J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.27.1.52 on 1 February 1964. Downloaded from J. Neurol. Neurosurg. Psychiat., 1964, 27, 52 Implications of Gerstmann's syndrome ROBERT F. HEIMBURGER, WILLIAM DEMYER, AND RALPH M. REITAN From the Sections of Neurological Surgery, Neurology, and Neuropsychology, Indiana University Medical Center, Indianapolis, Indiana, U.S.A. Four behavioural deficits-finger agnosia, right-left finger as soon as it was touched. Occasionally, a patient disorientation, dysgraphia, and dyscalculia-have needed practice with his eyes open to develop sufficient come to be known as Gerstmann's syndrome. skill to report reliably during the test. This precaution that the concurrence eliminated errors due to misunderstanding or lack of Generally, clinicians consider alertness. No patient was included whose tactile sensitivity of these deficits implies a lesion in the angular gyrus was sufficiently impaired to preclude perception of the of the language-dominant hemisphere. Gerstmann tactile stimulus. All mistakes of identification were (1957) has reiterated his earlier contention that the recorded for each patient. The patient was considered to syndrome is an entity with specific localizing sig- have finger agnosia if he made incorrect responses in a nificance but Benton (1961) has reported that the minimum of 20% of the trials. components of the syndrome have no stronger Examination for right-left disorientation (a) The associative bonds than a variety of concurrent patient was shown a card with the printed instruction, intellectual deficits. He concludes that the syndrome 'Place left hand to right ear'. He was requested to read theProtected by copyright. bias and unsystematic, in- command aloud. If his reading was incorrect, the is an artifact of observer examiner communicated the instructions to the patient complete examination for associated deficits. verbally. Then the patient was requested to execute the In this study, we review some of the neurological instruction. (b) The patient was then requested to place deficits in patients with Gerstmann's syndrome and his left hand on his left elbow. If he became confused in relate these deficits to the location and type of brain executing part (a) or failed to recognize the impossibility lesion. of part (b), further commands of a similar nature were given to verify the presence of right-left disorientation. METHODS The results of previous testing of the patient's ability to comprehend visual and auditory instructions provided a PATIENT POPULATION At Indiana University Medical basis for distinguishing specific errors of right-left dis- Centre a large percentage of neurological and neuro- orientation from global receptive dysphasia. surgical patients suspected of having brain damage is Examinationfor dysgraphia (a) The patient was shown given an extensive battery of neuropsychological tests. a drawing of a clock and requested to write the name Since these tests require active cooperation by the patient, without first saying it. (b) He was shown the printed severely psychotic, stuporous, or recalcitrant patients are letters, SQUARE, and asked to transform the stimulus not tested. Otherwise, the patients were unselected except material to script. (c) After having read the word, SEVEN, that they were clinically suspected ofhaving brain damage and repeated it following oral presentation by the of some type. From 456 consecutively tested patients, 111 examiner, he was asked to write the word. (d) The patienthttp://jnnp.bmj.com/ had one or more components of Gerstmann's syndrome. was instructed to repeat the sentence, 'He shouted the warning', explain its meaning, and write it. In additional METHOD FOR ELICITING COMPONENTS OF GERSTMANN S instances the patient was asked to write the names of SYNDROME As an integral part of the neuropsychological common geometric figures after having copied their test battery, the Halstead-Wepman aphasia screening shapes. Provided that the patient's previous educational test (1949), as modified by Reitan, was given 'to each achievement indicated that he should be able to perform patient. The test contains 27 auditory and visual stimuli, correctly, failure to effect proper formation of successive presented in identical order and with identical instructions letters was considered to indicate dysgraphia. Simple to each patient. spelling errors were excluded. The Figure shows examples on October 1, 2021 by guest. Examination for finger agnosia First a system for of dysgraphia. It i.apparent that these patients had their designating the fingers was agreed upon by the patient and principal difficulty with the sentence, 'He shouted the the examiner. Usually the patients reported by number warning' but the second instance in the Figure illustrates but sometimes other designations were used. After the the occasional occurrence of extreme difficulty with patient's vision was obstructed by a blindfold or shield, individual words. his outstretched fingers were touched lightly by the Examination for dyscalculia (a) The patient was re- examiner. The patient was instructed to identify the quired to copy '85-27= 'from a card and compute it. 52 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.27.1.52 on 1 February 1964. Downloaded from Implications of Gerstmann's syndrome 53 --- cation of the patient as derived from the neuropsycho- r -q--,P C,& . Ct t,4 logical battery. I Five years after the initial study was completed, the records of the 111 patients with one or more Gerstmann components were again reviewed to check the course of A4. C patients whose diagnosis or localization of lesion had been obscure initially. This precaution minimized errors in CA...,4 Ctriangle) ce,.A.. diagnosis or localization of the lesion. RESULTS ?b~- ~44d& 4.,j-4 LO A~ Of 456 consecutive neuropsychologically tested patients, 111 had one or more components of Gerstmann's syndrome (Table I). Among the 111 3 M7t7 *su patients, 33 had only one of the components of the /V<4kSi syndrome, 32 had two components, 23 had three components, and 23 had all four components of the 2nd attempt syndrome. We will refer to these groups as group I, FIG. 1. 1 Example by 45-year-old white male with 10 11, III, or IV, depending on the number of Gerst- years' education; 2 of 42-year-old white male with seven mann components. years' education; 3 of 71-year-old white female with The one common characteristic of the 111 patients 14 years' education. is that all had organic (non-emotionally determined) neurological disorders. The evidence for the organic (b) He was required to multiply 17 by 3 in his head without neurological diagnosis in each patient was derived seeing the figures written down. If these problems were from the neurological work-up, independently of the Protected by copyright. not correctly solved, similar problems were presented for number of Gerstmann components. According to verification of dyscalculia. the type of lesion or diagnosis, the patients were METHOD FOR LOCALIZING BRAIN LESIONS Each of the classified into five categories as follows (Table I): patients with one or more components of Gerstmann's 1 Cerebrovascular disease, including cerebral in- syndrome was evaluated by review of the medical history, farction, intracerebral haematomas, and generalized physical and neurological examination, and, when arteriosclerotic brain disease; 2 brain tumours, available, skull radiographs, neuroradiological contrast including gliomas, meningiomas, metastatic neo- studies, electroencephalograms, surgical notes, and plasms, and abscesses; 3 trauma, including open and necropsy findings. From the neurological information, closed head injuries; 4 diffuse or symmetrical multi- a drawing was made on brain diagrams of the presumed focal cerebral disease, including encephalitis, multiple site and extent of brain damage, and the type of lesion or sclerosis, pre-senile degenerative diseases, carbon diagnosis was recorded. The patients were then classified monoxide or 5 according to aetiology, lateralization of lesion, and intra- drug intoxication, and syphilis; hemispheric location of the lesion. In preparing the brain convulsions, including only patients considered to lesion charts, the results of the neuropsychological test have idiopathic epilepsy. (Only three patients were battery were ignored, but all other clinical and anatomical diagnosed as having idiopathic epilepsy, and they information was utilized. At the time of the original case exhibited only one or two components of Gerst- review, the referee did not know the Gerstmann classifi- mann's syndrome.) http://jnnp.bmj.com/ TABLE I NUMBER, AGE, AND TYPES OF LESION FOR PATIENTS WITH ONE THROUGH FOUR COMPONENTS OF GERSTMANN'S SYNDROME Group Number of Mean Age Age Range % of Group Lesion Type (% of Group) Patients (yr.) (yr.) over 40 in Group Years Old Vascular Tumour Trauma Multifocal Idiopathic or Diffuse Epilepsy 33 42 21-68 55 21 18 33 21 6 on October 1, 2021 by guest. II 32 40 11-71 41 25 22 22 28 3 III 23 45 17-68 61 30 35 1 3 22 0 IV 23 50 16-77 74 48 43 4 0 0-10 0-10 0-025 0-20 0-70 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.27.1.52 on 1 February 1964. Downloaded from 54 Robert F. Heimburger, William Demyer, and Ralph M. Reitan On comparing groups I-IV, we found a shift in the the mid-brain is 48% in group I and 92% in group character of the lesions (Table I). In group I, the IV; right or left-sided delta focus in the E.E.G., lesions tended to be diffuse, relatively small, and 16% versus 60%; E.E.G. focalization in the posterior static. In group IV, the lesions tended to be large, part of the left cerebral hemisphere, 20% versus highly destructive of tissue, and progressive or re- 73%; known dead at five years, 21 % versus 48%; current. The combined percentage of patients with and the incidence ofdysphasia other than Gerstmann either a brain tumour or cerebrovascular disease in components, 39% versus 100%.