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抗 N-甲基-D-天冬氨酸受体脑炎与髓鞘少突胶质细胞糖蛋白抗体相关脑脊髓炎共存的临床特征及管理:一例报告并文献复习

Clinical features and management of coexisting anti-N-methyl-D-aspartate receptor encephalitis and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis: a case report and review of the literature.

作者信息

Nan Di, Zhang Ying, Han Jinming, Jin Tao

机构信息

Department of Neurology and Neuroscience Center, the First Hospital of Jilin University, Xinmin Street 1#, Changchun, 130021, China.

Department of Clinical Neuroscience, Karolinska Institutet, Solna, Sweden.

出版信息

Neurol Sci. 2021 Mar;42(3):847-855. doi: 10.1007/s10072-020-04942-0. Epub 2021 Jan 7.

Abstract

BACKGROUND

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an autoimmune encephalitis caused by antibodies targeting the GluN1 subunit of NMDA receptors. Myelin oligodendrocyte glycoprotein (MOG) antibody disorders are now widely accepted as peculiar neuroimmunological diseases with specific clinical and pathological features. Some rare cases of overlapping anti-NMDA receptor encephalitis and MOG antibody-associated diseases have been reported, presenting complex clinical symptoms that make the disease more difficult to recognize.

METHOD

In accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, the terms "NMDAR" and "MOG," "NMDAR" and "demyelination," and "MOG" and "encephalitis" were searched in PubMed. Clinical cases with dual-positive anti-NMDA cerebrospinal fluid receptors and MOG serum antibodies during the disease course were included in this study.

RESULTS

A total of 25 patients were analyzed in this study. The age at onset ranged from 3 to 54 years. The median number of relapses was 2.8. Administration of intravenous methylprednisolone and immunoglobulin was the most widely used treatment strategy (19/25 patients). Second-line treatments such as administration of mycophenolate mofetil, rituximab, interferon-β, azathioprine, cyclophosphamide, and temozolomide were also reported, followed by good outcomes.

CONCLUSIONS

The rates of coexisting anti-NMDA receptor encephalitis and MOG antibody-associated encephalomyelitis may be underestimated. Clinical symptoms such as seizures and cognitive decline accompanied by atypical central nervous system demyelination serve as warning signs of possible coexisting anti-NMDA receptor encephalitis and MOG antibody-associated encephalomyelitis. These patients could achieve good outcomes under proper immunotherapies.

摘要

背景

抗N-甲基-D-天冬氨酸(NMDA)受体脑炎是一种自身免疫性脑炎,由靶向NMDA受体GluN1亚基的抗体引起。髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病现被广泛认为是具有特定临床和病理特征的特殊神经免疫性疾病。已有一些抗NMDA受体脑炎与MOG抗体相关疾病重叠的罕见病例报道,其临床表现复杂,增加了疾病的识别难度。

方法

按照系统评价和Meta分析的首选报告项目(PRISMA)指南,在PubMed中检索“NMDAR”和“MOG”、“NMDAR”和“脱髓鞘”以及“MOG”和“脑炎”等关键词。本研究纳入了病程中脑脊液抗NMDA受体和血清MOG抗体双阳性的临床病例。

结果

本研究共分析了25例患者。发病年龄为3至54岁。复发次数中位数为2.8次。静脉注射甲泼尼龙和免疫球蛋白是最常用的治疗策略(19/25例患者)。也有使用霉酚酸酯、利妥昔单抗、干扰素-β、硫唑嘌呤、环磷酰胺和替莫唑胺等二线治疗的报道,随后取得了良好疗效。

结论

抗NMDA受体脑炎与MOG抗体相关脑脊髓炎并存的发生率可能被低估。癫痫发作和认知功能下降等临床症状伴有非典型中枢神经系统脱髓鞘,是抗NMDA受体脑炎与MOG抗体相关脑脊髓炎可能并存的警示信号。这些患者在适当的免疫治疗下可取得良好疗效。

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