NOTE

GERSTMANN SYNDROME: A CASE REPORT

M. Mazzoni\ L. Pardossi1, R. Cantini2, V. Giorgetti2 and R. Arena1

(1Neuropsychology Laboratory, Institute of Clinical Neurology and 2Institute of Neurosurgery, University of Pisa, Italy)

In the Twenties (1924, 1927 and 1930) Gerstmann defined a syndrome characterized by the association of finger agnosia, dysgraphia, dyscalculia and right-left disorientation and pointed out that it had a localizing value, indicating a pathology of the dominant parietal lobe, specifically the left angular gyrus.

In 1961, however, Benton found that the four elements of the syndrome are no more clo­sely intercorrelated than are the many other signs of parietal lesion, casting serious doubts on the autonomy of this distinctive constellation which he defined as "a fiction". Other au­thors (Heimburger, Demyer and Reitan, 1964; Poeck and Orgass, 1966, 1975) also denied the diagnostic value and theoretical significance of the Gerstmann syndrome.

This paper describes a case of Gerstmann syndrome following a trauma strictly confined to the left angular gyrus.

CASE REPORT

P .L. is a 44 year-old right-handed male with 5 years of school education, owner of a road­haulage firm. His medical history included no significant pathologies.

On February 17, 1986 he was hit by a metal bar which fell from a shelf in his workshop: the bar penetrated the cranium in the left parietal region. He did not lose consciousness, re­moved the bar and went to the First Aid of the nearest hospital. After medication and suture of the wound, he was hospitalized for observation. During the afternoon, the patient began to complain of severe headache in the area of lesion, paresthesia on the right hemisoma and difficulty in finding the right words to express his thoughts. I_n spite of antiedema treatment, the paresthesia worsened and became a true hypoesthesia. The patient was urgently trans­ferred to the Institute of Neurosurgery of the University of Pisa; the neurological examina­tion performed on admission revealed lateral misplacement of the right arm and a slight an­omic aphasia. There were no visual field deficits. A cranial CT scan revealed a comminuted fracture in the left posterior parietal zone, with fragments penetrating the underlying cere­bral parenchyma which showed a laceration.

The following day, the patient underwent surgery toilette of the galea and lesion. The bone lacuna had a regular, rounded shape, with a diameter of 2.5 cm. The post-operatory course was satisfactory: penicillin, phenobarbital and antiedema were administrated. Fever dropped after 5 days, and the patient was dismissed on February 27, 1986. The neurological examination was normal. -

On returning to work, he realized that he could no longer read, write or do calculations correctly. His reading ability returned after only a few days, but difficulties in writing and calculating persisted, preventing him from taking care of the administration of his firm per­sonally.

Cortex, (1990) 26, 459-467

460

Description of an event Boston Naming Test Auditory Comprehension

Semantic Oral commands Token Test

Verbal fluency Repetition

Words Non-words Sentences

Writing Written naming Dictation

words sentences

Copying Reading aloud

Words Non-words Sentences

M. Mazzoni and Others

TABLE I

P.L. 's Performance on Language Tests

Comprehension of written stimuli Words Semantically related words Sentences

Neuropsychological Examination

April '86 October '86

10/10 10/10 57160 59160

20120 20120 20120 20120 33/36 35/36

39 49

40/40 40/40 20120 20120 10/10 10/10

14/20 14120

14120 14/20 4/10 5110

10/10 10/10

20120 20120 10/10 10/10 10/10 10/10

40140 40140 40140 40140 10/10 10/10

The following April he underwent a neuropsychological examination at the Neuropsy­chology Laboratory of the Institute of Clinical Neurology, University of Pisa.

Language (Table I)

His speech was unimpaired, conveying a normal amount of reliable information. No phonologic, semantic or syntactic disorders emerged during either the description of the case history nor that of an event (Basso, Capitani and Vignolo, 1979). Oral naming, evaluated by means of the Boston Naming Test (Kaplan, Goodglass and Weintraub, 1983), was per­formed precisely and without hesitation, requiring no phonemic or semantic cues.

The patient made no errors in a semantic comprehension test (Gainotti et al., 1979), nor in understanding oral commands (Basso et al., 1979). The score on Token Test (De Renzi and Vignolo, 1962; shortened version of 36 items, De Renzi and Faglioni, 1978) confirmed the integrity of language comprehension functions.

Repetition tasks (Basso et al., 1979) gave normal results, as did verbal fluency, assessed by means of the F-A-S test of Borkowski, Benton and Spreen (1967).

Reading was also unimpaired: the patient read letters, syllables, words, semantically re­lated words, sentences (Basso et al., 1979) and stories without hesitation or error; he under­stood not only simple written orders but also complex stories perfectly and was able to give an excellent summary of their content, considering his level of school education.

Writing, on the other hand, was compromised. The patient was asked to write sentences and passages of various types as well as a written naming test (Gainotti et al., 1979) and to write words and sentences to dictation (Basso et al., 1979). In all these tasks there were rare elisions and substitution of letters, with some use of block letters replacing normal hand­writing; the letters n and m almost consistently had too many vertical stems (Figure 1). The patient was extremely slow in performing these tasks, with frequent hesitations and self-cor­rections. Copying, however, was normal.

Gerstmann Syndrome 461

Praxis (Table II)

Tests for buccofacial and ideomotor praxis (De Renzi, Pieczuro and Vignolo, 1966) proved normal. Constructional abilities were assessed using two tests: (a) Copying three sim­ple models (a star, a cube and a house - Gainotti, Miceli and Caltagirone, 1977); each drawing is assigned 2 points for essentially correct reproductions, 1 point if the reproduction is partially defective but not to such an extent as to prevent the identification of the figure, 0 points for unrecognizable reproduction. The patient obtained a low score in this test. (b) Three-dimensional block constructional praxis (Benton, 1968). The patient has to reproduce three structures built out of 6, 8 or 15 blocks of various size and shapes, selecting the neces­sary blocks from those in a tray. Here, the patient made some errors (displacements), but his score proved normal.

Memory (Table III)

Verbal memory was assessed with the following three tests: (a) Digit span (De Renzi and Nichelli, 1975). (b) Logical memory: immediate and 10 minute filled delay recall of a short story (De Renzi, Faglioni and Ruggerini, 1977), in which the score was the sum of idea units correctly reported in two trials. (c) Learning a 10-word-list to the criterion of two successful, consecutive repetitions, with maximum 20 trials (De Renzi et al., 1977), where two scores were obtained: the number of trials to criterion and the sum of the words correctly recalled over the trials divided by the number of trials to criterion. The patient performed normally on all three tests.

Visual memory was assessed with the "Immediate Visual memory Test" (Gainotti, Cal­tagirone and Miceli, 1978): abstract stimuli, using figural material of P.M. '47, were pre­sented, one at a time for about 3 sec. The stimulus was then removed and patient asked to point it out from among 4 different alternatives. The patient made six errors, obtaining a be­low-normal score.

Spatial memory was tested with Corsi's cube span test (De Renzi and Nichelli, 1975) and with a supra-span cube sequence learning test. Its score was trials to criterion to learn a se-

Fig. l - P.L. 's example of writing on dictation of a short story.

v~~p._ Uui~) ~ . ~ l-i ~ & c I~ + Q ri:_ ~.:. tc,_ ~-LC\ _\..1'.,'D.-l.>-£>

J- ~~~·,o •

\J(!_e.c..H 0 }~S¥-4Vlt ~~i~to

Ve.cc.l-1: e.f!"::>0-... 1

Q_ ~ea oG-'1-t_; o) o H CL C· ~~

462 M. Mazzoni and Others

TABLE II

P.L. 's Performance on Praxis Tests

April '86 October '86

Bucco-facial praxis 20/20 20/20 Ideomotor praxis 20/20 20/20 Constructional praxis

Copying drawings l/6 516 Three-dimensional block constructional praxis 26/29 29/29

TABLE lll

P.L. 's Performances on Memory Tests

April '86 October '86

Verbal tests Digit span Story recall 10-word-list:

trials to criterion mean of recalled words

Visual test Immediate visual memory

Spatial tests Cube span Cube supraspan learning (trials to criterion)

5.5 31

6 8.5

16

4 9

5.5 34

2 8

22

6 11

Normal values

> 15 > 16

> 4 ~26

Normal values

~4 > 15.75

< 14.32 >6.5

> 16

~4 <25

quence two cubes longer than the span (De Renzi, Faglioni and Previdi, 1977). The patient's performance was normal.

Calculation abilities

Calculation abilities were assessed with a written test (Grafman et al., 1981), which in­cluded addition (7 problems), subtraction (7 problems), multiplication (7 problems) and di­vision (6 problems). There were two different scoring systems: a quantitative score (number of digits that were correct numerically and put in the correct position; maximum= 106) and a qualitative score distinguishing six different types of error (misplacement and rotation: er­ror of spatial configuration; size error and distortion: error of form; omission and persev­eration: error of attention).

In this task P.L. achieved a good quantitative score (86/106), but made numerous qual­itative errors of malalignment and distortion and particularly evident mistakes in carrying and borrowing. He was unsure of himself, frequently going back to check his calculation and correct figures (Figure 2).

Right-left orientation (Table IV)

Evaluation of right-left orientation was performed adopting the criteria indicated by Benton (1966): (a) orientation with regard to one's own body: the patient points to parts of his body named by the examiner (e.g. right eye, left hand) or executes double commands, uncrossed (e.g. right hand on right eye) and crossed (e.g. right hand on left eye). These tasks were performed first with open eyes and then with closed eyes; (b) orientation with regard to another person: the patient identifies single lateral parts on the examiner's body, with open eyes; (c) combined orientation with regard to one's own body and that of another person:

Gerstmann Syndrome 463

Fig. 2 - P.L. 's examples of performance on calculation tasks.

55+ 835+ 89= 98279=

/4 l.i q q· p I 4

5000- 19-354 = 8= --£ :S ~ -4> q

60100- 60100-4712 = 4712=

p. Ji.142' 1i ~ {, 3~ ~

308x 73= 38x q 04 4=

2. s p -

~ 14 '

'8694~ 8694~ 1-~ I 111- 211 I~

I 0 li' -ss

TABLE IV

P.L. 's Performances on Right-left Orientation Tasks and on Finger Localization Test (see text for description)

Right-left orientation (1) Indication of single lateral parts on

the patient's own body (2) Indication of parts on the patient's

own body on dquble uncrossed and crossed commanas

(3) Identification of single lateral parts on the examiner's body

( 4) Combined identification of lateral parts on patient's own body and on that of the examiner

Finger localization test (A) right hand

left hand (B) right hand

left hand (C) right hand

left hand

April '86 Open Closed eyes eyes

11/12

4/8

2/4

0/4

10/10 10/10

9/12

3/8

10/10 8/10

16/20 10/20

October '86 Open Closed eyes eyes

11/12 10/12

618 518

3/4

114

10/10 10/10

10/10 9/10

16/20 12/20

464 M. Mazzoni and Others

Fig. 3 - CT scan (October 31, 1986) showing a cortical-subcortical hypodense area limited to the left angular gyrus region.

with open eyes the patient uses his right or left hand to touch a homolateral or controlateral part of the examiner's body, named by the examiner himself (e.g . "using your right hand touch my left ear"). One point was given for each correctly performed command.

P.L. made numerous mistakes. When asked to point out single lateral parts of his body, he seemed unsure, hesitating with open eyes and making mistakes with his closed eyes. On executing double uncrossed and crossed commands, he made still more mistakes and his per­formance did not differ with open or closed eyes. His ability to identify lateral parts of the examiner's body was also impaired, but the greatest number of errors emerged in the com­bined orientation test involving his own body and that of the examiner.

Finger agnosia (Table IV)

Assessment of finger agnosia was performed using the Benton finger localization test (1966), modified by Gainotti, Cianchetti and Tiacci (1972). The patient was given paper mo­dels of the back of each hand, with the fingers numbered from 1 (thumb) to 5 (little finger). He was then asked to touch the finger on the model corresponding to the finger on his own hand touched by the examiner; any language interference was thus avoided since fingers did not have to be named. First, with his hand visible, the patient had to localize a single finger touched by the examiner (test A, 10 stimuli for each hand): in this case he made no error on the right or the left hand. He was then asked to perform the same task with his hand hidden from view (test B, 10 stimuli for each hand): he made no errors on the right hand but did not recognise the third finger of his left hand, pointing once to the second and once to the fourth . Finally, he was asked to identify two fingers touched simultaneously while his hand was hid­den from view (test C, 10 double stimuli for each hand): 4 errors emerged on the right and 10 on the left. The errors almost all involved the three middle fingers.

We diagnosed dysgraphia, dyscalculia, right-left disorientation and finger agnosia (Gerstmann syndrome), and slight deficits of constructional praxis and visual memory.

The patient was examined again in October 1986. He reported continued impairment of his ability to write and to do calculations: " .. .I have to concentrate very hard, that's the only way to limit the number of mistakes ... I can't write or do any calculations without having to think hard ... ". His wife in fact had to help him with the administrative side of his business.

The results of the oral and written language tests were unchanged (Table I). The ability to copy drawings proved normal (Table II), as well as the visual memory test (Table III). The

Fig. 4 - CT scan mapping of the lesion. The continuous line marks the lesion area and the dashed line the edema area according to CT scan performed in February 1986; the dot­ted area shows the lesion area on CT scan performed in October 1986.

Gerstmann Syndrome 465

patient still made errors - though fewer than before - in right-left orientation, finger lo­calization (Table IV) and written calculations. On October 31, 1986, a second CT scan was obtained using last generation equipment: it showed a probably malacic cortical-subcortical hypodense area in the left posterior parietal region, which corresponded to the osteotomy (Figure 3). Both the lesion shown by this CT scan and that shown by the CT scan carried out in February were mapped according to the procedure described by Mazzocchi and Vignolo (1978): in the acute phase the presence of edema determined a broad cortical-subcortical in­volvement of the left parietal lobe, while eight months later damage was limited to the left angular gyms region.

DISCUSSION

The Gerstmann syndrome (1924, 1927, 1930) was initially accepted as a constellation of symptoms pointing to damage to the left angular gyms. However during the 1960s various authors (Benton, 1961; Heimburger et al., 1964; Poeck and Orgass, 1966; Critchley, 1966) questioned its existence as an autonomous syndrome and denied its clinical significance.

The full Gerstmann syndrome (presence of the four cardinal symptoms) is not a constant finding: there are incomplete forms with only two or three of the signs. Heimburger et al. (1964) demonstrated that when the number of symptoms increases, the lesion's area also in­creases, preventing a precise anatomical correlation. Other symptoms of parietal damage of­ten occur (anomia, alexia, constructional apraxia, memory disorders). Benton (1961) de­monstrated that the frequency of their association with the cardinal symptoms of the syn­drome is not different from that of the association of the four cardinal symptoms themselves and submitted that the Gerstmann syndrome was "an artifact of defective and biased obser­vation".

Based on study of patients with right, left or bilateral brain lesions, Poeck and Orgass (1966) concluded that a lesion in the left hemisphere can be predicted only if at least three of the four symptoms are present, but remarked that this has little diagnostic value since in such cases aphasia also occurs.

On the other hand, Strub and Geschwind (1974), describing a patient with the Ger­stmann syndrome but without language disorders, stressed that the association between the syndrome and aphasia is not necessary and, when present, it is likely due to the contiguity of the cortical area subserving each. The full Gerstmann syndrome would thus maintain a high localizing value in cases without aphasia. However, their patient presented a diffuse cortical

466 M. Mazzoni and Others

atrophy, making it difficult to prove that the symptoms derived from damage to a circum­scribed cortical region and not from the interaction of various aspecific impairments (Poeck and Orgass, 1975).

Benton (1977), after an analysis of these studies on Gerstmann syndrome, concluded that there was no evidence that the cardinal symptoms had a distinctive neuropsychology signif­icance, pointing out that the components of the syndrome were, "in reality, collective terms for diverse types of disability of a perceptual, praxis, linguistic and conceptual nature".

Unlike Strub and Geschwind's patient, our case is not susceptible of the same type of cri­ticism. There were no history or clinical indications of deterioration nor of aphasia, which was present in the first day only, and CT scan revealed an extremely localized lesion, deter­mined by the trauma and the subsequent surgery. As the cerebral mapping shows, once sta­bilized, the lesion corresponds to the left angular gyms.

The neuropsychological course in our patient is also worthy of interest. Immediately af­ter the operation he reported a constellation of several symptoms typical of parietal injury (dyslexia, dysgraphia, dyscalculia). A few days later dyslexia was no longer present and after one month only slight visual memory and constructional praxis deficits were still associated with the four symptoms of the Gerstmann syndrome. Eight months after the trauma, when edema and diaschisis had receded, only the cardinal symptoms of the Gerstmann syndrome remained, in accordance with selective damage of the left angular gyms.

Our case therefore gives credence to the existence of a pure Gerstmann syndrome in the absence of any aphasic disorder, strictly linked to a lesion limited to the left angular gyms. It is worth reminding that this location closely corresponds to the small area found by Mor­ris et al., (1984) to be the only one producing an associations of these four deficits when sti­mulated with subdural electrodes. Stimulation inferior to the left angular gyms evoked a more complex picture where elements of the syndrome appeared with other signs such as al­exia, constructional apraxia and anomia.

ABSTRACT

A case of Gerstmann syndrome following a trauma is presented. After one month the patient showed the four symptoms of the Gerstmann syndrome associated with slight visual memory and constructional praxis deficits. Eight months later, however, he showed only dyscalculia, dysgraphia, right-left disorientation and finger agnosia, in accordance with se­lective damage of the left angular gyms revealed by CT scan.

The findings seems to support the existence and the localizing value of Gerstmann syn­drome.

Acknowledgements. The authors whish to express their gratitude to Prof. L.A. Vignolo for his valuable advice on an earlier version of this paper, and to Dr. S. Cappa for his help on mapping CT scan.

REFERENCES

BAsso, A., CAPITANI, E., and VIGNOLO, L.A. Influence of rehabilitation on language skills in aphasic pa­tients. Archives of Neurology, 36: 190-1%, 1979.

BENTON, A.L. The fiction of the Gerstmann syndrome. Journal of Neurology, Neurosurgery and Psy-chiatry, 24: 176-181, 1961.

BENTON, A.L. Problemi di Neuropsicologia. Firenze: Editrice Universitaria Barbera, 1966. BENTON, A.L. La praxie constructive tridimensionnelle. Revue de Psychologie Appliquee, 18: 63-80, 1%8. BENTON, A.L. Reflections on the Gerstmann Syndrome. Brain and Language, 4: 45-62, 1977. BoRKOWSKY, J.C., BENTON, A.L., and SPREEN, 0. Word fluency and brain damage. Neuropsychologia,

5: 135-140, 1%7. CRITCHLEY, M. The enigma of Gerstmann's syndrome. Brain, 89: 364-381, 1966. DE RENZI, E., and FAGLIONI, P. Normative data and screening power of a shortened version of Token

Test. Cortex, 8: 364-381, 1978. DE RENZI, E., FAGLIONI, P., and PREVIDI, P. Spatial memory and hemispheric locus of lesion. Cortex,

13: 424-433, 1977.

Gerstmann Syndrome 467

DE RENZI, E., FAGLIONI, P., and RuaGERINI, C. Prove di memoria verbale di impiego clinico per la dia­gnosi di amnesia. Archivio di Psicologia, Neurologia e Psichiatria, 38: 303-318, 1977.

DE RENZI, E., and NICHELL!, P. Verbal and non-verbal short-term memory impairment following hem­ispheric damage. Cortex, 11: 341-354, 1975.

DE RENZI, E., PIECZURO, A., and VIGNOLO, L.A. Oral apraxia and aphasia. Cortex, 2: 50-73, 1966. DE RENZI, E., and VIGNOLO, L.A. The Token Test: a sensitive test to detect receptive disturbances in

aphasics. Brain, 85: 665-678, 1962. GAINOTTI, G., CALTAGIRONE, C., and MICELI, G. Immediate visual spatial memory in hemisphere-dam­

aged patients: impairment of verbal coding and of perceptual processing. Neuropsychologia, 16: 501-504, 1978.

GAINOTTI, G., CIANCHETTI, C., and TIACCI, C. The influence of the hemispheric side of lesion on non verbal tasks of finger localization. Cortex, 8: 364c38 l, 1972.

GAINOTTI, G., MICELI, G., and CALTAGIRONE, C. Constructional apraxia in left brain-damaged patients: a planning disorder? Cortex, 13: 109-ll8, 1977.

GAINOTTI, G., MICELI, G., SILVERI, M.C., and MASULLO, c. La disintegrazione semantico-lessicale nel­l'afasia: correlazione tra le varie modalita di espressione e di ricezione linguistica. Archivio di Psi­cologia, Neuro/ogia e Psichiatria, 15: 7-22, 1979.

GERSTMANN, J. Fingeragnosie: eine umschriebene Stoerung der Orientierung am eigenen Koerper. Wie­ner k/inische Wochenschrift, 37: 1010-1012, 1924.

GERSTMANN, J. Fingeragnosie und insolierte Agraphie: ein neues Syndrom. Zeitschrift fur die gesamte Neuro/ogie und Psychiatrie, 108: 152-177, 1927.

GERSTMANN, J. Zur Symptomatologie der Hirnlaesionen im Uebergangsgebiet der unteren Parietal- und mittleren Occipitalwindung. Nervenartz, 3: 691-695, 1930.

GRAFMAN, J., PASSAFIUME, D., FAGLIONI, P., and BOLLER, F. Calculation disturbances in adults with fo­cal hemispheric damage. Cortex, 18: 37-49, 1982.

HEIMBURGER, R., DEMYER, W., and REITAN, R. Implications of Gerstmann's syndrome. Journal of Neu­rology, Neurosurgery and Psychiatry, 27: 52-57, 1964.

KAPLAN, E., GOODGLASS, H., and WEINTRAUB, s. Boston Naming Test. Philadelphia: Lea and Febiger, 1983.

MAzzoccm, F., and VIGNOLO, L.A. Computer assisted tomography in neuropsychological research: a simple procedure for lesion mapping. Cortex, 14: 136-144, 1978.

MORRIS, H., LUEDERS, H., LESSER, R., DINNER, D., and HAHN, J. Transient neuropsychological abnor­malities (including Gerstmann's syndrome) during cortical stimulation. Neurology, 34: 877-883, 1984.

POECK, K., and 0RGASS, B. Gerstmann's syndrome and aphasia. Cortex, 2: 421-437, 1966. PoEcK, K., and 0RGASS, B. Gerstmann syndrome without aphasia: comment on the paper by Strub and

Geschwind. Cortex, 11: 291-295, 1975. STRUB, R., and GESCHWIND, N. Gerstmann syndrome without aphasia. Cortex, JO: 378-387, 1974.

Monica Mazzoni, Istituto di Clinica Neurologica, via Roma 67, 56127 Pisa, Italy.