Metabolic Brain Disease

, Volume 10, Issue 1, pp 17–24 | Cite as

An international perspective on the prevalence of the Wernicke-Korsakoff syndrome

  • Clive Harper
  • Paul Fornes
  • Charles Duyckaerts
  • Dominique Lecomte
  • Jean-Jacques Hauw


In the Western world previous studies have shown that the majority of cases of the Wernicke-Korsakoff syndrome (WKS), which is caused by thiamine deficiency, occur in alcoholics. However, in France, a country with one of the highest per capita consumptions of alcohol, the prevalence of the WKS was found to be only 0.4% in a small retrospective autopsy study. This figure is compared with data sent to the authors by a number of neuropathologists from the U.S.A., Europe, Scandinavia and Australia. There was no obvious correlation between the prevalence rates of the WKS, which were highest in Australia (2.8% - previously published), and per capita consumption of alcohol. Other issues such as diet, National programs for supplementation of foods with thiamine, and drinking habits are considered. The pathological diagnosis of the WKS can often be made on macroscopic examination of the brain after fixation in formalin. The mammillary bodies are smaller than normal in most cases of chronic WKS. However in this study it was found that the most common causes of small mammillary bodies were Alzheimer's disease and atrophy due to transneuronal degeneration secondary to lesions in the hippocampus.

Key words

Thiamine deficiency Wernicke-Korsakoff syndrome prevalence international perspective pathology 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Axford, D. W. E. and Williams, D. A. (1981). Flour enrichment around the world.British Flour Milling and Baking Research Association 4:156–163.Google Scholar
  2. Butterworth, R. F., Gaudreau, C., Vincelette, J., Bourgault, A.-M., Lamothe, F. and Nutini, A.-M. (1991). Thiamine deficiency and Wernicke's encephalopathy in AIDS.Metab. Brain Dis. 6:207–212.CrossRefPubMedGoogle Scholar
  3. Dreneck, E. J., Joven, C. B. and Swendseid, M. E. (1966). Occurrence of acute Wernicke's encepathalopathy during prolonged starvation for the treatment of obesity.N. Eng J. Med. 274:937–939.Google Scholar
  4. Edwards, G. (1979). Drinking problems. Putting the Third World on the map.Lancet 8:402–404.CrossRefGoogle Scholar
  5. Harper, C. G. (1980). Sudden, unexpected death and Wernicke's encephalopathy. A complication of prolonged intravenous feeding.Aust. N. Z. J. Med. 10:230–235.PubMedGoogle Scholar
  6. Harper, C. G. (1983). The incidence of Wernicke's encephalopathy in Australia - a neuropathological study of 131 cases.J. Neurol. Neurosurg. Psychiatry 46: 593–598.PubMedGoogle Scholar
  7. Harper, C. G., Gold, J., Rodrigeuz, M. and Perdices, M. (1989). The prevalence of the Wernicke-Korsakoff syndrome in Sydney, Australia: a prospective necropsy study.J Neurol Neurosurg Psychiatry 52:282–285.PubMedGoogle Scholar
  8. Harper, C. G. and Kril, J. J. (1990). The changing face of the Wernicke-Korsakoff syndrome.Aust. Drug Alcohol Rev. 9:299–301.Google Scholar
  9. Haut comité de la Santé publique. (1992). Les indicateurs d'alcoolisation 1992. La consommation, la mortalité. La Documentation française, Paris.Google Scholar
  10. Hauw, J.-J., De Baecque, C., Hausser-Hauw, C. and Serdaru, M. (1988). Chromatolysis in alcoholic encephalopathies.Brain 111:843–857.PubMedGoogle Scholar
  11. Jellinger, K. (1976). Neuropathological aspects of dementia resulting from abnormal blood and cerebral fluid dynamics.Acta Neurol. Belg. 76:83–102.PubMedGoogle Scholar
  12. Lindboe, C. F., Erichsen, A. A. and Strøm, E. H. (1989). Atrophy and sponginess of the mammillary bodies with neuronal sparing: Not only inactive Wernicke's encephalopathy.APMIS 97:667–670.PubMedGoogle Scholar
  13. Lindboe, C. F. and Loberg, E. M. (1989). Wernicke's encephalopathy in non-alcoholics. An autopsy study.J. Neurol. Sci. 90:125–129.CrossRefPubMedGoogle Scholar
  14. Naidoo, P. N., Bramdev, A. and Cooper, K. (1991). Wernicke's encephalopathy and alcohol-related disease.Postgrad. Med. J. 67:978–981.PubMedGoogle Scholar
  15. Schubert, J., and Friede, R. L. (1979). Transneuronal mammillary atrophy.J. Neurol. 221:67–72.CrossRefPubMedGoogle Scholar
  16. Thomson, A. D., Jeyasingham, M. D., Pratt, O. E. and Shaw, G. K. (1987). Nutrition and alcoholic encephalopathies.Acta Med. Scand. Suppl 717:55–65.Google Scholar
  17. Verhoek, J. J. M. (1992).World Drink Trends., Produktschap voor Gedistilleerde Dranken in association with NTC Publications Ltd,Google Scholar
  18. Victor, M., Adams, R. D. and Collins, G. H. (1989).The Wernicke-Korsakoff Syndrome, 2nd ed., Davis, Philadelphia.Google Scholar
  19. Wood, B. and Breen, K. J. (1980). Clinical thiamine deficiency in Australia: the size of the problem and approaches to prevention.Med. J. Aust. 1:461–464.PubMedGoogle Scholar
  20. Yellowlees, P. M. (1986). Thiamin deficiency and prevention of the Wernicke-Korsakoff syndrome. A major public health problem.Med. J. Aust. 745:216–219.Google Scholar

Copyright information

© Plenum Publishing Corporation 1995

Authors and Affiliations

  • Clive Harper
    • 1
  • Paul Fornes
    • 2
  • Charles Duyckaerts
    • 3
  • Dominique Lecomte
    • 2
  • Jean-Jacques Hauw
    • 3
  1. 1.The Neuropathology Unit, Department of PathologyUniversity of SydneySydneyAustralia
  2. 2.The Institute of Forensic MedicineParisFrance
  3. 3.Laboratoire de Neuropathologie R. EscourolleHôpital de la SalpêtrièreParisFrance

Personalised recommendations