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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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References

Blass, J. P. & Gibson, G. E. (1977) Abnormality of a thiamine requiring enzyme in patients with Wernicke-Korsakoff Syndrome. New England Journal of Medicine, 297, 1367–70.CrossRefGoogle ScholarPubMed
Bowman, K. M., Goodhart, R. & Jolliffe, N. (1939) Observations on the role of vitamin B1 in the etiology and treatment of Korsakoff psychosis. Journal of Nervous and Mental Disease, 90, 569–75.Google Scholar
Brin, M. (1962) Erythrocyte transketolase in early thiamine deficiency. Annals of the New York Academy of Science, 98, 528–41.CrossRefGoogle ScholarPubMed
Brin, M., Vincent, W. & Watson, J. (1962) Human thiamine deficiency and erythrocyte transketolase (abstract). Federation Proceedings, 21, 468.Google Scholar
Cole, M., Turner, A., Frank, O., et al (1969) Extraocular palsy and thiamine therapy in Wernicke's encephalopathy. American Journal of Clinical Nutrition, 22, 44—51.Google Scholar
Cooper, J. R., Itokawa, Y. & Pincus, W. H. (1969) Thiamine triphosphate deficiency in subacute necrotising encephalomyelopathy. Science, 164, 74–5.CrossRefGoogle Scholar
Dreyfus, P. M. (1962) Clinical applications of blood transketolase determination. New England Journal of Medicine, 267, 567–98.Google Scholar
Eichenbaum, J. W. & Cooper, J. R. (1971) Restoration thiamine of neuromuscular transmission. Brain Research, 32, 258–60.Google Scholar
Garrod, A. (1931) Inborn Factors in Disease. New York: Oxford University Press.Google Scholar
Itokawa, Y. & Cooper, J. R. (1970) Ion movements and thiamine in nervous tissue. Intact nerve preparations. Biochemical Pharmacology, 19, 985–92.CrossRefGoogle Scholar
Jolliffe, N., Wortis, H. & Fein, H. D. (1941) The Wernicke Syndrome. Archives of Neurology and Psychiatry, 46, 569–97.CrossRefGoogle Scholar
McBurney, A., Leigh, D. & McIlwain, H. (1980) Erythrocyte transketolase activity in suspected cases of Leigh's Disease, or subacute necrotising encephalomyelopathy. Archives of Disease in Childhood, 55, 789–94.CrossRefGoogle ScholarPubMed
Shields, J. (1977) Genetics and alcoholism. In Alcoholism. New Knowledge and New Responses, (eds.) Edwards, G. and Grant, M. London: Croom Helm.Google Scholar
Slater, E. (1953) Psychotic and Neurotic Illnesses in Twins. Medical Research Council Special Report Series No. 278. London: HMSO.Google Scholar
Victor, M., Adams, R. D. & Collins, G. H. (1971) The Wernicke—Korsakoff Syndrome. Contemporary Neurology Series. Vol. 7. Oxford: Blackwell Scientific Publications.Google Scholar
Waldenlind, L. (1978) Studies on thiamine and neuromuscular transmission. Acta Physiologica Scandinavica (Supp) 459.Google Scholar
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