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1例以肿胀性脱髓鞘病变为表现的髓鞘少突胶质细胞糖蛋白抗体相关疾病

A case of myelin oligodendrocyte glycoprotein-antibody-associated disease presenting with tumefactive demyelinating lesion.

作者信息

Ataka Takuya, Kimura Noriyuki, Matsubara Etsuro

机构信息

Department of Neurology, Faculty of Medicine, Oita University.

Department of Neurology, Faculty of Medicine, Oita University.

出版信息

Mult Scler Relat Disord. 2020 Aug;43:102191. doi: 10.1016/j.msard.2020.102191. Epub 2020 May 23.

DOI:10.1016/j.msard.2020.102191
PMID:32474283
Abstract

We report the case of a patient with myelin oligodendrocyte glycoprotein (MOG)- antibody-associated disease presenting with tumefactive demyelinating lesion. Neurological examination showed aphasia, acalculia, agraphia, alexia, left-right disorientation, and right hemiplegia. Brain magnetic resonance imaging revealed a large monofocal lesion with mild brain edema and ring enhancement. Stereotactic brain biopsy was performed, and neuropathological findings showed inflammatory demyelination and preserved axons without tumor cells. A cell-based assay detected anti-MOG antibody in the cerebrospinal fluid. Neurological symptoms gradually improved after steroid pulse therapy. MOG-antibody-associated diseases should be considered in the differential diagnosis of tumefactive demyelinating lesion.

摘要

我们报告了一例患有髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病并伴有肿胀性脱髓鞘病变的患者。神经系统检查显示失语、失算、失写、失读、左右定向障碍和右侧偏瘫。脑磁共振成像显示一个大的单发病灶,伴有轻度脑水肿和环形强化。进行了立体定向脑活检,神经病理学结果显示炎性脱髓鞘且轴突保留,无肿瘤细胞。基于细胞的检测在脑脊液中检测到抗MOG抗体。类固醇脉冲治疗后神经症状逐渐改善。在肿胀性脱髓鞘病变的鉴别诊断中应考虑MOG抗体相关疾病。

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