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Wernicke-Korsakoff Syndrome in Monozygotic Twins: A Biochemical Peculiarity

Published online by Cambridge University Press:  29 January 2018

Denis Leigh ,
Alan Mcburney  and
Henry Mcllwain
Denis Leigh
Affiliation:
The Maudsley Hospital, London SE5 8AZ
Alan Mcburney
Affiliation:
Department of Clinical Pharmacology, The Groby Road Hospital, Leicester LE3 9AE
Henry Mcllwain
Affiliation:
Department of Biochemistry, St Thomas's Hospital Medical School, London SE1 7EH
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Summary

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.

Type
Papers
Information
The British Journal of Psychiatry , Volume 139 , Issue 2 , August 1981 , pp. 156 - 159
Copyright
Copyright © Royal College of Psychiatrists, 1981 

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