Korsakoff's syndrome

Definition

Korsakoff's syndrome is a memory disorder which is caused by a deficiency of vitamin B₁, also called thiamine.

Description

In the United States, the most common cause of thiamine deficiency is alcoholism. Other conditions which 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.

Causes

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.

Symptoms

An individual with Korsakoff's syndrome displays much 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 is unable to remember what occurred 20 minutes ago. This memory defect is referred to as anterograde amnesia, and leads to a peculiar symptom called "confabulation," in which a person suffering from Korsakoff's fills in the gaps in his or her memory with fabricated or imagined information. For instance, a person may insist that a doctor to whom he or 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 ten minutes earlier, a person with Korsakoff's may list three entirely different objects and be completely convincing in his or her certainty. In fact, one of the hallmarks of Korsakoff's is the person's complete unawareness of the memory defect, and complete lack of worry or concern when it is pointed out.

Diagnosis

Whenever someone has a possible diagnosis of alcoholism, and then has the sudden onset of memory difficulties, it is important to seriously consider the diagnosis of Korsakoff's syndrome. While there is no specific laboratory test to diagnose Korsakoff's syndrome in a patient, a careful exam of the individual's mental state should be rather revealing. Although the patient's ability to confabulate answers may be convincing, checking the patient's retention of factual information (asking, for example, for the name of the current president of the United States), along with the patient's ability to learn new information (repeating a series of numbers, or recalling the names of three objects ten minutes after having been asked to memorize them) should point to the diagnosis. Certainly a patient known to have just begun recovery from Wernicke's syndrome, who then begins displaying memory difficulties, would be very likely to have developed Korsakoff's syndrome. A physical examination may also show signs of Wernicke's syndrome, such as peripheral neuropathy.

Treatment

Treatment of both Korsakoff's and Wernicke's 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, should receive thiamine. The combined Wernicke-Korsakoff syndrome has actually been precipitated in alcoholic patients hospitalized for other medical illnesses, due to 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). Also, the vitamin therapy may be impaired by the feeding of carbohydrates prior to the giving of thiamine; since carbohydrates cannot be metabolized with thiamine.

Prognosis

Fifteen to twenty percent of all patients hospitalized for Wernicke's syndrome will die of the disorder. Although the degree of ataxia 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 thiamine treatment. Recovery from Wernicke's begins to occur rapidly after thiamine is given. Improvement in the symptoms of Korsakoff's syndrome, however, can take months and months of thiamine replacement. Furthermore, patients who develop Korsakoff's 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 Korsakoff's syndrome often results in an individual requiring a supervised living situation.

Prevention

Prevention depends on either maintaining a diet with a sufficient intake of thiamine, or supplementing an inadequate diet with vitamin preparations. Certainly, one of the most important forms of prevention involves treating the underlying alcohol addiction.

Key Terms

Amnesia

Inability to remember events or experiences. Memory loss. Includes: 1) Anterograde amnesia: inability to retain the memory of events occurring after the time of the injury or disease which brought about the amnesic state. 2) 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.

Confabulation

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

Diencephalon

A part of the brain that binds the mesencephalon to the cerebral hemispheres. Considered by some as part of the brain stem.

Further Reading

For Your Information

Books

• Messing, Robert. "Nutritional Disorders of the Nervous System." In Cecil Textbook of Medicine, edited by J. Claude Bennett and Fred Plum. Philadelphia: W.B. Saunders, 1996.
• Rossor, Martin. "Disorders of Higher Cerebral Cortical Function and Behavioral Neurology." In Brain's Diseases of the Nervous System, edited by John Walton. New York: Oxford Medical Publications, 1993.
• Schuckit, Marc Alan. Educating Yourself About Drugs and Alcohol. New York: Plenum Press, 1995.

  Periodicals

• Langlais, Philip J. "Alcohol-Related Thiamine Deficiency." Alcohol Health and Research World (Spring 1995): p. 113.

  Organizations

• National Institute on Alcoholism Abuse and Alcoholism. 6000 Executive Boulevard - Willco Building, Bethesda, Maryland 20892-7003. http://www.niaaa.nih.gov.

Gale Encyclopedia of Medicine. Gale Research, 1999.