Summary
A 67-year-old Japanese woman with liver cirrhosis was affected by an unusual acute progressive encephalopathy, presenting mental confusion and slurred speech as its initial symptoms. She died in profound coma, following the entire course of 17 days. Autopsy disclosed bilateral symmetrical, widespread, edematous and necrotic lesions, their centers being located in the basal ganglia, diencephalon and midbrain, and their peripheries expanding into the cerebral white matter, cerebellum, pons and medulla. Diapedesis of erythrocytes and serum plasma was conspicuous, in contrast to paucity of capillary proliferation. Although the lesions were somewhat similar to those of Wernicke's and Leigh's encephalopathies, they were considered to be representative of a more acute metabolic disorder distinct from the latter conditions.
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References
Campbell ACP, Russell WR (1941) Wernicke's encephalopathy: the clinical features and their probable relationship to vitamin B deficiency. Q J Med 10:41–64
Cravioto H, Korein J, Silberman J (1961) Wernicke's encephalopathy. A clinical and pathological study of 28 autopsied cases. Arch Neurol 4:510–519
Feigin I, Goebel HH (1969) “Infantile” subacute necrotizing encephalopathy in the adult. Neurology 19:749–759
Gray F, Louarn F, Gherardi R, Eizenbaum JF, Marsault C (1984) Adult form of Leigh's disease: a clinicopathological case with CT scan examination. J Neurol Neurosurg Psychiatry 47:1211–1215
Kalimo H, Lundberg PO, Olsson Y (1979) Familial subacute necrotizing encephalomyelopathy of the adult form (adult Leigh syndrome). Ann Neurol 6:200–206
Lindenberg R (1982) Tissue reactions in the gray matter of the central nervous system. In: Haymaker W, Adams RD (eds) Histology and histopathology of the nervous system, vol 1. CC Thomas, Springfield, pp 973–1275
Martin JJ (1972) Sur la délimitation clinicopathologique de l'encéphalopathie de Gayet-Wernicke et de la forme adulte de l'encéphalopathie de Leigh. Acta Neurol Belg 72:347–354
Masó E, Ferrer I, Herraiz J, Roquer J, Serrano S (1984) Leigh's syndrome in an adult. J Neurol 231:253–257
Montpetit VJA, Andermann F, Carpenter S, Fawcett JS, Zborowska-Slius D, Giberson HR (1971) Subacute necrotizing encephalomyelopathy. A review and a study of two families. Brain 94:1–30
Riggs EH, Boles HS (1944) Wernicke's disease: a clinical and pathological study of 42 cases. Q J Stud Alchol 5:361–370
Sipe JC (1973) Leigh's syndrome: the adult form of subacute necrotizing encephalomyelopathy with predilection for the brain stem. Neurology 23:1030–1038
Solheid C, Stoupel N, Martin JJ (1971) La forme adulte à évolution chronique de l'encéphalopathie nécrosante de Leigh. Sa situation vis-à-vis des formes infanto-juvéniles. Acta Neurol Belg 71:282–295
Torvik A (1987) Topographic distribution and severity of brain lesions in Wernicke's encephalopathy. Acta Neuropathol (Berl) 70:82–85
Ulrich J, Fankhauser-Mauri C (1978) Subacute necrotizing encephalopathy (Leigh) in an adult. Eur Neurol 17:241–246
Victor M, Adams RD, Collins GH (1971) The Wernicke-Korsakoff syndrome. FA Davis, Philadelphia, pp 71–130
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Mizuguchi, M., Tomonaga, M., Fukusato, T. et al. Acute necrotizing encephalopathy with widespread edematous lesions of symmetrical distribution. Acta Neuropathol 78, 108–111 (1989). https://doi.org/10.1007/BF00687411
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DOI: https://doi.org/10.1007/BF00687411