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酒精性糙皮病脑病的临床谱:22例病理研究的回顾性分析。

The clinical spectrum of alcoholic pellagra encephalopathy. A retrospective analysis of 22 cases studied pathologically.

作者信息

Serdaru M, Hausser-Hauw C, Laplane D, Buge A, Castaigne P, Goulon M, Lhermitte F, Hauw J J

机构信息

Clinique de Neurologie et de Neuropsychologie, Hôpital de la Salpêtrière, Université Paris VI, France.

出版信息

Brain. 1988 Aug;111 ( Pt 4):829-42. doi: 10.1093/brain/111.4.829.

Abstract

A retrospective clinical study of 22 heavy alcohol drinkers is reported in which postmortem study showed diffuse chromatolysis of neurons identical to that found in neurological pellagra, associated in 13 cases with Marchiafava-Bignami disease and/or Wernicke-Korsakoff disease. The clinical features included confusion and/or clouding of consciousness, marked oppositional hypertonus ('gegenhalten') and myoclonus. Because of the frequent coexistence of other alcoholic encephalopathies in the same patient, alcoholic pellagra was often unrecognized. Fifteen patients received thiamine and pyridoxine therapy without niacin. It appeared to aggravate the neurological state or to trigger the development of alcoholic pellagra encephalopathy in 9 cases. The relationship between pellagra occurring during thiamine and pyridoxine therapy and 'nicotinic acid deficiency' is discussed. Multiple vitamin therapy should be given in the treatment of undiagnosed encephalopathies in alcoholic patients.

摘要

本文报告了一项对22名重度饮酒者的回顾性临床研究,尸检研究显示神经元弥漫性染色质溶解,与神经型糙皮病中发现的情况相同,13例与马基亚法瓦-比尼亚米病和/或韦尼克-科尔萨科夫病相关。临床特征包括意识模糊和/或意识混浊、明显的对立性肌张力亢进(“gegenhalten”)和肌阵挛。由于同一患者中常并存其他酒精性脑病,酒精性糙皮病常未被识别。15名患者接受了硫胺素和吡哆醇治疗,但未使用烟酸。在9例患者中,这似乎加重了神经状态或引发了酒精性糙皮病脑病的发展。文中讨论了硫胺素和吡哆醇治疗期间发生的糙皮病与“烟酸缺乏”之间的关系。在治疗酒精性患者未确诊的脑病时,应给予多种维生素治疗。

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