Gerstmann Syndrome"

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Gerstmann Syndrome J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.24.2.176 on 1 May 1961. Downloaded from J. Neurol. Neurosurg. Psychiat., 1961, 24, 176. THE FICTION OF THE "GERSTMANN SYNDROME" BY A. ARTHUR L. BENTON From the Departments ofNeurology and Psychology, University of Iowa, Iowa City, Iowa, U.S..4. The problem with which this study is concerned designating it as "a circumscribed disorder of can be stated as follows. A patient with parieto- orientation to one's own body". In 1927, he occipital disease may show one or more of a relatively advanced the idea that finger agnosia and agraphia I4 large number of diverse behavioural deficits. When formed "a new syndrome". However, in 1930 he he presents with two, three, or more of these enlarged the syndrome to include right-left dis- symptoms, the latter may be viewed by the clinical orientation and acalculia and proposed that it had observer as forming a naturally occurring combina- a highly specific neuropathological significance. At tion of deficits and given the status of a syndrome, the same time, he discussed the question of the this status implying that the concurrence of deficits Grundstorung responsible for this presumably is not a chance one, that there is an underlying "natural" concurrence of behavioural deficits. factor responsible for it, and that it possesses a Clinical experience appeared to confirm the real distinctive neuropathological significance. Once existence of the syndrome; case reports of patientsProtected by copyright. such a special combination or syndrome is esta- manifesting it appeared in the literature and specula- blished, not only is it used in the observation and tions regarding the basic impairment underlying its description of subsequent cases but it may also occurrence were offered. However, it was certainly determine which aspects of a patient's behaviour are not rare to encounter patients who showed one, two, selected for study and which are not. The "Gerst- or three deficits but not the full syndrome. While mann syndrome" represents such a combination of such observations were often interpreted as simply behavioural deficits. The study to be reported representing formes frustes of the syndrome, they examines the question of whether, as is usually did indicate at least that the four symptoms did not assumed, this "assembly of unlikely and unexpected necessarily occur together in every case and they did symptoms" (Critchley, 1953) is a naturally occurring have implications regarding the cogency of those combination or whether it is only one of a very large theoretical formulations which had been advanced number of more or less fortuitous combinations of to account for an obligatory concurrence of the behavioural deficits which may be encountered in deficits. On the other hand, a wide variety of other patients with cerebral disease. deficits were also observed to occur in connexion The history of the Gerstmann syndrome has been with any or all of the four symptoms. These were recounted in detail elsewhere (cf. Critchley, 1953; given a subordinate status as accompanying symp- http://jnnp.bmj.com/ Benton, 1959); only a few major points need be toms which might or might not be manifested. mentioned to introduce the present investigation. Among them were general mental impairment, Before the end of the nineteenth century, the four aphasic disorders, colour agnosia, visual disorienta- behavioural deficits comprising the syndrome- tion, dyslexia, and constructional apraxia. Indeed, right-left disorientation, acalculia, agraphia, and the last-named deficit, constructional apraxia, has finger agnosia-had been described as occurring in been observed so often to accompany one or more patients with cerebral disease. The first three were of the four deficits that some authors have come to well-known symptoms but the description of finger consider it, explicitly or implicitly, as part of the on September 23, 2021 by guest. agnosia in 1888 by Jules Badal had escaped the syndrome. For example, Ajuriaguerra and Hecaen attention of neurologists, a fact which is not al- (1960), discussing the interrelations of the elements together surprising in view of the circumstances that of the syndrome and the connexions with other the Bordeaux eye specialist published in an ophthal- symptoms, remark that "the most frequent and most mological journal, and the finger agnosia which he important association is that between finger agnosia quite clearly described was only one of a large and constructional apraxia. We have seen that such number of deficits shown by his patient. In 1924, an association cannot be considered to be simply Gerstmann once again described finger agnosia, fortuitous or anatomically conditioned". 176 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.24.2.176 on 1 May 1961. Downloaded from THE FICTION OF THE "GERSTMANN SYNDROME" 177 If the "Gerstmann syndrome" is a naturally Case Material and Methods occurring combination of interrelated deficits, all of The case material consisted of 100 non-psychotic which are expressions of a single basic impairment, patients with unequivocal diagnoses of disease or injury and not merely the product of selective attention and involving the cerebral hemispheres who had been seen in observation, the four behavioural deficits which the neurological and neurosurgical services of the comprise it should show stronger "associative University Hospitals and Veterans Administration than Hospital, Iowa City. Any patient who was suffering from bonds", so to speak, among themselves they do acute illness, whose behavour raised the question of with symptoms which are considered to be outside psychosis, who had a history of treatment in hospital for of the syndrome. If unbiased analysis should show a psychiatric disorder, or whose history was suggestive this to be the case, the findings could be reasonably of a state of mental defect dating back to childhood, was interpreted as evidence for a special coherence of excluded from the study. All the patients were between the four symptoms and both the designation of the the ages of 16 and 64 years and were clearly capable of combination as a syndrome and a search for the understanding directions, cooperating in the test pro- common factor responsible for the coherence are cedures and undergoing one to two hours of examination justified. On the other hand, if such analysis should without discomfort. The mean age of the group was disclose that this is not the the could 42 years. The mean educational level was 10 years (range: case, findings 5 to 16 years). A variety of cerebral conditions, focal and be reasonably interpreted as indicating that this diffuse, was represented in the group. The performances particular combination of deficits is no more or less of 12 right-handed patients with focal disease involving likely to occur in patients with cerebral disease than the left parietal lobe were analysed separately, in addition a great many similar combinations and that, unless to the main analyses of the total group of 100 patients it can be shown to have a distinctive neuropatho- of which they formed a part. logical significance, neither its designation as a A control group of 100 non-psychotic patients from the syndrome nor the postulation of a common neurological, neurosurgical, and medical services who showed no evidence or history of cerebral disease or psychoneurological factor to account for it Protected by copyright. (as distinguished from other is injury were also examined, their performances being combinations) utilized to establish objective normative standards on the justified. basis of which the presence or absence of impairment It might be thought that a comprehensive survey could be defined. The mean age of this group was of the pertinent clinical literature would provide the 41 years (range: 16 to 65 years) and mean educational answer to this question of the phenomenological level was 10 years (range: 5 to 14 years). reality of the Gerstmann syndrome. However, the All the patients were given a battery of seven tests literature proves to be virtually without value in this designed to provide objective and comprehensive assess- respect. The methods and criteria utilized for ments of the following capacities: (1) right-left orienta- making clinical judgments regarding the presence or tion; (2) finger localization; (3) arithmetic calculation; (4) writing; (5) constructional praxis; (6) reading; (7) absence of defect in the performances investigated visual are so diverse that the findings from one case to memory. The testing procedures were as follows: another are not comparable. Not infrequently the Right-left Orientation.-This test battery (32 items) criteria employed in making judgments of deficit required the patient to execute localizing movements to are not mentioned and notations such as "slight oral command and assessed the following aspects of finger agnosia", "questionable right-left disorienta- right-left orientation: (A) with the aid of vision, identi- tion", or "no noteworthy constructional apraxia" fication of single lateral body parts on one's own body; http://jnnp.bmj.com/ are made. Moreover, the majority of case reports (B) with the aid of vision, execution of double uncrossed fail to mention the presence or absence of one or and crossed commands with respect to lateral body more pertinent accompanying deficits and hence parts on one's own body, e.g., left hand on right eye; must be (C) without the aid of vision, identification of single excluded from a systematic analysis. lateral body parts; (D) without the aid ofvision, execution Finally, the factor of selective attention and obser- of double commands; (E) identification of single lateral vation when one is dealing with an already esta- body parts on a front-view representation of a man blished entity, such as the Gerstmann syndrome, (F) execution of double commands involving identi- may introduce a bias in the original observations for fication of lateral body parts of both the patient and the on September 23, 2021 by guest.
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