胼胝体变性六例影像学特点和临床表现

Imaging and clinical characteristics of Marchiafava-Bignami disease： six cases

目的 探讨胼胝体变性（Marchiafava-Bignami disease,MBD）的影像学特点和临床表现.方法 对我院诊治的6例MBD患者进行回顾性分析,包括影像学检查（如头颅CT、MRI）、实验室检查、临床症状和随访情况.结果 6例患者MRI检查均表现为胼胝体肿胀及T1WI等或低信号,T2WI高信号,液体衰减反转恢复序列高信号,弥散加权成像示弥散受限.其中例1、例4和例6以急性意识障碍起病,CT和MRI检查除累及整个胼胝体外双侧半卵圆区也受累,预后较差.例2和例3以反应迟钝、记忆力下降起病,CT和MRI检查病变主要累及胼胝体膝部和压部,经积极治疗,患者基本恢复正常.例5以双下肢麻木无力、反应迟钝起病,MRI检查病变累及胼胝体压部及体部,经治疗后也恢复正常.结论 MBD临床表现复杂,但具有特征性影像学表现,其起病形式和影像学特征可作为反映其预后的重要因素之一.

Objective To investigate the presentation and radiologic findings of MarchiafavaBignami disease （MBD）.Methods Six cases of MBD who were diagnosed and treated in our hospital were retrospectively analyzed,including the imaging examination（such as cranial CT,magnetic resonance imaging （MRI））,laboratory tests,clinical symptoms and prognosis.Results Six cases of MBD disease were presented with swelled,thickened corpus callosum,iso-or hypo-intensity on T1WI,hyper-intensity on T2WI and fluid attenuated inversion recovery and restricted diffusion on diffusion weighted imaging.Coma of acute onset was the major clinical finding in case 1,case 4 and case 6,which showed the lesions in the entire corpus callosum and extended to the white matter,and the prognosis of these cases were worse.Slow reaction and memory decrease were the clinical findings in case 2 and case 3,which showed the genu and splenium of corpus callosum involvement on MRI and CT,and returned to normal with aggressive treatment.Numbness and weakness of the lower lambs were the major clinical finding in case 5,which showed that the lesion was limited in the splenium and body of corpus callosum on MRI.The patient recovered after treatment.Conclusions MBD may present with various clinical forms,but have characteristic imaging findings.Outset form and neuroimaging characteristics of MBD are critical for improvement of the clinical outcome.