Korsakoff's syndrome is a memory disorder which is caused by a deficiency of vitamin B1, also called thiamine.
In the United States, the most common cause of thiamine deficiency is alcoholism. Other conditions that cause thiamine deficiency occur quite rarely, but can be seen in patients undergoing dialysis (a procedure used primarily for patients suffering from kidney failure, during which the patient's blood circulates outside of the body, is mechanically cleansed, and then is circulated back into the body), pregnant women with a condition called hyperemesis gravidarum (a condition of extreme morning sickness, during which the woman vomits up nearly all fluid and food intake), and patients after surgery who are given vitamin-free fluids for a prolonged period of time. Thiamine deficiency is an important cause of disability in developing countries where the main source of food is polished rice (rice with the more nutritious outer husk removed).
An associated disorder, Wernicke's syndrome, often precedes Korsakoff's syndrome. In fact, they so often occur together that the spectrum of symptoms produced during the course of the two diseases is frequently referred to as Wernicke-Korsakoff syndrome. The main symptoms of Wernicke's syndrome include ataxia (difficulty in walking and maintaining balance), paralysis of some of the muscles responsible for movement of the eyes, and confusion. Untreated Wernicke's will lead to coma and then death.
One of the main reasons that alcoholism leads to thiamine deficiency has to do with the high-calorie nature of alcohol. A person with a large alcohol intake often, in essence, substitutes alcohol for other, more nutritive calorie sources. Food intake drops off considerably, and multiple vitamin deficiencies develop. Furthermore, it is believed that alcohol increases the body's requirements for B vitamins, at the same time interfering with the absorption of thiamine from the intestine and impairing the body's ability to store and use thiamine. Direct neurotoxic (poisonous damage to the nerves) effects of alcohol may also play some role.
Thiamine is involved in a variety of reactions which provide energy to the neurons (nerve cells) of the brain. When thiamine is unavailable, these reactions cannot be carried out, and the important end-products of the reactions are not produced. Furthermore, certain other substances begin to accumulate, and are thought to cause damage to the vulnerable neurons. The area of the brain believed to be responsible for the symptoms of Korsakoff's syndrome is called the diencephalon, specifically the structures called the mamillary bodies and the thalamus.
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Author Info: Rosalyn Carson-DeWitt MD, The Gale Group Inc., Gale, Detroit, Gale Encyclopedia of Medicine, 2002 |