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Wernicke's Disease J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.2.134 on 1 April 1969. Downloaded from J. Neurol. Neurosurg. Psychiat., 1969, 32, 134-139 Vestibular paresis: a clinical feature of Wernicke's disease CLAUDE GHEZ From the Division ofNeurology, Cleveland Metropolitan General Hospital, and Case Western Reserve University School ofMedicine, Cleveland, Ohio, U.S.A. Wernicke's disease is a well-defined clinical and with unequivocal signs of Wernicke's disease. All showed pathological entity, and its aetiology has been an initial global confusional state and variable degrees of conclusively traced to thiamine deficiency (Phillips, memory loss as a persistent finding. All patients had Victor, Adams, and Davidson, 1952). Its essential nystagmus and severe ataxia of gait as well as signs of features are the subacute onset of confusion, mild polyneuropathy. Ophthalmoplegia was seen in 12 ophthalmoplegia and/or nystagmus, and ataxia patients (bilateral abducens palsies in 11 and total of external in stance and gait. It invariably occurs against a ophthalmoplegia one) and lasted from one to background of seven days. Mild cerebellar signs of the lower extremities malnutrition and often chronic could be elicited in nine and of the alcoholism. and the upper extremities in Ophthalmoplegia, ataxia, global two. Vertigo was not a complaint in any. Thiamine confusion respond readily to thiamine admin- was hydrochloride (100 mg) administered intramuscularlyProtected by copyright. istration, but the patient is often left with a upon admission to all patients and orally for several days conspicuous disorder in memory (Korsakoff's thereafter. All patients were examined neurologically in psychosis), and not infrequently some degree of a serial fashion. Head injuries were not a factor in any of ataxia when attempting to walk on a narrow base the patients and routine skull films were normal. All (heel to toe). patients had been hospitalized throughout the course of In the acute stages of the disease there is this study during which time they received an adequate frequently diet and no alcohol. a striking discrepancy between the patient's ability Control to stand and subjects were 12 chronic alcoholic patients, walk and his ability to perform tests seven with delirium tremens and five with alcoholic designed to assess cerebellar function-that is, cerebellar degeneration (Victor et al., 1959). The latter heel-to-shin, finger-to-nose, etc. The patient may group had no impairment in mental function or literally be unable to take even a few steps without ophthalmoplegia. Their neurological deficit consisted of support; his legs are rigidly held wide apart, the mild ataxia of stance and gait with an associated sym- body pitched forward as he clutches the nearest metrical ataxia of legs and to a lesser extent of the arms. solid The object for fear of falling. In a study of 245 patients onset of the disturbance had been gradual and no with Wernicke's disease by Victor, discernible episode of Wernicke's disease had occurred. Adams, Collins, No significant change in the of their severity ataxia was http://jnnp.bmj.com/ and Silby (to be published), it was found that ataxia noted of stance during the course of their hospital stay. and gait occurs in 87 %, whereas ataxia of Vestibular function was assessed the lower extremities in by means of a only 20 %. standard ice water caloric test. The ears were irrigated for One interpretation of this finding attributes the 30 seconds with 5 ml. ice water with at least a five minute ataxia of stance and gait to lesions in the midline interval between the testing of each ear. The patient was cerebellar vermis, and the ataxia of the lower instructed to fix his gaze on a point straight ahead and the extremities to an extension of the lesion into more time elapsing between the time of injection and end of the lateral areas of the anterior lobes ensuing nystagmus was determined. Past pointing with (Victor, Adams, eyes closed and Mancall, 1959). Vestibular before and after stimulation was evaluated. on September 28, 2021 by guest. function, which is Tests were known to be of paramount in the performed on more than one occasion in importance regula- 14 patients. Four were tion of postural adjustments, has not heretofore patients tested only twice; the been other 10 were tested on multiple occasions, on alternate determined. This study was undertaken to evaluate days at the beginning and at weekly and monthly intervals vestibular function in the acute and chronic stages later in the course of their illness. of Wemicke's disease. Five patients were tested for directional preponderance by means of a modification of the method of Fitzgerald MATERIAL AND METHODS and Hallpike (1942) in which the ears were alternately irrigated with 75 ml. water at 30 and 44°C. The subjects were 17 chronically malnourished patients With one none of our admitted to Cleveland exception, patients had any Metropolitan General Hospital hearing deficit by history or on routine clinical testing 134 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.2.134 on 1 April 1969. Downloaded from Vestibular paresis: a clinicalfeature of Wernicke's disease 135 (Weber, Rinne, and Schwabach). Pure tone audiograms Three patients showed past pointing towards the were obtained in six patients and the more sensitive side of the ice water irrigation, even though nystag- Von Bekesy audiograms, tone decay, and Short mus was completely absent. Vertigo, however, never Increment Sensitivity index were done in three cases. occurred without nystagmus. In contrast, the responses of all control subjects RESULTS were within normal limits. In 10, contralateral nystagmus developed on calorization and lasted . RESPONSES TO ICE WATER IRRIGATION (a) Ab- between two and three minutes. Asymmetries normalities on initial testing (Table I) Despite some between ears greater than 20 seconds were not seen. variation in the time at which the first test was Two patients suffering from delirium tremens who carried out, all 17 patients showed profound were heavily sedated with paraldehyde and chlor- abnormalities in vestibulo-ocular responses. In 11 diazepoxide showed ipsilateral tonic-ocular deviation patients the responses were absent bilaterally and in about two minutes six patients unilateral unresponsiveness was seen lasting following irrigation. (even in the first few days following hospitalization). (b) Mode ofrecovery Some degree of recovery of The presence of abducens palsies did not interfere vestibular function was ascertained by serial caloric with the reflex which could be recognized in the testing in 10 patients. The onset of recovery varied adducting eye. from six to 60 days, and in seven patients it began in the second week following thiamine administration (Table I). TABLE I In seven patients, the tests were carried out at INITIAL RESPONSES AND ONSET OF RECOVERY intervals sufficiently short so that the manner of evolution of the caloric responses could be assessed. Patients Protected by copyright. Absent response on initial These data are illustrated by five representative cases examination (no.) (Y%) in Fig. 1 and indicate that recovery occurs in a (17 patients) 17 100 gradual, often asymmetrical, fashion and can be Bilateral 11 64 7 prolonged and incomplete. On occasion, the Unilateral 6 35-3 duration of nystagmus was longer than normal, Onset ofrecovery (10 patients) although not necessarily in relation to directional Range 6 days-2 months preponderance (Fig. 3). In the early phases of 7 patients 6-16 days recovery, marked irregularity and a reduced 3' R 2' OAR CD http://jnnp.bmj.com/ 0 I I . ~ ~ ~~~~~.. .. .. .. :3 *~~~~~~~~vWS. 1A * WR. FIG. 1. Evolution of caloric re- cc o H.J sponses-selected cases. The _3C duration of nystagmus represents - the time from the beginning of the z injection to the end of the ensuing nystagmus. c 3ct - on September 28, 2021 by guest. E 0 ~~~~~~~% ..% .....% ..... .... ..................................... ............ L .:::.:.:_::: _................................................--0....................-@-N....T-R.---.--.................L.......... 3 10 20 30 40 50 60 70 ....~~~~~~~~~~~~~........._. .z.................Hospital Days J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.32.2.134 on 1 April 1969. Downloaded from 136 Claude Ghez amplitude of the nystagmic movements were com- monly observed and could be easily appreciated by visual inspection alone. The cumulative data from all patients are presented graphically in Figure 2. It was found that .-j after the tenth day following therapy the proportion of patients showing abnormal caloric responses started to decline. At eight months, 20 % were -i abnormal. It must be stat-ed that this latter figure represents an approximation of what is to be z expected in the chronic phase of Wernicke's disease co in view of the fact that at eight months a smaller number of patients were available for testing than in the acute stages of the disease. Caloric responses were deemed abnormal either if the duration of nystagmus was less than 1 minutes or if an asymmetry greater than 30 seconds between sides was found. Abnormal responses consisted in the TIME complete absence of vestibulo-ocular response on one or both sides, with the exception of six patients FIG. 2. Evolution of caloric responses-cumulative data. at 10 days, one at 20 days, and one at four months. Caloric responses were deemed abnormal if the duration of nystagmus was less than 1 min 30 sec or if the difference between the two sides was 2. DIFFERENTIAL CALORIC RESPONSES. DIRECTIONAL greater than 30 sec. PREPONDERANCE (Fig. 3) Significant directional Protected by copyright. preponderance was found in only one of five patients in whorn it was specifically sought during 300<r recovery. In this patient is was associated with Day 16 depressed responses to both warm and cold stimuli I44.1ii<~ t on one side. W.s. 3. AUDIOMETRY With one Day 23 exception, audiometry 440R F failed to reveal any significant abnormality despite AL the concurrent existence of severe vestibular paresis.
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