Wernicke-Korsakoff syndrome in monozygotic twins: a biochemical peculiarity.

Abstract

A pair of monozygotic twins, one suffering from the Wernicke-Korsakoff syndrome, verified at autopsy, and the other healthy, was studied biochemically. The erythrocyte transketolase of each twin showed abnormalities, though these differed in the two individuals. In the healthy twin, the basal transketolase was low, but responded normally to thiamine pyrophosphate (TPP) added in vitro. In the twin with the Wernicke-Korsakoff syndrome the basal level of the enzyme and its response in vitro were normal, but a period of treatment with thiamine tetrahydrofurfuryldisulphide, led to loss of the in vitro response. It is suggested that, initially, an inborn error of metabolism may have been common to both twins.

• Authors: D Leigh, A McBurney, H McIlwain
• Journal: The British journal of psychiatry : the journal of mental science (Br J Psychiatry) Vol. 139 Pg. 156-9 (Aug 1981) ISSN: 0007-1250 [Print] England
• PMID: 7197998 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Fursultiamin
• Transketolase
• Thiamine Pyrophosphate

Topics

• Alcohol Amnestic Disorder (drug therapy, enzymology, genetics)
• Diseases in Twins
• Erythrocytes (enzymology)
• Female
• Fursultiamin (therapeutic use)
• Humans
• Male
• Middle Aged
• Pregnancy
• Thiamine Pyrophosphate (metabolism)
• Transketolase (blood)
• Twins, Monozygotic
• Wernicke Encephalopathy (drug therapy, enzymology, genetics)