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[马基亚法瓦-比尼亚米综合征。慢性酒精中毒中的脑桥外髓鞘溶解症]

[Marchiafava-Bignami syndrome. Extrapontine myelinolysis in chronic alcoholism].

作者信息

Gabriel S, Grossmann A, Höppner J, Benecke R, Rolfs A

机构信息

Klinik für Neurologie und Poliklinik der Universität, Rostock.

出版信息

Nervenarzt. 1999 Apr;70(4):349-56. doi: 10.1007/s001150050447.

Abstract

Until now, the pathogenesis of Marchiafava-Bignami disease, an extrapontine myelinolysis, is unknown. Accept an abuse of alcohol for many years additional metabolic and vascular disturbances are supposed. The early performance of magnetic resonance imaging in patients with a sudden encephalopathy and history of alcoholism is essential for an assessment of the diagnosis. The bilateral lack of transcallosal inhibition--a parameter for the integrity of the transcallosal connections between motor cortices--is the consequence of the axonal degeneration of transcallosal fibers. Clinical and neuroradiological features of three patients with extrapontine myelinolysis are reported and possible etiologies of the complex disease are discussed. In two cases a severe alcohol abuse was present with the typical picture of the Marchiafava-Bignami disease. In a third patient an intoxication with methanol was present with a similar clinical picture. Although severe neurological disturbances were initially apparent in the patients, during the follow-up there was a significant amelioration of the clinical course under a high-dose vitamin B supplementation.

摘要

迄今为止,作为一种脑桥外髓鞘溶解症的马奇亚法瓦-比尼亚米病的发病机制尚不清楚。除了多年酗酒外,还推测存在其他代谢和血管紊乱。对于患有突发性脑病且有酗酒史的患者,早期进行磁共振成像检查对于诊断评估至关重要。双侧胼胝体抑制缺失——这是运动皮质之间胼胝体连接完整性的一个参数——是胼胝体纤维轴索变性的结果。本文报告了3例脑桥外髓鞘溶解症患者的临床和神经放射学特征,并讨论了这种复杂疾病可能的病因。其中2例存在严重酗酒情况,具有典型的马奇亚法瓦-比尼亚米病表现。第3例患者因甲醇中毒,临床表现相似。尽管患者最初出现了严重的神经功能障碍,但在随访期间,在大剂量补充维生素B的情况下,临床病程有了显著改善。

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