Coma and death in unrecognized wernicke's encephalopathy an autopsy study: estudo necroscópico

Lana-peixoto, Marco Aurélio; Santos, Eustáquio Claret Dos; Pittella, José Eymard H.

doi:10.1590/S0004-282X1992000300012

Abstracts

Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 36 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropatholo-gical examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelmolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with Coma of unknown etiology should be given parenteral thiamine.

Wernicke's encephalopathy; coma; clinical diagnosis; neuropathology

Numa série de 36 casos de encefalopatia de Wernicke autopsiados, 11 haviam desenvolvido coma. Em nenhum desses pacientes comatosos o diagnóstico foi estabelecido durante a vida. Seis desses pacientes eram homens e 5 mulheres, com idades entre 26 a 50 anos (média de 36,6). História de alcoolismo crônico foi positiva em 7 casos, sinais de desnutrição grave ocorreram em 3; uma paciente (apresentava câncer gástrico e uma, hiper emese gravídica. Dois pacientes foram admitidos ao hospital após terem sido encontrados em coma em suas residências. O exame neuropatológieo revelou alterações macroscópicas nos corpos mamilares em 8 casos - atrofia, descoloração e espongiose. Atrofia da porção anterior e superior do vermis do cerebelo foi observada em um caso. Era 5 casos havia hemorragias petequiais no diencéfalo, principalmente próximas às paredes do terceiro ventrículo. A microscopia revelou além das hemorragias, proliferação glial, hipertrofia do endotélio vascular e necrose de neurônios e mielina. Mielinólise pontina central ocorreu em um paciente. A encefalopatia de Wernicke é pouco diagnosticada clinicamente. O coma pode mascarar as outras manifestações clínicas da doença ou pode constituir sua única manifestação. Embora seja um sinal de mau prognóstico, o coma pode ser revertido pela administração de tiamina. É enfatizado que todo paciente em coma de causa não estabelecida deve receber altas doses de tiamina.

encefalopatia de Wernicke; coma; diagnóstico clínico; neuropa-tologia

Marco Aurélio Lana-peixoto^I; Eustáquio Claret Dos Santos^II; José Eymard H. Pittella^III

^IAssociate Professor of Neurology

^IINeurologist

^IIIFull-Professor. Department of Neurology (Dr. Lana-Peixoto) and Pathology (Dr. PittelDa), Federal University of Minas Gerais

SUMMARY

Eleven out of 36 autopsied cases of Wernicke's encephalopathy had developed coma. None of these patients had the diagnosis during life. There were six men and five women with ages ranging from 36 to 50 years (mean 36.6). Seven of these patients were heavy drinkers, three exhibited signs of severe malnutrition, whereas one was being evaluated for a disseminated gastric cancer and one was in treatment of hyperemesis gravidarum. Two patients were brought to the hospital after found unconscious at home. Neuropatholo-gical examination disclosed gross changes in the mammillary bodies in eight cases and microscopic changes in all cases. In one case there was atrophy of the anterior superior part of the vermis. Petechial hemorrhages were observed particularly in the walls of the third ventricle. Microscopically there were in addition to hemorrhages, glial proliferation, endothelial hypertrophy and necrosis of nerve cells and myelin. Central pontine myelmolysis was observed in one case. Wernicke's encephalopathy is a clinically underdiagnosed condition. Coma may mask its classical clinical picture or even be the sole manifestation. Although coma points to a poor outlook it may be reversed by thiamine administration. Any patient with Coma of unknown etiology should be given parenteral thiamine.

Key words: Wernicke's encephalopathy, coma, clinical diagnosis, neuropathology.

RESUMO

Numa série de 36 casos de encefalopatia de Wernicke autopsiados, 11 haviam desenvolvido coma. Em nenhum desses pacientes comatosos o diagnóstico foi estabelecido durante a vida. Seis desses pacientes eram homens e 5 mulheres, com idades entre 26 a 50 anos (média de 36,6). História de alcoolismo crônico foi positiva em 7 casos, sinais de desnutrição grave ocorreram em 3; uma paciente (apresentava câncer gástrico e uma, hiper emese gravídica. Dois pacientes foram admitidos ao hospital após terem sido encontrados em coma em suas residências. O exame neuropatológieo revelou alterações macroscópicas nos corpos mamilares em 8 casos atrofia, descoloração e espongiose. Atrofia da porção anterior e superior do vermis do cerebelo foi observada em um caso. Era 5 casos havia hemorragias petequiais no diencéfalo, principalmente próximas às paredes do terceiro ventrículo. A microscopia revelou além das hemorragias, proliferação glial, hipertrofia do endotélio vascular e necrose de neurônios e mielina. Mielinólise pontina central ocorreu em um paciente. A encefalopatia de Wernicke é pouco diagnosticada clinicamente. O coma pode mascarar as outras manifestações clínicas da doença ou pode constituir sua única manifestação. Embora seja um sinal de mau prognóstico, o coma pode ser revertido pela administração de tiamina. É enfatizado que todo paciente em coma de causa não estabelecida deve receber altas doses de tiamina.

Palavras-chave: encefalopatia de Wernicke, coma, diagnóstico clínico, neuropa-tologia.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Dr. Marco Aurélio Lana-Peixoto Departamento de Neurologia, Instituto Hilton Rocha Av. Anel dm Serra 1355 - 30210 Belo Horizonte MG - Brasil.

1. Barrie HJ. Wernicke's encephalopathy in surgical practice: report of three cases. Lancet 1947, 2:278-279.
2. Cravioto H, Korein J, Silberman J. Wernicke's encephalopathy: a clinical and pathological study of 28 autopsied cases. Arch Neurol 1961, 4 :510-519.
3. Devathasan G, Koh C. Wernicke's encephalopathy in prolonged fasting. Lancet 1982, 2:1108-1109.
4. Donnan GA, Seeman E. Coma and hypothermia in Wernicke's encephalopathy. Aust N Z J Med 1980, 10:438-440.
5. Gibb WRG, Gorsuch AN, Lees AJ, Yuokin JS. Reversible coma in Wernicke's encephalopathy. Postgrad Med J 1985, 61:607-610.
6. Handler CE, Perkin GD. Anorexia nervosa and Wernicke's encephalopathy: an underlying association. Lancet 1982, 2:771-772.
7. Harper C. Wernicke's encephalopathy: a more common disease than realised: a neuropathological study of 51 cases. J Neurol Neurosurg Psychiatry 1979, 42:226-231.
8. Harper CG. Sudden unexpected death and Wernicke's encephalopathy, a complication of prolonged intravenous feeding. Aust N Z J Med 1980, 10:230-235.
9. Harper C. Confusion, coma and death from a preventable disease. Med J Aust 1981, 2:219-221.
10. Harper C. The incidence of Wernicke's encephalopathy in Australia: a neuropathological study of 131 cases. J Neurol Neurosurg Psychiatry 1983, 46 : 593-598.
11. Harper CG, Giles M, Finley-Jones R. Clinical signs in the Wernicke-Korsakoff complex: a retrospective analysis of 131 cases at necropsy. J Neurol Neurosurg Psychiatry 1886, 49:341-345.
12. Lavin PJM, Smith D, Kori SH. Ellenburger C Jr. Wernicke's encephalopathy, a pre-predictable complication of hyperemesis gravidarum. Obstet Gynecol 1983, 62 (Suppl) : 13-15.
13. Lishman WA. Cerebral disorder in alcoholism: syndromes of impairment. Brain 1981, 104:1-20.
14. Lopez RI, Collins GH. Wernicke's enrephalopathy: a complication of chronic hemodyalisis. Arch Neurol 1968, 18:248-259.
15. Mumford CJ. Papilloedema delaying diagnosis of Wernicke's encephalopathy in a comatose patient. Postgrad Med J 1989, 65 : 371-373.
16. Nakada T, Knight Rt. Alcohol and the central nervous system. Med Clin N Amer 1984, 68:121-131.
17. Nightingale S, Bates D, Heath PD, Barron SL. Wernicke's encephalopathy in hypere-mesis gravidarum. Postgrad Med J 1982, 58 : 558-559.
18. Pentland B, Mawdsley C. Wernicke's encephalopathy following «hunger strike». Postgrad Med J 1982, 58:427-428.
19. Revler JB, Girard DOB, Cooney TG. Wernicke's encephalopathy. N Engl J Med 1985, 312: 1035-1039.
20. Shaw S, Gorkin BD, Lieber CS. Effects of chronic alcohol feeding on thiamine status: biochemical and neurological correlates. Am J Clin Nutr 1981, 34:856-860.
21. Torvik A, Lindboe CF, Rogde S, Brain lesions in alcoholics. J Neurol Sci 1982, 56 : 233-248.
22. Victor M. The Wernicke-Korsakoff syndrome. In Vinken PJ, Bruyn GW (eds): Handbook of Clinicad Neurology. Amsterdam: Elsevier-North Holland, 1976, Vol 28, p 245-270.
23. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff syndrome. Philadelphia; F. A Davis Company, 1971, p 17.
24. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff syndrome. Philadelphia: F. A. Davis Company, 1971, p 21.
25. Wallis WE, Willoughby E, Baker P. Coma in the Wernicke-Karsakoff syndrome. Lancet 1978, 2:400-401.
26. Wernicke C. Lehrbuch der Gehirnkrankheiten für Aerzte und Studirende. Kassel: Theo-dor Fischer, 1981, vol 2, p 229-242.
27. Wood P, Murray A, Sinha B, Godley M, Goldsmith HJ. Wernicke's encephalopathy induced by hyperemesis gravidarum: case reports. Br J Obstet Gynaecol 1983, 90:583-586.
28. Ziporin ZZ, Nunes WT, Powell RC. Excretion of thiamine and its metabolites in the urine of young adult males receiving restricted intakes of the vitamin. J Nutr 1965, 85: 287-296.
29. Ziporin ZZ, Nunes WT, Powell RC. Thiamine requirement in the adult human measured by urinary exertion of thiamine metabolites. J Nutr 1965, 85:297-304.

Coma and death in unrecognized wernicke's encephalopathy an autopsy study

Coma e morte na encefalopatia de Wernioke: estudo necroscópico

Publication Dates

Publication in this collection
22 Feb 2011
Date of issue
Sept 1992

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Barrie HJ. Wernicke's encephalopathy in surgical practice: report of three cases. Lancet 1947, 2:278-279.

[2] 2. Cravioto H, Korein J, Silberman J. Wernicke's encephalopathy: a clinical and pathological study of 28 autopsied cases. Arch Neurol 1961, 4 :510-519.

[3] 3. Devathasan G, Koh C. Wernicke's encephalopathy in prolonged fasting. Lancet 1982, 2:1108-1109.

[4] 4. Donnan GA, Seeman E. Coma and hypothermia in Wernicke's encephalopathy. Aust N Z J Med 1980, 10:438-440.

[5] 5. Gibb WRG, Gorsuch AN, Lees AJ, Yuokin JS. Reversible coma in Wernicke's encephalopathy. Postgrad Med J 1985, 61:607-610.

[6] 6. Handler CE, Perkin GD. Anorexia nervosa and Wernicke's encephalopathy: an underlying association. Lancet 1982, 2:771-772.

[7] 7. Harper C. Wernicke's encephalopathy: a more common disease than realised: a neuropathological study of 51 cases. J Neurol Neurosurg Psychiatry 1979, 42:226-231.

[8] 8. Harper CG. Sudden unexpected death and Wernicke's encephalopathy, a complication of prolonged intravenous feeding. Aust N Z J Med 1980, 10:230-235.

[9] 9. Harper C. Confusion, coma and death from a preventable disease. Med J Aust 1981, 2:219-221.

[10] 10. Harper C. The incidence of Wernicke's encephalopathy in Australia: a neuropathological study of 131 cases. J Neurol Neurosurg Psychiatry 1983, 46 : 593-598.

[11] 11. Harper CG, Giles M, Finley-Jones R. Clinical signs in the Wernicke-Korsakoff complex: a retrospective analysis of 131 cases at necropsy. J Neurol Neurosurg Psychiatry 1886, 49:341-345.

[12] 12. Lavin PJM, Smith D, Kori SH. Ellenburger C Jr. Wernicke's encephalopathy, a pre-predictable complication of hyperemesis gravidarum. Obstet Gynecol 1983, 62 (Suppl) : 13-15.

[13] 13. Lishman WA. Cerebral disorder in alcoholism: syndromes of impairment. Brain 1981, 104:1-20.

[14] 14. Lopez RI, Collins GH. Wernicke's enrephalopathy: a complication of chronic hemodyalisis. Arch Neurol 1968, 18:248-259.

[15] 15. Mumford CJ. Papilloedema delaying diagnosis of Wernicke's encephalopathy in a comatose patient. Postgrad Med J 1989, 65 : 371-373.

[16] 16. Nakada T, Knight Rt. Alcohol and the central nervous system. Med Clin N Amer 1984, 68:121-131.

[17] 17. Nightingale S, Bates D, Heath PD, Barron SL. Wernicke's encephalopathy in hypere-mesis gravidarum. Postgrad Med J 1982, 58 : 558-559.

[18] 18. Pentland B, Mawdsley C. Wernicke's encephalopathy following «hunger strike». Postgrad Med J 1982, 58:427-428.

[19] 19. Revler JB, Girard DOB, Cooney TG. Wernicke's encephalopathy. N Engl J Med 1985, 312: 1035-1039.

[20] 20. Shaw S, Gorkin BD, Lieber CS. Effects of chronic alcohol feeding on thiamine status: biochemical and neurological correlates. Am J Clin Nutr 1981, 34:856-860.

[21] 21. Torvik A, Lindboe CF, Rogde S, Brain lesions in alcoholics. J Neurol Sci 1982, 56 : 233-248.

[22] 22. Victor M. The Wernicke-Korsakoff syndrome. In Vinken PJ, Bruyn GW (eds): Handbook of Clinicad Neurology. Amsterdam: Elsevier-North Holland, 1976, Vol 28, p 245-270.

[23] 23. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff syndrome. Philadelphia; F. A Davis Company, 1971, p 17.

[24] 24. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff syndrome. Philadelphia: F. A. Davis Company, 1971, p 21.

[25] 25. Wallis WE, Willoughby E, Baker P. Coma in the Wernicke-Karsakoff syndrome. Lancet 1978, 2:400-401.

[26] 26. Wernicke C. Lehrbuch der Gehirnkrankheiten für Aerzte und Studirende. Kassel: Theo-dor Fischer, 1981, vol 2, p 229-242.

[27] 27. Wood P, Murray A, Sinha B, Godley M, Goldsmith HJ. Wernicke's encephalopathy induced by hyperemesis gravidarum: case reports. Br J Obstet Gynaecol 1983, 90:583-586.

[28] 28. Ziporin ZZ, Nunes WT, Powell RC. Excretion of thiamine and its metabolites in the urine of young adult males receiving restricted intakes of the vitamin. J Nutr 1965, 85: 287-296.

[29] 29. Ziporin ZZ, Nunes WT, Powell RC. Thiamine requirement in the adult human measured by urinary exertion of thiamine metabolites. J Nutr 1965, 85:297-304.