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一例伴有抗髓鞘少突胶质细胞糖蛋白抗体的单侧复发性大脑皮质脑炎

A case of unilateral recurrent cerebral cortical encephalitis with anti-myelin oligodendrocyte glycoprotein antibodies.

作者信息

Guo Liying, Yun Ho Jun, Tan Xiaomu, Geng Xiaokun, Ding Yuchuan

机构信息

Department of Neurology, Beijing Luhe Hospital, Capital Medical University, Beijing, China.

Department of Neurosurgery, Wayne State University School of Medicine, Detroit, MI, USA.

出版信息

Brain Circ. 2022 Jun 30;8(2):102-107. doi: 10.4103/bc.bc_30_22. eCollection 2022 Apr-Jun.

Abstract

Myelin oligodendrocyte glycoprotein (MOG)-antibody-associated disease (MOGAD) is an independent inflammatory demyelinating disease. A rare phenotype of MOGAD is cerebral cortical encephalitis (CCE). This case report presents unilateral recurrent cerebral cortical encephalitis (CCE) with positive anti-MOG antibodies from a 55 year old man who was admitted with headache, fever and aphasia. This case highlights the findings of hyperintense lesions in the cortex of the right temporal gyrus with slight swelling on T2 FLAIR and anti MOG antibodies in serum (1:20) and CSF (1:80) when the patient presented again to hospital after the initial improvement with IVIG and glucocorticoids. In addition, the patient was found to have atrophy of the whole brain, especially the right temporal lobe, after becoming symptom-free with glucocorticoids. In summary, anti-MOG-associated CCE can be diagnosed with headache, fever, and seizures associated with the presence of anti-MOG antibodies. Unilateral CCE is a special clinical feature of MOGAD and cerebral atrophy can be found. Steroid therapy remains to be the standard treatment.

摘要

髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)是一种独立的炎性脱髓鞘疾病。MOGAD的一种罕见表型是大脑皮质脑炎(CCE)。本病例报告介绍了一名55岁男性的单侧复发性大脑皮质脑炎(CCE),其抗MOG抗体呈阳性,该患者因头痛、发热和失语入院。该病例突出了以下发现:患者在接受静脉注射免疫球蛋白(IVIG)和糖皮质激素初步治疗好转后再次入院时,右侧颞回皮质出现T2液体衰减反转恢复序列(FLAIR)高信号病变且伴有轻度肿胀,血清(1:20)和脑脊液(1:80)中存在抗MOG抗体。此外,该患者在使用糖皮质激素症状缓解后,发现全脑萎缩,尤其是右侧颞叶。总之,抗MOG相关的CCE可根据头痛、发热、癫痫发作以及抗MOG抗体的存在进行诊断。单侧CCE是MOGAD的一个特殊临床特征,且可发现脑萎缩。类固醇疗法仍是标准治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3bd3/9336595/e7d606a35689/BC-8-102-g001.jpg

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