Duan Tianjiao, Ouyang Song, Hu Zhaolan, Zeng Qiuming, Yin Weifan
Department of Neurology, the Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Clinical Medical Research Center for Stroke Prevention and Treatment of Hunan Province, Department of Neurology, the Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Eur J Neurosci. 2025 Jan;61(1):e16654. doi: 10.1111/ejn.16654.
To summarise the clinical characteristics, radiological features, treatments and prognosis of patients with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) overlapped with NMDA receptor (NMDAR) encephalitis. We retrospectively analysed patients who exhibited dual positivity for MOG antibodies and NMDAR antibodies in serum/CSF from Jan 2018 to Jun 2023. Ten patients with MOGAD and NMDAR encephalitis were enrolled. The median age of initial attacks was 23 (range: 10-43) years old. Common symptoms were cortical encephalopathies (8/10), focal neurological deficits (4/10), as well as other presentations including headache, fever, optic neuritis and transverse myelitis. CSF pleocytosis was general (9/10, median 63.9 cells/μl). Lesions on brain MRI included brainstem (37.5%), cerebral cortex (33.3%), basal ganglia (25.0%) and hippocampus (20.8%). The average follow-up duration was 25.4 months. 10/10 patients developed more than one relapse attacks, with MOG positivity before (10%), simultaneous (40%) or after anti-NMDAR encephalitis (50%). Most patients (7/10) had good response to first-line therapy but experienced next relapse with an average interval of 6.7 (range: 2-14) months. We conducted initial analysis of lymphocyte subsets in these patients, which revealed that CD3+ and CD4 + T cells increased after immunosuppressants medication (p < 0.01 and p < 0.05, respectively). We concluded that MOGAD overlapping with NMDAR encephalitis presents a distinct clinical phenotype which differs from either MOGAD or NMDAR encephalitis. Brainstem in combination with cortical lesions might be warning signs for this overlapping syndrome. Due to the high recurrent rates, we recommend early diagnosis and timely treatment with efficient immunosuppressants at onset.
总结髓鞘少突胶质细胞糖蛋白（MOG）抗体相关疾病（MOGAD）与N-甲基-D-天冬氨酸受体（NMDAR）脑炎重叠患者的临床特征、影像学特征、治疗方法及预后。我们回顾性分析了2018年1月至2023年6月期间血清/脑脊液中MOG抗体和NMDAR抗体呈双重阳性的患者。纳入了10例MOGAD合并NMDAR脑炎患者。首次发病的中位年龄为23岁（范围：10 - 43岁）。常见症状为皮质脑病（8/10）、局灶性神经功能缺损（4/10），以及包括头痛、发热、视神经炎和横贯性脊髓炎在内的其他表现。脑脊液细胞增多较为常见（9/10，中位值63.9个细胞/μl）。脑部MRI病变包括脑干（37.5%）、大脑皮质（33.3%）、基底节（25.0%）和海马（20.8%）。平均随访时间为25.4个月。10/10患者出现不止一次复发发作，MOG阳性出现在抗NMDAR脑炎之前（10%）、同时（40%）或之后（50%）。大多数患者（7/10）对一线治疗反应良好，但平均间隔6.7个月（范围：2 - 14个月）出现下一次复发。我们对这些患者的淋巴细胞亚群进行了初步分析，结果显示免疫抑制剂治疗后CD3 +和CD4 + T细胞增加（分别为p < 0.01和p < 0.05）。我们得出结论，MOGAD与NMDAR脑炎重叠呈现出一种独特的临床表型，与MOGAD或NMDAR脑炎均不同。脑干合并皮质病变可能是这种重叠综合征的警示信号。由于复发率高，我们建议早期诊断并在发病时及时使用有效的免疫抑制剂进行治疗。
