Abstract
In the literature concerning alcoholic brain damage, Wernicke–Korsakoff syndrome (WK) is considered a distinct entity, in which memory is impaired disproportionately to other cognitive functions [1]. However, neuropathological investigations have indicated that a substantial proportion of brains of people with alcoholism exhibit lesions originally considered to be specific to WK, leading to a ‘continuum’ hypothesis, in which repeated subclinical episodes of WK represent the underlying cause of so-called alcoholic dementia [2], [3]. In regard to this hypothesis, as subclinical episodes inherently cannot be demonstrated in clinical series, clinical evidence for this argument might be provided by case reports demonstrating repeated clinical episodes of WK. However, to our knowledge, no such cases have yet been reported; possibly owing in part to the variable clinical manifestation of WK, leading to difficulty in correctly diagnosing this syndrome. We report a case involving repeated episodes of WK that fulfilled clinical diagnostic criteria.
The patient, a 51-year-old man with alcohol dependence syndrome, had convulsions following a drinking bout in December 2000. On admission, confusion and horizontal nystagmus were evident and poor nutritional state was noted. Although his general condition improved after 90 days of treatment, severe amnesia was noted. One year after admission, WAIS-R was normal (FIQ = 91) but delayed recall in WMS-R was impaired (< 50) and the patient could not achieve even a single category on the Wisconsin Card Sorting Test (WCST). Diagnostic criteria for WK [4] were therefore fulfilled at this stage.
In September 2002, the patient was readmitted after a further drinking bout. The classical WK triad of ophthalmoplegia, gait ataxia, and confusion was evident. Laboratory testing was unremarkable, other than the following: vitamin B1, 18.3 ng/mL (normal range: 18.4–53.4), γGTP 165 IU (3–60), choline esterase 0.5 ΔPH (0.8–1.3) and total protein 5.6 g/dL (6.5–8.3). MRI revealed atrophy of the mamillary body and a high intensity area around the ventricle from the cerebral aqueduct to the third ventricle. EEG demonstrated mild diffuse bilateral slowing.
After recovery, neuropsychological testing revealed little change in cognitive function (FIQ in WAIS-R: 94; delayed recall in WMS-R: < 50; categories achieved in WCST: 0). However, motivation and spontaneity had deteriorated, and occasional compulsive behaviour (clothes washing) emerged.
The present case, in demonstrating repetition of clinically distinct episodes of WK, provides clinical evidence supporting the hypothesized continuum between WK and alcoholic dementia. In this patient, although amnesia and frontal lobe dysfunction (which emerged after the first episode) continued, other cognitive functions remained normal even after the second episode. Although at first glance this does not support the hypothesis that repeated episodes of WK lead to cognitive deterioration, emotional and behavioural changes after the second episode were consistent with alcoholic dementia. Further prospective investigations are obviously required to elucidate the pathogenesis of this syndrome.