Abstract
The Wernicke-Korsakoff syndrome (WKS) is the most frequently encountered manifestation of thiamine deficiency in Western society. It is commonly seen in alcoholic patients, but may also occur in patients with impaired nutrition from other causes, such as those with gastrointestinal disease or AIDS. The pathology is restricted to the central nervous system and is characterised by neuronal loss, gliosis and vascular damage in regions surrounding the third and fourth ventricles and the cerebral aqueduct. In addition to WKS, thiamine deficiency may also result in beriberi, a cardiac and peripheral nervous system disease, and it has been implicated in the pathogenesis of cerebellar degeneration and peripheral neuropathy. Thus thiamine deficiency results in significant nervous system pathology and vigilance should be maintained in the diagnosis and treatment of this readily preventable cause of disease.
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References
Adams, R.D. (1976)Nutritional cerebellar degeneration., ed., Elsevier, Amsterdam.
Beatty, W.W., Bailly, R.C. and Fisher, L. (1989). Korsakoff-like amnesic syndrome in a patient with anorexia and vomiting.Intern. J. Clin. Neuropsych. 11:55–65.
Becker, J.T., Furman, M.M.R., Panisset, M. and Smith, C. (1990). Characteristics of the memory loss of a patient with Wernicke-Korsakoff's syndrome without alcoholism.Neuropsychologica,28:171–179.
Butters, N. (1984). Alcoholics Korsakoff's syndrome: An update.Seminars in Neurology,4:229–247.
Butterworth, R.F. (1985). Pyruvate dehydrogenase deficiency disorders. In (D.W. McCandless, ed.),Cerebral Energy Metabolism and Metabolic Encephalopathy, Plenum Publishing Corp., pp. 121–141.
Butterworth, R.F., Gaudreau, C., Vincelette, J., Bourgault, A., Lamothe, F., and Nutini, A. (1991). Thiamine deficiency and Wernicke's encephalopathy in AIDS.Metab. Brain Dis.,6:207–212.
Cooper, J.R. and Pincus, J.H. (1979). The role of thiamine in nervous tissue.Neurochem. Res.,4:223–239.
D'Amour, M., Bruneau, J., and Butterworth, R.F. (1991). Abnormalities of peripheral nerve conduction in relation to thiamine status in alcoholic patients.Can. J. Neurol. Sci.,18:126–128.
D'Aprile, P., Gentile, M.A., and Carella, A. (1994). Enhanced MR in the acute phase of Wernicke encephalopathy.A. J. N. R.,15:591–593.
De Wardener, H.E., and Lennox, B. (1947). Cerebral beriberi.Lancet II:,3-2:
Denny-Brown, D. (1958). The neurological aspects of thiamine deficiency.Fed. Proc.,17:35–9.
DiMauro, S., Servidei, S., Zeviani, M., DiRocco, M., DeVivo, D.C., DiDonato, S.,et al. (1987). Cytochrome c oxidase deficiency in Leigh disease.Ann. Neurol. 22:498–506.
Ferrer, I., Fabreques, I., Pineda, M., Gracia, I., and Ribalta, T. (1984). A Golgi study of cerebellar atrophy in human chronic alcoholism.Neuropathol. Appl. Neurobiol.,10:245–253.
Fulham, M., Lawrence, C., and Harper, C. (1988). Diagnostic clues in an adult case of Leigh's disease.Med. J. Aust.,149:320–322.
Geyer, C.A., Sartor, K.J., Prensky, A.J., Abramson, C.L., Hodges, F.S., and Gado, M.H. (1988). Leigh disease (subacute nectrotizing encephalomyelopathy): CT and MRI in five cases.J. Computer Assisted Tomography,12:40–44.
Gilman, S., Adams, K., Koeppe, R.A., Berent, S., Kluin, K.J., Modell, J.G.,et al. (1990). Cerebellar and frontal hypometabolism in alcohol cerebellar degeneration studied with positron emission tomography.Ann. Neurol.,28:775–785.
Halliday, G.M., Cullen, K., and Harding, A. (1994). Neuropathological correlates of memory dysfunction in the Wernicke-Korsakoff syndrome.Alcohol Alcohol,Suppl 2:247–253.
Handler, C.E. and Perkin, G.D. (1982). Anorexia nervosa and Wernicke's encephalopathy: An underdiagnosed association.Lancet,2:771–772.
Harper, C. (1979). Wernicke's encephalopathy: a more common disease than realised.J. Neurol. Neurosurg. Psychiatry,42:226–231.
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.
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.
Harper, C.G., and Butterworth, R.F. (1996). Metabolic Diseases. In (D.I. Graham and P. Lantos, eds.),Greenfield's Neuropathology, Edward Arnold, London, (in press).
Koch, T.K., Yee, M.H.C., Hutchinson, H.T., and Berg, B.O. (1986). Magnetic resonance imaging in subacute necrotizing encephalomyelopathy (Leigh disease).Ann. Neurol.,19:605–607.
Langlais, P.J. (1992). Role of diencephalic lesions and thiamine deficiency in Korsakoff's amnesia: Insights from animal models. In (L.R. Squire and N. Butters, eds.),Neuropsychology of Memory,. The Guilford Press, New York, pp. 440–450.
Lindboe, C.F., and Loberg, E.M. (1989). Wernicke's encephalopathy in non-alcoholics. An autopsy study.J. Neurol. Sci.,90:125–129.
Malamud, N., and Skillikorn, S.A. (1956). Relationship between the Wernicke and the Korsakoff syndrome.Arch. Neurol. Psychiat.,76:585–596.
Mancall, E.L., and McEntee, W.J. (1965). Alterations of the cerebellar cortex in nutritional encephalopathy.Neurology,15:303–313.
Maso, E., Ferrer, I., Roquer, J., and Serrano, S. (1984). Leigh's disease in an adult.J. Neurol.,231:253–257.
McLane, J.A., Khna, T., and Held, I.R. (1987). Increased axonal transport in peripheral nerves of thiamine-deficient rats.Exp. Neurol.,95:482–491.
Mishkin, M. (1982). A memory system in the monkey.Philosophical Transactions of the Royal Society of London,B298:85–95.
Ohnishi, A., Tsuji, S., Tgisu, H., Murai, Y., Goto, I., Kuroiwa, Y.et al. (1980). Beriberi neuropathy. Morphological study of sural nerve.J. Neurol. Sci.,45:177–190.
Phillips, S.C., Harper, C.G., and Kril, J.J. (1987). A quantitative histological study of the cerebellar vermis in alcoholic patients.Brain,110:301–314.
Phillips, S.C., Harper, C.G., and Kril, J.J. (1990). The contribution of Wernicke's encephalopathy to alcohol-related cerebellar damage.Aust. Drug Alcohol Rev.,9:53–60.
Pincus, J.H., Itokawa, Y., and Cooper, J.R. (1969). Enzyme-inhibiting factor in subacute necrotizing encephalomyelopathy.Neurology,19:841–845.
Pincus, J.H., Solitare, G.B., and Cooper, J.R. (1976). Thiamine triphosphate levels and histopathology. Correlation with Leigh disease.Arch. Neurol.,33:759–763.
Sasa, M., Takemoto, I., Nishino, K., and Itokawa, Y. (1976). The role of thiamine on excitable membrane of crayfish giant axon.J. Nutri. Science Vitaminol.,22:21–24.
Shogry, M.E., and Curnes, J.T. (1994). Mamillary body enhancement as the only sign of acute Wernicke encephalopathy.A. J. N. R.,15:172–174.
Thomas, P.K., London, D.N., and King, R.H.M. (1984). Diseases of the peripheral nerves. In (J.H. Adams, J.A.N. Corsellis and, L.W. Duchen, eds.),Greenfield's Neuropathology, Edward Arnold, London, pp. 806–920.
Torvik, A. (1985). Two types of brain lesions in Wernicke's encephalopathy.Neuropathol. Appl. Neurobiol.,11:179–190.
Victor, M., Adams, R.D., and Collins, G.H. (1989).The Wernicke-Korsakoff Syndrome and related Neurological Disorders of Alcoholism and Malnutrition, 2nd ed., FA Davis Co., Philadelphia.
Walsh, J.C., and McLeod, J.G. (1970). Alcoholic neuropathy. An electrophysiological and histological study.J. Neurol. Sci.,10:459–469.
Willems, J.L., Monnens, L.A.H., Trijbels, J.M.F., Veerkamp, J.H., Meyer, A.E.F.H., van Dam, K., and van Haelst, U. (1977). Leigh's encephalopathy in a patient with cytochrome c oxidase deficiency in muscle tissue.Pediatrics,60:850–857.
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Kril, J.J. Neuropathology of thiamine deficiency disorders. Metab Brain Dis 11, 9–17 (1996). https://doi.org/10.1007/BF02080928
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DOI: https://doi.org/10.1007/BF02080928