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一例抗髓鞘相关糖蛋白性多发性神经病合并多发性硬化症:是一种疾病而非两种?

A case of anti-myelin-associated glycoprotein polyneuropathy and multiple sclerosis: one disease instead of two?

作者信息

Sotgiu Stefano, Giua Alessandra, Murrighile Maria R, Ortu Renato

机构信息

University of Sassari, Section of Neurology, Department of Neuroscience and Maternal-Infant Sciences, Viale San Pietro, 10, Sassari, 07100, Italy.

出版信息

BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.06.2008.0212. Epub 2009 Feb 20.

Abstract

A male patient diagnosed elsewhere as having multiple sclerosis (MS) was recently referred to our MS centre. Despite the presence of scattered T2-hyperintense MS-like lesions on MRI and cerebrospinal fluid (CSF) oligoclonal bands, his MS diagnosis was unpersuasive. Distal symmetric hypotonia, tendon areflexia and distal muscle weakness were present. A mostly demyelinating sensory polyneuropathy was disclosed at electroneurography. Serum IgM band, free monoclonal light chains and increased anti-myelin-associated glycoprotein IgM were detected. At 18 months later, and after three intravenous Ig treatments, a clinical electroneurographic improvement was evident along with the disappearance of some brain MRI lesions, reduction of serum anti-myelin-associated glycoprotein (MAG) IgM level and of the number of CSF oligoclonal bands. Although the cause/effect relation cannot be proven, we hypothesise that not only peripheral but also central demyelination may be related to the presence of anti-MAG antibodies with central nervous system (CNS) patterns on MRI resembling those seen in MS.

摘要

一名在其他地方被诊断为患有多发性硬化症(MS)的男性患者最近被转诊至我们的MS中心。尽管磁共振成像(MRI)上存在散在的T2高信号、类似MS的病变以及脑脊液(CSF)寡克隆带,但他的MS诊断并不令人信服。患者存在远端对称性肌张力减退、腱反射消失和远端肌肉无力。神经电生理检查显示为主要脱髓鞘性感觉性多发性神经病。检测到血清IgM条带、游离单克隆轻链以及抗髓鞘相关糖蛋白IgM升高。18个月后,在接受三次静脉注射免疫球蛋白治疗后,临床和神经电生理明显改善,同时一些脑MRI病变消失,血清抗髓鞘相关糖蛋白(MAG)IgM水平降低,CSF寡克隆带数量减少。尽管因果关系无法得到证实,但我们推测不仅周围神经脱髓鞘,而且中枢神经脱髓鞘可能与抗MAG抗体的存在有关,其在MRI上的中枢神经系统(CNS)表现类似于MS所见。

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