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Brain, Vol. 122, No. 6, 1107-1120, June 1999
© 1999 Oxford University Press

A pure case of Gerstmann syndrome with a subangular lesion

Eugène Mayer1, Marie-Dominique Martory1, Alan J. Pegna1, Theodor Landis2, Jacqueline Delavelle3 and Jean-Marie Annoni1

1 Neuropsychology Unit, 2 Neurology Clinic and 3 Neuroradiology Unit, Department of Radiology, Geneva University Hospital, Switzerland

Correspondence to: Eugène Mayer, Neuropsychology Unit, Neurology Clinic, Geneva University Hospital, 1211 Geneva 14, Switzerland E-mail: euma{at}diogenes.hcuge.ch

The four symptoms composing Gerstmann's syndrome were postulated to result from a common cognitive denominator (Grundstörung) by Gerstmann himself. He suggested that it is a disorder of the body schema restricted to the hand and fingers. The existence of a Grundstörung has since been contested. Here we suggest that a common psychoneurological factor does exist, but should be related to transformations of mental images rather than to the body schema. A patient (H.P.) was studied, who presented the four symptoms of Gerstmann's syndrome in the absence of any other neuropsychological disorders. MRI showed a focal ischaemic lesion, situated subcortically in the inferior part of the left angular gyrus and reaching the superior posterior region of T1. The cortical layers were spared and the lesion was seen to extend to the callosal fibres. On the basis of an extensive cognitive investigation, language, praxis, memory and intelligence disorders were excluded. The four remaining symptoms (finger agnosia, agraphia, right–left disorientation and dyscalculia) were investigated thoroughly with the aim of determining any characteristics that they might share. Detailed analyses of the tetrad showed that the impairment was consistently attributable to disorders of a spatial nature. Furthermore, cognitive tests necessitating mental rotation were equally shown to be impaired, confirming the essentially visuospatial origin of the disturbance. In the light of this report, the common cognitive denominator is hypothesized to be an impairment in mental manipulation of images and not in body schema.

Gerstmann's syndrome; MRI; acalculia; mental imagery

VC = visual control


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