Korsakoff's syndrome
From Psy3241
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Definition
• Korsakoff's syndrome is a memory disorder caused by a deficiency of vitamin B1, also called thiamine
• Named after Sergei Korsakoff the neuropsychiatrist who popularized the theory
History
• First mentioned in 1822 by James Jackson, a physician at Massachusetts General Hospital and professor at Boston Medical School, in a review of the peripheral neuritis of alcoholism
• 1868 Sir Samuel Wilks, a physician at Guy’s Hospital, London, gave an account of the characteristic mental symptoms in an article on alcoholic paraplegia
• In his doctoral thesis, Korsakoff called the condition “cerebropathia psychica toxaemica”
• The term Korsakoff’s psychosis was introduced by Friedrich Jolly
• Korsakoff emphasized the association of alcoholic polyneuropathy with a specific pattern of mental disturbances by stating: “This mental disorder appears at times in the form of sharply delineated irritable weakness of the mental sphere, at times in the form of confusion with characteristic mistakes in orientation for place, time and situation, and at times as an almost pure form of acute amnesia, where the recent memory is most severely involved, while the remote memory is well preserved . . . Some have suffered so widespread memory loss that they literally forget everything immediately."
• Korsakoff’s psychosis was used originally to describe the combination of alcoholic polyneuropathy and characteristic mental disturbance and Korsakoff’s syndrome was the non-alcoholic form with or without polyneuritis, which resulted from acquired conditions such as head injury, brain tumors, and encephalitis
• Blass and Gibson in 1977 identified a genetically determined disturbance of transketolase, which is a thiamine pyrophosphate-binding factor in fibroblasts
• Individuals who are homozygous for this defect are at an increased risk for developing thiamine deficiency in circumstances of dietary inadequacy
Description
• The most common cause of thiamine deficiency is alcoholism in the U.S.
• Thiamine deficiency can also be seen in patients undergoing dialysis, pregnant women with the condition called hyperemesis gravidarum, and patients after surgery who are given vitamin-free fluids for a prolonged period of time
• Thiamine deficiency is a cause of disability in developing countries where the main source of food is rice lacking the nutritious outer husk
• Wenicke’s syndrome often precedes Korsakoff’s syndrome
• Commonly referred to as Wenicke-Korsakoff’s syndrome
