It is perhaps in part due to the emphasis in previous editions of this book that alcoholism has been inordinately associated with this disease complex. The disease arises in many other clinical settings. One of Wernicke’s original cases, for example, occurred in a woman with hyperemesis gravidarum and such instances are still found. However, bariatric surgery, cancer chemotherapy, inanition in HIV and from anorexia nervosa, and even in the frailty of older age, in nutritionally susceptible persons, starvation for economic and social reasons all may give rise to thiamine deficiency. Even the elderly and frail who subsist for years on “tea and toast” can acquire the disease. In addition, there are common medical circumstances in which a subclinical thiamine deficiency becomes manifest. Perhaps the most important of these is a carbohydrate load, particularly the administration of intravenous glucose to a malnourished individual; other precipitants are unbalanced intravenous hyperalimentation, refeeding syndrome, thyrotoxicosis, and hypomagnesemia.
Autopsy studies demonstrate that the many cases of Wernicke disease are undiagnosed. As summarized in the review by Sechi and Serra of published series from several countries, there is a discrepancy between the detection of the process in autopsy series, 0.5 to 3 percent, and the prevalence of the clinical diagnosis, 0.04 to 0.13 percent, indicating that approximately three-quarters of cases are not recognized during life.
Historical note In 1881, Carl Wernicke first described an illness of sudden onset characterized by paralysis of eye movements, ataxia of gait, and mental confusion. His observations were made in 3 patients, of whom 2 had alcohol dependency and malnutrition and 1 was a young woman with persistent vomiting following the ingestion of sulfuric acid. In each of these patients there was progressive stupor and coma culminating in death. The pathologic changes described by Wernicke consisted of punctate hemorrhages affecting the gray matter around the third and fourth ventricles and aqueduct of Sylvius; he considered these changes to be inflammatory in nature and confined to the gray matter, hence his designation “polioencephalitis hemorrhagica superioris.” In the belief that Gâyet had described an identical disorder in 1875, the term Gâyet-Wernicke is used frequently by French authors. Such a designation is hardly justified insofar as the clinical signs and pathologic changes in Gâyet’s patients differed from those of Wernicke’s patients in all essential details.
The first comprehensive account of this disorder was given by the Russian psychiatrist S.S. Korsakoff in a series of articles published between 1887 and 1891 (for English translation and commentary, see reference by Victor and Yakovlev). Korsakoff stressed the relationship between “neuritis” (a term used at that time for all types of peripheral nerve disease) and the disorder of memory seen in patients with alcoholism, which he believed to be “2 facets of the same disease” and which he called “psychosis polyneuritica.” But he also made the points that neuritis need not accompany the amnesic syndrome and that both disorders could affect nonalcoholic as well as alcoholic patients. His clinical descriptions were remarkably complete and have not been surpassed to the present day. It is of interest that the relationship between Wernicke disease and Korsakoff polyneuritic psychosis was appreciated neither by Wernicke nor by Korsakoff. It was Murawieff, in 1897, who first postulated that a single cause was responsible for both. The intimate clinical relationship was established by Bonhoeffer in 1904, who stated that in all cases of Wernicke disease he found neuritis and an amnesic psychosis. Confirmation of this relationship on pathologic grounds came much later. For further details the reader is referred to the extensive monograph by Victor et al (1989).