原发性胼胝体变性临床特点

Clinical features of primary Marchiafava-Bignami disease

目的探讨原发性胼胝体变性(MBD)的临床及影像学特点。方法回顾性分析7例MBD患者的临床资料。结果 7例均有大量饮酒史,临床上以意识障碍、精神症状、智能下降、反应迟钝、肢体震颤为主要表现。急性患者主要以意识障碍、精神异常起病;亚急性以快速进展的痴呆起病;慢性以智能下降、反应迟钝起病。患者MRI检查均可见胼胝体异常信号,为长T_1、T_2信号,Flair高信号。急性及亚急性起病者以胼胝体膨胀性改变为特征,慢性起病以"三文治征"为特征;2例伴胼胝体邻近白质病灶。结论 MBD多由于慢性酒精中毒所致,临床表现无特异性。MRI检查发现胼胝体对称性病灶有助于诊断。

Objective To investigate the clinical features and imaging features of primary Marchiafava-Bignami disease（MBD）. Methods The clinical data of 7 cases of MBD were analyzed retrospectively. Results All the 7 patients had a history of heavy alcohol consumption. The main clinical manifestations included disturbance of consciousness, mental symptoms, decreased intelligence, slow reaction and limb tremor. Acute onset patients mainly presented with disturbance of consciousness and mental symptoms; subacute onset patients presented with the rapid progress of dementia; chronic onset patients mainly presented with decreased intelligence and slow reaction. MRI examination showed abnormal signals on corpus eallosum in all the eases, with hypointensity on TI WI single, hyperintensity on T2WI single, and hyperintensity on Flair. The characteristic MRI of acute and subacute onset cases were swollen corpus callosum, while chronic onset cases showed the ＂ sandwich sign＂; neighboring white matter lesions beyond the corpus callosum were detected in 2 patients. Conclusions MBD is closely associated with chronic alcoholism etiologically, and the clinical manifestations lacked specificity. Symmetry of corpus callosum lesions on MRI is very heloful to the diagnosis.