Wernicke–Korsakoff Syndrome

   Wernicke and Korsakoff are two very familiar eponyms that only recently have been joined. First: Korsakoff. There is a Korsakoff syndrome, also referred to as Korsakoff psychosis, that the Russian psychiatrist Sergei S. Korsakoff (1853–1900) discovered in 1887. (See DEMENTIA: Korsakoff [1887].) The syndrome refers mainly to memory loss about time and space, confabulation, and polyneuritis. (The personality is preserved.) It is caused by a deficiency of the vitamin thiamine and typically is seen in chronic alcoholism.

   There is also a Wernicke’s disease: In 1881, Breslau psychiatry professor Carl Wernicke (1848–1905) described in his neurology textbook (Lehrbuch der Gehirnkrankheiten) a disease that he called "acute, hemorrhagic polioencephalitis superior," meaning that the histopathology resembled that of poliomyelitis in the spine, yet it was situated in the brain and was clinically characterized by limited eye movement, ataxic gait, and disorientation. The three cases he discussed in his textbook had quickly ended fatally. Two of the three had been heavy drinkers. This was the first description of what became known as "Wernicke’s disease," a sometimes fatal form of necrosis (death) of brain tissue.

   In retrospect, it is clear that the basic pathology of the acute form of Wernicke’s disease is lesions involving the limbic lobe, especially the mammillary bodies, and other areas of the brain as well, caused by deficiency of thiamine—and typically seen in severe alcoholics who are poorly nourished.

   As stated above, 6 years after Wernicke, in 1887 Korsakoff described in a Russian journal a syndrome involving memory loss and polyneuritis. Although Korsakoff did not know it at the time, what he was portraying represented the chronic version of Wernicke’s disease, in which a prominent symptom is also memory loss. Suspicion began to grow that these two memory-loss diseases were the same. In 1904, psychiatry professor Karl Bonhoeffer (1868–1948), then at Heidelberg, noted in the Allgemeine Zeitschrift für Psychiatrie how closely the clinical picture of Korsakoff’s syndrome resembled Wernicke’s disease, though he did not assert that they were the same disease.

   In 1947, Hugh Edward de Wardener (M.B. 1939), in the department of medicine in St. Thomas’s Hospital, and Bernard Lennox (M.B. 1936), a lecturer in morbid anatomy at the Postgraduate Medical School of the University of London, both of whom had been held prisoner-of-war in Singapore by the Japanese under what must have been appalling conditions, attributed Wernicke’s disease to thiamine deficiency (in an article in the Lancet on "Cerebral Beriberi (Wernicke’s Encephalopathy: Review of 52 Cases in a Singapore Prisoner-of-War Hospital"). Rather tongue-in-cheek the authors noted, "An opportunity for placing a large number of healthy adults simultaneously on a standardised deficient diet and observing the results over a period of years is one which the many workers on the vitamin-B complex must have coveted" (p. 11).

   It was Harvard neurologist Raymond Adams (1911–), together with his longtime collaborator Maurice Victor (1920–), later professor of neurology at Case Western University in Cleveland, who put the two diseases together. In 1961, in an article in the American Journal of Clinical Nutrition, they confirmed the role of thiamine deficiency in Wernicke’s disease—without citing the De Wardener–Lennox contribution—and noted that "Korsakoff’s psychosis and alcoholic dementia or pseudoparesis are the common psychic manifestations of Wernicke’s disease," also attributable, at least in part, "to a deficiency of thiamine" (p. 394). Then, in their book The Wernicke-Korsakoff Syndrome (1971), Victor and Adams, together with George H. Collins (1927–), demonstrated that Korsakoff’s psychosis (or dementia) and Wernicke’s disease were the same disease. In their textbook Principles of Neurology (1977), Adams and Victor said succinctly: "Stated in another way, Korsakoff’s psychosis is the psychic manifestation of Wernicke’s disease" (quote from second edition, 1981, p. 704).