摘要：
目的 分析Marchiafava-Bignami病伴胼胝体外脱髓鞘病变的临床及影像学特点,提高临床医生对该病的认识.方法 收集1例以意识障碍为主要表现,最终诊断为Marchiafava-Bignami病伴脑白质广泛脱髓鞘病变的患者的临床资料,结合文献进行回顾性分析.结果 患者以意识障碍入院,有大量饮酒史20 y,影像学表现为胼胝体、双侧脑室旁、半卵圆中心及额叶白质下多发斑片状长T2信号,符合脱髓鞘改变,综合分析临床特点、详细查体及影像学检查,最终诊断为Marchiafava-Bignami病伴脑白质广泛脱髓鞘病变.检索伴胼胝体外脱髓鞘病变的Marchiafava-Bignami病患者国外报道20例,国内报道3例,我院收治1例,共24例.结论 Marchiafava-Bignami病相对少见,伴胼胝体外脱髓鞘病变的Marchiafava-Bignami病更为罕见,早期诊断及早期治疗对患者的预后具有重要意义.%Objective Aim To analyze the clinical and imaging features of Marchiafava-Bignami disease with demy-elination out of corpus callosum ,in order to improve the clinician ' s knowledge of the disease .Methods The clinical data of 1 case with manifestation of consciousness disorder which was diagnosed Marchiafava -Bignami disease with diffuse demy-elination of cerebral white matter was collected ,and retrospective analysis was carried out with literature .Results The pa-tient with consciousness disorder on admission ,a long drinking history of more than 20 years,the imaging manifestations of corpus callosum ,bilateral periventricular and centrum semiovale and white matter under frontal lobe with multiple patchy long T2 signal,consistent with demyelination ,a comprehensive analysis of the clinical features ,detailed examination and im-aging examination ,the final diagnosis was Marchiafava-Bignami disease with diffuse demyelination of cerebral white matter . 20 cases of Marchiafava-Bignami disease with demyelination out of corpus callosum were reported abroad ,3 cases were re-ported in our country ,and 1 case in our hospital ,altogether 24 cases.Conclusion Marchiafava-Bignami disease is rela-tively rare,and Marchiafava-Bignami disease with demyelination out of corpus callosum is more rare .Early diagnosis and early treatment are important for the prognosis of the patients .
