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抗 MOG 抗体相关疾病伴左侧半球肿块样病变前的失语性癫痫持续状态。

Aphasic status epilepticus preceding tumefactive left hemisphere lesion in anti-MOG antibody associated disease.

机构信息

Department of Neurology, The University of Tokyo, Tokyo, Japan.

Department of Neurology, The University of Tokyo, Tokyo, Japan.

出版信息

Mult Scler Relat Disord. 2019 Jan;27:91-94. doi: 10.1016/j.msard.2018.10.012. Epub 2018 Oct 15.

DOI:10.1016/j.msard.2018.10.012
PMID:30347340
Abstract

INTRODUCTION

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have recently been associated with epilepsy with FLAIR hyperintense cortical lesions on MRI. Association between anti-MOG antibodies and epilepsy without detectable structural brain lesion on MRI is unknown.

CASE REPORT

A 48-year-old right-handed man with a four-and-a-half year history of anti-MOG antibody associated demyelinating disease presented with persistent global aphasia. Brain MRI showed no new lesion or cortical lesion in the left hemisphere. Electroencephalogram, magnetoencephalography, and brain perfusion single-photon emission computed tomography suggested epileptic foci in the left temporal and parietal lobes, and the patient's aphasia transiently responded to intravenous diazepam, compatible with aphasic status epilepticus. Cerebrospinal fluid showed mildly elevated cell count and positive oligoclonal bands. The patient only partially responded to antiepileptic drugs but responded to steroid pulse therapy. Six months later, the patient again exhibited global aphasia. Brain MRI showed tumefactive white matter lesion in the left temporo-parietal lobes.

CONCLUSION

Autoimmune epilepsy without obvious causative lesion on MRI can be seen in the course of anti-MOG antibody associated demyelinating disease. The subsequent emergence of tumefactive lesion closely located to the epileptic foci may suggest some association between autoimmune epilepsy and demyelinating lesions.

摘要

简介

抗髓鞘少突胶质细胞糖蛋白(MOG)抗体最近与 MRI 上出现 FLAIR 高信号皮质病变的癫痫相关。抗 MOG 抗体与 MRI 上未检测到结构性脑病变的癫痫之间的关联尚不清楚。

病例报告

一名 48 岁右利手男性,患有抗 MOG 抗体相关脱髓鞘疾病四年半,表现为持续性全面性失语。脑部 MRI 未显示左侧半球有新的病变或皮质病变。脑电图、脑磁图和脑灌注单光子发射计算机断层扫描提示左颞叶和顶叶有癫痫灶,患者的失语症短暂性地对静脉注射地西泮有反应,与失语性癫痫状态相符。脑脊液显示细胞计数轻度升高且存在寡克隆带。患者仅对抗癫痫药物部分反应良好,但对类固醇冲击治疗有反应。六个月后,患者再次出现全面性失语。脑部 MRI 显示左侧颞顶叶有肿块样的白质病变。

结论

在抗 MOG 抗体相关脱髓鞘疾病的病程中,可能会出现 MRI 上无明显病因病变的自身免疫性癫痫。随后紧邻癫痫灶出现肿块样病变可能提示自身免疫性癫痫与脱髓鞘病变之间存在一定关联。

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