Korsakoff’s Syndrome

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Korsakoffs Syndrome

Symptoms of Korsakoffs syndrome

Why alcoholism can lead to Korsakoffs

Diagnosis

Treatment

Korsakoffs syndrome is a memory disorder caused by a vitamin B1 (thiamine) deficiency. In the United States, the most common cause of such a deficiency is alcoholism. Other conditions that cause thiamine deficiency are rare, but can be seen in patients undergoing dialysis (a procedure during which the individuals blood circulates outside of the body, ismechanically cleansed, and then 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. In developing countries, people whose main source of food is polished rice (rice with the more nutritious outer husk removed) may suffer from beriberi, another form of thiamine deficiency.

An associated disorder, Wernickes syndrome, often precedes Korsakoffs 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 WernickeKorsakoff syndrome. The main symptoms of Wernickes syndrome include ataxia (difficulty in walking and maintaining balance), paralysis of some of the muscles responsible for movement of the eyes, and confusion. Untreated Wernickes Syndrome will lead to coma and then death.

Symptoms of Korsakoffs syndrome

An individual with Korsakoffs syndrome displays difficulty with memory. The main area of memory affected is the ability to learn new information. Usually, intelligence and memory for past events is relatively unaffected, so that an individual may remember what occurred 20 years previously, but be unable to remember what occurred 20 minutes previously. This memory defect is referred to as anterog-rade amnesia, and leads to a peculiar symptom called confabulation, in which an individual suffering from Korsakoffs fills in the gaps in his/her memory with fabricated or imagined information. An individual may insist that a doctor to whom he/she has just been introduced is actually an old high school classmate, and may have a lengthy story to back this up. When asked, as part of a memory test, to remember the name of three objects which the examiner listed 10 minutes earlier, an individual with Korsakoffs may list three entirely different objects and be completely convincing in his/her certainty. In fact, one of the hallmarks of Korsakoffs is the individuals complete unawareness of his/her memory defect, and complete lack of worry or concern when it is pointed out.

Why alcoholism can lead to Korsakoffs

One of the main reasons that alcoholism leads to thiamine deficiency occurs because of 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 bodys requirements for B vitamins, at the same time it interferes with thiamine absorption from the intestine, and impairs the bodys ability to store and use thiamine.

Thiamine is involved in a variety of reactions that 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 damage vulnerable neurons. The area of the brain believed to be responsible for the symptoms of Korsakoffs syndrome is called the diencephalon, specifically, structures called the mammillary bodies and the thalamus.

Diagnosis

Whenever an individual has a possible diagnosis of alcoholism, and then has the sudden onset of memory difficulties, it is important to seriously consider the diagnosis of Korsakoffs syndrome. There is no specific laboratory test to diagnose Korsakoffs syndrome in a patient, but a careful exam of the individuals, mental state can be revealing. Although the patients ability to confabulate answers may be convincing, checking the patients retention of factual information (asking, for example, for the name of the current president of the United States), along with his/her ability to learn new information (repeating a series of numbers, or recalling the names of three objects 10 minutes after having been asked to memorize them) should point to the diagnosis. Certainly a patient known to have just begun recovery from Wernickes syndrome, who then begins displaying memory difficulties, would be very likely to have developed Korsakoffs syndrome.

Treatment

Treatment of both Korsakoffs and Wernickes syndromes involves the immediate administration of thiamine. In fact, any individual who is hospitalized for any reason and who is suspected of being an alcoholic, must receive thiamine. The combined WernickeKorsakoff syndrome has actually been precipitated in alcoholic patients hospitalized for other medical illnesses, by the administration of thiamine-free intravenous fluids (intravenous fluids are those fluids containing vital sugars and salts which are given to the patient through a needle inserted in a vein).

Fifteen to twenty percent of all patients hospitalized for Wernickes syndrome will die. Although the

KEY TERMS

Amnesia Inability to remember events or experiences. Memory loss.

Anterograde amnesia Inability to retain the memory of events occurring after the time of the injury or disease which brought about the amnesic state.

Confabulation An attempt to fill in memory gaps by fabricating information or details.

Retrograde amnesia Inability to recall the memory of events which occurred prior to the time of the injury or disease which brought about the amnesic state.

degree of a taxia nearly always improves with treatment, half of those who survive will continue to have some permanent difficulty walking. The paralysis of the eye muscles almost always resolves completely with thi-amine treatment. Recovery from Wernickes begins to occur rapidly after thiamine is given. Improvement in the symptoms of Korsakoffs syndrome, however, can take many months of thiamine replacement. Furthermore, patients who develop Korsakoffs syndrome are almost universally memory-impaired for the rest of their lives. Even with thiamine treatment, the memory deficits tend to be irreversible, with less than 20% of patients even approaching recovery. The development of Korsakoffs syndrome often results in an individual requiring a supervised living situation.

Resources

BOOKS

Andreoli, Thomas E., et al. Cecil Essentials of Medicine. Philadelphia: W.B. Saunders Company, 1993.

Berkow, Robert, and Andrew J. Fletcher. The Merck Manual of Diagnosis and Therapy. Rahway, NJ: Merck Research Laboratories, 1992.

Isselbacher, Kurt J., et al. Harrisons Principles of Internal Medicine.New York: McGraw Hill, 1994.

OTHER

Medical Council on Alcohol. The WernickeKorsakoff Syndrome Can Be Treated <http://www.medicouncilalcol.demon.co.uk/articles.htm#wern> (accessed December 2, 2006).

National Institutes of Health, National Institute of Neurological Disorders and Stroke. NINDS WernickeKorsakoff Syndrom Information Page <http://www.ninds.nih.gov/disorders/wernicke_korsakoff/wernicke-korsakoff.htm> (accessed December 2, 2006).

Rosalyn Carson-DeWitt