Wernicke-Korsakoff syndrome

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Wernicke-Korsakoff syndrome
Classifications and external resources
ICD-10 E51.2, F10.6
ICD-9 294.0

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. The combined syndrome is named after the two doctors, Carl Wernicke and Sergei Korsakoff, who popularized the respective illnesses.

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[edit] Causes

Wernicke-Korsakoff syndrome results from severe acute deficiency of thiamine (Vitamin B1) 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 neurological damage caused by Wernicke-Korsakoff syndrome in alcoholics has been found in several areas of the brain, namely the anterior region of the thalamus (accounting for amnesic symptoms), the medial dorsal thalamus, the basal forebrain, mamillary bodies, and median and dorsal raphe nuclei (Mann et al., 2001).

Some studies have associated susceptiblity to this syndrome with a hereditary deficiency of transketolase, an enzyme involved in thiamine metabolism.

[edit] Diagnosis

Diagnosis of Wernicke-Korsakoff syndrome is by clinical impression, but it can be confirmed by measuring blood levels of thiamine.

[edit] Treatment

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 treatment 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.

[edit] References

Mann, K., Agartz, I., Harper, C., Shoaf, S. et al., 2001. Neuroimaging in Alcoholism: Ethanol and Brain Damage. Alcoholism: Clinical and Experimental Research, 25(5), pp. 104-109.

[edit] External link