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Wernicke-Korsakoff syndrome is a combination of Korsakoff's syndrome, which is characterized by confusion, severe anterograde and retrograde amnesia and confabulation; and Wernicke's encephalopathy, which is characterized by nystagmus, ophthalmoplegia, coma and, if untreated, death.
Wernicke-Korsakoff syndrome results from severe acute deficiency of thiamine superimposed on a chronic deficiency. Thiamine is a cofactor in glucose metabolism, and so anything that encourages glucose metabolism without replenishing stores of thiamine may result in Wernicke-Korsakoff syndrome, which is usually found in malnourished chronic alcoholics, though it is also found in patients who undergo prolonged intravenous (IV) therapy, gastric stapling or intensive care unit (ICU) stays. The site of damage in the brain is the mammillary bodies.
Some studies have associated susceptiblity to this syndrome with a hereditary deficiency of transketolase, an enzyme involved in thiamine metabolism.
Treatment consists of reversing the thiamine deficiency by giving supplemental thiamine, usually by starting with an initial intravenous or intramuscular dose followed by supplemental oral doses. It is important to start the thiamine before giving any glucose as the encephalopathy will be worsened by the glucose. By the time amnesia and psychosis have occurred, complete recovery is unlikely.
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