Wernicke-Korsakoff syndrome is the best known neurologic complication of thiamine (vitamin B1) deficiency . The term refers to two different syndromes, each representing a different stage of the disease. Wernicke encephalopathy (WE) is an acute syndrome requiring emergent treatment to prevent death and neurologic morbidity. Korsakoff syndrome (KS) refers to a chronic neurologic condition that usually occurs as a consequence of WE.
In 1881, Carl Wernicke described an acute encephalopathy characterized by mental confusion, ophthalmoplegia, and gait ataxia and associated it with autopsy findings of punctate hemorrhages around the third and fourth ventricles and the aqueduct. A few years later, Russian psychiatrist Sergei Korsakoff described a chronic amnestic syndrome in which memory was impaired far out of proportion to other cognitive domains. While both observations were described in the context of chronic alcoholism, neither Wernicke nor Korsakoff initially recognized the relationship between the disorders, which was not appreciated until later by other investigators.
This topic will review Wernicke encephalopathy. Korsakoff syndrome and other chronic neurologic complications of alcohol abuse, including alcohol withdrawal are discussed separately. (See "Overview of the chronic neurologic complications of alcohol" and "Management of moderate and severe alcohol withdrawal syndromes".)
Typical brain lesions of Wernicke encephalopathy (WE) are observed at autopsy in 0.4 to 2.8 percent of the general population in the Western world, and the majority of affected patients are alcoholic [2,3]. The prevalence of WE lesions seen on autopsy was 12.5 percent of alcohol abusers in one report . Among those with alcohol-related deaths, it has been reported to be even higher, 29 to 59 percent [5,6]. Autopsy studies have consistently revealed a higher incidence of Wernicke lesions in the general population than is predicted by clinical studies, suggesting that it is under-recognized clinically [1,7].
While cases of WE in men outnumber those in women, women appear to be more susceptible to developing WE than men. In several series, the female to male ratio for WE was higher than the ratio for alcohol dependence [1,7].