Marchiafava-Bignami病2例报告并文献复习

Two Case Reports of Marchiafava-Bignami Disease and Literature Review

目的 :探讨 Marchiafava- Bignami病 (MBD)的病因、临床表现、影像特点及诊断。方法 :分析 2例 MBD并复习文献。结果 :2例男性酗酒者均有情感淡漠、步态障碍、认知损害等脑病表现。脑 MRI均发现大片对称的胼祗体 (CC)T1 WI低信号 ,T2 WI高信号病变 ,但脑 CT未发现明显 CC病变。均予维生素 B1 治疗 ,例 1好转 ,例 2死亡。结论 :MBD的临床表现缺乏特异性 ,CT对 MBD不敏感。酗酒和 /或营养不良史 ,脑病表现和 MRI发现大片对称的 CC病变支持 MBD的诊断。

Objective:To investigate the etiology,clinical representation,MRI finding and diagnosis of Marchiafava-Bignami disease(MBD).Methods:2 cases of MBD were analyzed and the literature was reviewed.Results:Both male alcoholics had apathy, gait disturbance and cognitive impairment. MRI showed large and symmetrical low-intensity in T 1WI and high-intensity in T 2WI lesion of the corpus callosum(CC),while CT did not find definite CC lesion. Both were treated with thiamine,one case improved and another case died.Conclusions:Clinical manifestation of MBD is nonspecific, CT is not sensitive for MBD.History of alcoholism and/or malnutrition, encephalopathic manifestation,large and symmetrical CC lesion in MRI support the diagnosis of MBD.