Summary
Experimental Wernicke's encephalopathy, induced in rhesus monkeys with a diet lacking thiamine (vitamin B1), is characterized by cavitary necrosis of the striatum as well as a microvacuolar periventricular lesion of the brain stem such as occurs in man. With high resolution light microscopy and electron microscopy, the primary structural alteration in the brain stem lesion, and probably also in the striatum, appears to be that of widespread “blister” formation due to splitting of myelin at the intraperiod line. Microvascular alterations were minimal, even in the most severely affected regions. It is the myelin blisters which give rise to the spongy texture of the neuropil. A similar splitting of myelin has been deseribed in several other experimental encephalopathies, and it is probable that it also occurs in Wernicke's encephalopathy in man.
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Adachi, M., Wallace, B., Schneck, L., Volk, B. W.: Fine structure of spongy degeneration of the central nervous system (Van Bogaert and Bertrand type). J. Neuropath. exp. Neurol.25, 598–610 (1966)
Adornato, B. T., O'Brien, J. S., Lampert, P. W., Roe, T. F., Neustein, H. B.: Cerebral spongy degeneration of infancy: A biochemical and ultrastructural study of affected twins. Neurology (Minneap.)22, 202–210 (1972)
Aleu, F. P., Katzman, R., Terry, R. D.: Fine structure and electrolyte analysis of cerebral edema induced by alkytin intoxication. J. Neuropath. exp. Neurol.22, 403–413 (1963)
Aleu, F., Samuels, S., Ransohoff, J.: The pathology of cerebral edema associated with gliomas in man. Amer. J. Path.48, 1043–1061 (1966)
Alexander, L.: Wernicke's disease: Identity of lesions produced experimentally by B1 avitaminosis in pigeons with hemorrhagic polio-encephalitis occurring in chronic alcoholism in man. Amer. J. Path.16, 61–69 (1940)
Aparicio, S., Lumsden, C. E., Jennings, M.: Electron microscopy of glial reactions observed in CNS cultures. Brain Res.7, 268–285 (1968)
Blakemore, W. F.: Observations on oligodendrocyte degeneration, the resolution of status spongiosjs and remyelination in cuprizone intoxication in mice. J. Neurocytology1, 413–426 (1972)
Blakemore, W. F., Palmer, A. C., Noel, P. R. B.: Ultrastructural changes in isoniazid-induced brain oedema in the dog. J. Neurocytology1, 263–278 (1972)
Brightman, M. W., Reese, T. S.: Junetions between intimately opposed cell membranes in the vertebrate brain. J. Cell. Biol.40, 648–677 (1969)
Caley, D. W., Maxwell, D. S.: Development of the blood vessels and extracellular spaces during postnatal maturation of rat cerebral cortex. J. comp. Neurol.138, 31–48 (1970)
Collins, G. H.: Glial cell changes in the brain stem of thiamine-deficient rats. Amer. J. Path.50, 791–814 (1967)
Collins, G. H., Converse, W. K.: Cerebellar degeneration in thiamine-deficient rats. Amer. J. Path.58, 219–233 (1970)
Collins, R. C., Kirkpatrick, J. B., McDougal, D. B.: Some regional pathologic and metabolic consequences in mouse brain of pyrithiamine-induced thiamine deficiency. J. Neuropath. exp. Neurol.29, 57–69 (1970)
Collins, G. H., Webster, H. DeF., Victor, M.: The ultrastructure of myelin and axonal alterations in sciatic nerves of thiamine deficient and chronically starved rats. Acta neuropath. (Berl.)3, 511–521 (1964)
Drenick, E. J., Joven, C. B., Swendseid, M. E.: Occurrence of acute Wernicke's encephalopathy during prolonged starvation for the treatment of obesity. New Engl. J. Med.274, 937–939 (1966)
Dreyfus, P. M.: The quantitative histochemical distribution of thiamine in normal rat brain. J. Neurochem.4, 183–190 (1959)
Dreyfus, P. M.: The quantitative histochemical distribution of thiamine in deficient rat brain. J. Neurochem.8, 139–145 (1961)
Dreyfus, P. M.: The regional distribution of transketolase in the normal and the thiamine deficient nervous system. J. Neuropath. exp. Neurol.24, 119–129 (1965)
Dreyfus, P. M., Hauser, G.: The effect of thiamine deficiency on the pyruvate decarboxylase system of the central nervous system. Biochim. biophys. Acta (Amst.)104, 78–84 (1965)
Dreyfus, P. M., Victor, M.: Effects of thiamine deficiency on the central nervous system. Amer. J. clin. Nutr.9, 414–425 (1961)
Farquhar, M. D., Hartmann, J. F.: Neuroglial structure and relationships as revealed by electron microscopy. J. Neuropath. exp. Neurol.16, 18–39 (1957)
Gambetti, P., Mellmann, W. J., Gonates, N. K.: Familial spongy degeneration of the central nervous system (Van Bogaert-Betrand disease). An ultrastructural study. Acta neuropath. (Berl.)12, 103–115 (1969)
Gruner, J. E.: Spongiose athiamique expérimentale. Structure fine. Acta neuropath. (Berl.) Suppl. III, 131–136 (1967)
Hirano, A., Rembitzer, H. M., Becker, N. H., Zimmerman, H. H.: The distribution of peroxidase in the triethyltin-intoxicated rat brain. J. Neuropath. exp. Neurol.28, 507–511 (1969)
Hirano, A., Zimmerman, H. M., Levine, S.: Intramyelinic and extracellular spaces in triethyltin intoxication. J. Neuropath. exp. Neurol.27, 571–580 (1968)
Jellinger, K., Seitelberger, F.: Subacute necrotizing encephalomyelopathy (Leigh) Ergebn. inn. Med. Kinderheilk., N. F.29, 155–219 (1970a)
Jellinger, K., Seitelberger, F.: Spongy degeneration of the central nervous system in infancy. Path.53, 90–160 (1970b)
Katzman, R., Aleu, F., Wilson, C.: Further observations on triethyltin edema. Arch. Neurol. (Chic.)9, 178–187 (1963)
Lampert, P., O'Brien, J., Garrett, R.: Hexachlorophene encephalopathy. Acta neuropath. (Berl.)23, 326–333 (1973)
Lampert, P. E., Schochet, S. S.: Electron microscopic observations on experimental spongy degeneration of the cerebellar white matter. J. Neuropath. exp. Neurol.27, 210–220 (1968)
Mancall, E. L., McEntee, W. J.: Alterations of the cerebellar cortex in nutritional encephalopathy. Neurology (Minneap.)15, 303–313 (1965)
Manz, H. J., Robertson, D. M.: Vascular permeability to horseradish peroxidase in brain stem lesions of thiamine-deficient rats. Amer. J. Path.66, 565–576 (1972)
McCandless, D. W., Schenker, S.: Encephalopathy of thiamine deficiency: Studies of intracerebral mechanisms. J. clin. Invest.47, 2268–2280 (1968)
Montpetit, V. J. A., Andermann, F., Carpenter, S., Fawcett, J. S., Zborowska-Sluis, D., Giverson, H. R.: Subacute necrotizing encephalomyelopathy. A review and a study of two families. Brain94, 1–30 (1971)
Prados, M., Swank, R. L.: Vascular and interstitial cell changes in thiamine deficient animals. Arch. Neurol. Psychiat. (Chic.)47, 626–644 (1942)
Raimondi, A. J., Evans, J. P., Mullan, S.: Studies of cerebral edema. III. Alteration in white matter: An electron microscopic study using ferritin as a labeling compound. Acta neuropath. (Berl.)2, 177–197 (1962)
Richter, R. B.: Infantile subacute necrotizing encephalopathy (Leigh's disease). Its relationship to Wernicke's encephalopathy. Neurology (Minneap.)18, 1125–1132 (1968)
Riggs, H. E., Boles, R. S.: Wernicke's disease. A clinical and pathological study of 42 cases. Quart. J. Stud. Alcohol5, 361–370 (1944)
Rinehart, J. F., Friedman, M., Greenberg, L. D.: Effect of experimental thiamine deficiency on the nervous system of the rhesus monkey. Arch. Path.48, 129–139 (1949)
Robertson, D. M., Wasan, S. M., Skinner, D. B.: Ultrastructural features of early brain stem lesions of thiamine-deficient rats. Amer. J. Path.52, 1081–1097 (1968)
Schröder, J. M., Wechsler, W.: Ödem und Nekrose in der grauen und weißen Substanz beim experimentellen Hirntrauma (Licht- und elektronenmikroskopische Untersuchungen). Acta neuropath. (Berl.)5, 82–111 (1965)
Seider, D., Trautwein, G.: Encephalopathie bei Thiaminmangel des Nerzes. Acta neuropath. (Berl.)19, 155–165 (1971)
Sipe, J. C.: Leigh's syndrome: The adult form of subacute necrotizing encephalomyelopathy with predilection for the brain stem. Neurology (Minneap.)23, 1030–1038 (1973)
Sipe, J. C., Vick, N. A., Schulman, S.: Plasmocid encephalopathy in the rhesus monkey: A study of selective vulnerability. J. Neuropath. exp. Neurol.32, 446–457 (1973)
Suzuki, K.: Some new observations in triethyltin intoxication of rats. Exp. Neurol.31, 207–213 (1971)
Suzuki, K., Kikkawa, Y.: Status spongiosus of CNS and hepatic changes induced by cuprizone (biscyclohexone oxalyldihydrazone). Amer. J. Path.54, 307–321 (1969)
Tellez, I., Terry, R. D.: Fine structure of the early changes in the vestibular nuclei of the thiamine-deficient rat. Amer. J. Path.52, 777–794 (1968)
Torack, R., Gordon, J., Prokop, J.: Pathobiology of acute triethyltin intoxication. Int. Rev. Neurobiol.12, 45–86 (1970)
Ule, G., Kolkmann, F. W.: Experimentelle Untersuchungen zur Wernickeschen Encephalopathie. Acta neuropath. (Berl.)11, 361–367 (1968)
Van Harreveld, A., Khattab, F. I.: Electron microscopy of asphyxiated spinal cords of cats. J. Neuropath. exp. Neurol.26, 521–536 (1967)
Vick, N. A., Bigner, D. D.: Microvascular abnormalities in virally-induced canine brain tumors: Structural bases for altered blood-brain barrier function. J. neurol. Sci.17, 29–39 (1972)
Vick, N. A., Schulman, S.: Fine structure of the thiamine deficient rhesus monkey brain. (Abstract.) Neurology (Minneap.)24, 362–363 (1974)
Victor, M., Adams, R. D., Collins, G. H.: The Wernicke-Korsakoff syndrome. Philadelphia: F. A. Davis Comp. 1971
Webster, H. DeF.: Transient focal accumulation of axonal mitochondria during the early stages of Wallerian degeneration. J. Cell. Biol.12, 361–383 (1962)
Webster, H. DeF., Ulsamer, A. G., O'Connel, M. F.: Hexachlorophene induced myelin lesions in the develo** nervous system ofXenopus tadpoles: Morphological and biochemical abservations. J. Neuropath. exp. Neurol.33, 144–163 (1974)
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Supported by NIH grants 05033-20, NS-09789 and MH-02744, and the Louis Block fund for Research in the Physical and Biological Sciences at the University of Chicago.
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Blank, N.K., Vick, N.A. & Schulman, S. Wernicke's encephalopathy. Acta Neuropathol 31, 137–150 (1975). https://doi.org/10.1007/BF00688148
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DOI: https://doi.org/10.1007/BF00688148