Dai Yuwei, Yin Qing, Han Binhong, Yuan Tianxiang, Su Kai, Dai Weiqi, Wang Dan, Yang Li
Department of Neurology, Second Xiangya Hospital, Central South University, No. 139 Middle Renmin Road, Changsha, 410011, China.
Clinical Medical Research Center for Stroke Prevention and Treatment of Hunan Province, Department of Neurology, Second Xiangya Hospital, Central South University, Changsha, 410011, China.
J Neurol. 2025 Jul 22;272(8):528. doi: 10.1007/s00415-025-13278-8.
To assess the diagnostic significance and clinical features of cerebrospinal fluid (CSF)-restricted MOG-IgG in Chinese adults with MOG-IgG-associated disease (MOGAD), and to compare phenotypes across MOG-IgG statuses and ethnicities.
Eighty-two adults with suspected inflammatory demyelinating diseases and positive MOG-IgG (serum and/or CSF) were retrospectively analyzed from 2014 to 2024. Patients were categorized into two groups based on MOG-IgG status: MOG-IgG Seropositive group (MOG-IgG Seropositive and CSF Negative) and CSF Positive group (MOG-IgG paired serum and CSF positive, CSF-restricted MOG-IgG). Clinical, radiological, and therapeutic features were evaluated. Logistic regression was used to identify predictors of poor prognosis. Literatures on CSF-restricted MOG-IgG in different ethnicities were reviewed.
Six patients (7.3%) had CSF-restricted MOG-IgG, and 83.3% met MOGAD phenotypes. Optic neuritis was the most common presentation (54.8%), followed by cerebral cortical encephalitis and acute disseminated encephalomyelitis. Visual and motor impairments at onset were more frequent in the seropositive group. Independent risk factors for EDSS ≥ 3.0 included higher nadir EDSS score (OR: 2.39), visual impairment (OR: 10.94), and brainstem dysfunction (OR: 8.75).
CSF-restricted MOG-IgG defines a distinct MOGAD subgroup, supporting its diagnostic relevance. Visual and brainstem involvement predict worse outcomes. Larger, multiethnic prospective studies are warranted to refine diagnostic thresholds and clarify the role of intrathecal antibody production.
评估脑脊液(CSF)限制性MOG-IgG在中国成人MOG-IgG相关疾病(MOGAD)中的诊断意义和临床特征,并比较不同MOG-IgG状态和种族的表型。
回顾性分析2014年至2024年82例疑似炎性脱髓鞘疾病且MOG-IgG(血清和/或脑脊液)阳性的成人患者。根据MOG-IgG状态将患者分为两组:MOG-IgG血清阳性组(MOG-IgG血清阳性且脑脊液阴性)和脑脊液阳性组(MOG-IgG配对血清和脑脊液阳性,脑脊液限制性MOG-IgG)。评估临床、影像学和治疗特征。采用逻辑回归确定预后不良的预测因素。回顾了不同种族中脑脊液限制性MOG-IgG的文献。
6例患者(7.3%)存在脑脊液限制性MOG-IgG,83.3%符合MOGAD表型。视神经炎是最常见的表现(54.8%),其次是大脑皮质脑炎和急性播散性脑脊髓炎。血清阳性组发病时视力和运动障碍更为常见。EDSS≥3.0的独立危险因素包括最低EDSS评分较高(OR:2.39)、视力障碍(OR:10.94)和脑干功能障碍(OR:8.75)。
脑脊液限制性MOG-IgG定义了一个独特的MOGAD亚组,支持其诊断相关性。视力和脑干受累预示预后较差。需要开展更大规模的多民族前瞻性研究,以完善诊断阈值并阐明鞘内抗体产生的作用。