile X has been described in similar terms [14, 22, 351. The presence of these focal neuropsychological deficits in the absence of a history of neurological insult may suggest the possiblity of Fragile X syndrome. The occurrence of a stable configuration of such parietal lobe dysfunction in persons with Fragile X may also provide an opportunity for detailed exploration of the underlying disorder.

890 JAMES P. GRIGSBY. MELINDA B. KEMPER and RANDI J. HAGERMAN

Table 2. Test data for nine cytogenetically positive Fragile X subjects

Subject Finger Const

Sex Age VIQ PIQ FSIQ Dyscalculia R-L agnosia Dysgraphia dyspr.

M M 9 90 81 84 2 F 18 64 67 65 3 F 19 71 68 69 4 F 12 89 103 95 5 F 20 78 75 75 6 F 8 88 87 87 7 F 28 71 75 72 x F 41 71 x2 15 9 F 33 123 122 127

+ + + + + + + + + + + + + + + + + + + + + + + + + + + + + + + _ + + + _

_

+ Symptom present. -symptom absent. IQ scores are from either the Wechsler Adult Intelligence Scale-Revised (WAIS-R) or the Wechsler Intelligence

Scale for Children-Revised (WISC-R).

Table 3. Percentages of correct responses for Fragile X subjects on tests of finger agnosia and dyscalculia

Subject Dyscalculia Finger agnosia % correct Nonverbal % Verbal %

M 54 37 43 2 SO 63 47 3 40 57 33 4 x4 100 47 5 X9 73 20 6 72 I 00 100 I 21 70 63 8 65 30 57 9 100 100 I ci)

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