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Yuwei Dai^1,2,3, Yu Yuan^1,4, Fangfang Bi^1,4

¹Department of Neurology, Xiangya Hospital, Central South University, Changsha, 410008, Hunan Province, China.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology

|March 25, 2024

概括

患有同时存在N-甲基-D-酸盐受体抗体 (NMDAR-IgG) 和髓寡干细胞糖蛋白抗体 (MOG-IgG) 的成年人表现出类似于NMDAR-IgG脑炎的症状,但MRI发现与MOG-IgG相关疾病重叠. 建议在非典型脑炎病例中对两种抗体进行检测.

科学领域:

神经免疫学神经免疫学
神经学神经学
自身免疫性脑炎是什么?

背景情况:

通过N-甲基-D-酸盐受体抗体 (NMDAR-IgG) 和髓寡干细胞糖蛋白抗体 (MOG-IgG) 介导的疾病是不同的自身免疫神经系统疾病.
在成年患者中,NMDAR-IgG和MOG-IgG的共存是罕见的,需要对其独特的临床和放射性资料进行分析.

研究的目的:

分析与NMDAR-IgG和MOG-IgG同时存在的成年患者的临床和放射特征.
将这些特征与患有孤立NMDAR-IgG脑炎或MOG-IgG相关疾病 (MOG-AD) 的患者进行比较.

主要方法:

从雅医院 (2017年6月至2021年12月) 对11名患有NMDAR-IgG和MOG-IgG并存的成年患者的回顾性分析.
与55名抗NMDAR脑炎患者和49名MOG-AD患者进行比较.
评估临床症状,发病率,精神状态和磁共振成像 (MRI) 发现,包括T2/FLAIR超强度分布.

主要成果:

同时存在的NMDAR-IgG/MOG-IgG组呈现出突出的发作和精神病症状,发作的平均年龄为27岁.
在90.9%的患者中,MRI显示T2/FLAIR超强度主要在皮质,脑干和视神经.
与单独使用NMDAR-IgG相比,与NMDAR-IgG共存的组显示出更多的动力衰竭,脑干和视神经损伤. 与单独使用MOG-IgG相比,他们有更多的发作和精神症状.

结论:

与NMDAR-IgG和MOG-IgG同时存在的患者表现出更类似于NMDAR-IgG脑炎的临床症状,但MRI模式类似于MOG-AD.
同时存在的组在随访时显示出更高的皮质类固醇治疗,复发的倾向,以及更低的修改Rankin尺度得分.
针对NMDAR-IgG和MOG-IgG的测试对于表现为异型脑炎症状和内脱髓化病变的成年患者至关重要.

关键词:

抗NMDAR脑炎的治疗方法这就是MOG-ab.与MOG相关的疾病叠加性脱髓化综合征的发生

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