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马基亚法瓦-比尼亚米病的临床和放射学特征。

Clinical and radiological features of Marchiafava-Bignami disease.

作者信息

Dong Xiaoyu, Bai Chaobo, Nao Jianfei

机构信息

Department of Neurology, Shengjing Hospital of China Medical University, Heping District, Shenyang, Liaoning, People's Republic of China.

出版信息

Medicine (Baltimore). 2018 Feb;97(5):e9626. doi: 10.1097/MD.0000000000009626.

Abstract

Marchiafava-Bignami disease (MBD) is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and necrosis. Our aims were to describe the clinicoradiological features and identify factors that may affect the prognosis of patients with MBD.We examined clinical manifestations, laboratory results, and neuroradiological features of 9 patients with MBD. The patients were classified into 2 subgroups (favorable and poor outcome subgroups) based on the Modified Oxford Handicap Scale (MOSH). In addition, we compared the clinical and neuroimaging features between the 2 subgroups.Nine adult male patients (age of onset range 37-62 years, with a mean age of 47.00 ± 14.50 years) were included in this study. According to MOSH, 4 patients were placed in the poor outcome subgroup (MOHS ≥ 3), 5 patients were placed in the favorable outcome subgroup (MOHS ≤ 2). Relatively high score of MAST-C (≥6) (P = .008), extracallosal lesions (P = .048), GCS (P = .026), cerebral lobe impairment (P = .048) was significantly more common in the poor outcome subgroup.Clinical manifestations of MBD are variable and lack specificity. Early diagnosis by relatively specific performance of bisymmetric lesions in corpus callosum of diffusion-weighted imaging (DWI) may affect the prognosis. The prognosis of patients with severe disturbance of consciousness, heavy alcohol consumption, extracallosal lesions, cerebral lobe impairment is probably unfavorable.

摘要

马基亚法瓦-比尼亚米病(MBD)是一种罕见的神经系统疾病,通常与慢性酒精中毒有关,其特征为脱髓鞘和坏死。我们的目的是描述临床放射学特征,并确定可能影响MBD患者预后的因素。我们检查了9例MBD患者的临床表现、实验室检查结果和神经放射学特征。根据改良牛津残疾量表(MOSH)将患者分为2个亚组(预后良好和预后不良亚组)。此外,我们比较了2个亚组之间的临床和神经影像学特征。本研究纳入了9例成年男性患者(发病年龄范围为37-62岁,平均年龄为47.00±14.50岁)。根据MOSH,4例患者被归入预后不良亚组(MOHS≥3),5例患者被归入预后良好亚组(MOHS≤2)。MAST-C评分较高(≥6)(P = 0.008)、胼胝体外病变(P = 0.048)、格拉斯哥昏迷量表(GCS)评分(P = 0.026)、脑叶损害(P = 0.048)在预后不良亚组中明显更常见。MBD的临床表现多样且缺乏特异性。通过弥散加权成像(DWI)胼胝体双侧对称病变的相对特异性表现进行早期诊断可能会影响预后。意识严重障碍、大量饮酒、胼胝体外病变、脑叶损害患者的预后可能不佳。

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