MOG抗体相关疾病与抗nmda受体脑炎重叠的临床分析:一项长期回顾性研究

IF 2.7 4区 医学 Q3 NEUROSCIENCES European Journal of Neuroscience Pub Date : 2025-01-01 DOI:10.1111/ejn.16654
Tianjiao Duan, Song Ouyang, Zhaolan Hu, Qiuming Zeng, Weifan Yin
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引用次数: 0

摘要

目的总结髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病(MOGAD)与NMDA受体(NMDAR)脑炎重叠的临床特点、影像学特点、治疗及预后。我们回顾性分析了2018年1月至2023年6月血清/CSF中MOG抗体和NMDAR抗体双重阳性的患者。纳入10例MOGAD和NMDAR脑炎患者。初次发病的中位年龄为23岁(范围:10-43岁)。常见症状为皮质性脑病(8/10),局灶性神经功能缺损(4/10),以及头痛、发热、视神经炎和横脊髓炎等其他表现。脑脊液多细胞增生一般(9/10,中位63.9个细胞/μl)。脑MRI病变包括脑干(37.5%)、大脑皮层(33.3%)、基底节区(25.0%)和海马(20.8%)。平均随访时间为25.4个月。10/10患者出现一次以上复发,MOG阳性在抗nmdar脑炎前(10%),同时(40%)或之后(50%)。大多数患者(7/10)对一线治疗反应良好,但再次复发的平均间隔时间为6.7个月(范围:2-14个月)。我们对这些患者的淋巴细胞亚群进行了初步分析,发现免疫抑制剂治疗后CD3+和CD4 + T细胞增加(p
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Clinical Analysis of MOG Antibody-Associated Disease Overlapped With Anti-NMDA Receptor Encephalitis: A Long-Term Retrospective Study.

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.

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来源期刊
European Journal of Neuroscience
European Journal of Neuroscience 医学-神经科学
CiteScore
7.10
自引率
5.90%
发文量
305
审稿时长
3.5 months
期刊介绍: EJN is the journal of FENS and supports the international neuroscientific community by publishing original high quality research articles and reviews in all fields of neuroscience. In addition, to engage with issues that are of interest to the science community, we also publish Editorials, Meetings Reports and Neuro-Opinions on topics that are of current interest in the fields of neuroscience research and training in science. We have recently established a series of ‘Profiles of Women in Neuroscience’. Our goal is to provide a vehicle for publications that further the understanding of the structure and function of the nervous system in both health and disease and to provide a vehicle to engage the neuroscience community. As the official journal of FENS, profits from the journal are re-invested in the neuroscientific community through the activities of FENS.
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