Clinical characteristics of myelin oligodendrocyte glycoprotein antibody-associated cortical encephalitis

doi:10.3969/j.issn.1672-6731.2025.12.006

Abstract

Abstract:

Objective: To explore clinical characteristics of myelin oligodendrocyte glycoprotein (MOG) antibody-associated cortical encephalitis. Methods and Results: Retrospective analysis was conducted on clinical data of 8 patients diagnosed with MOG antibody-associated cortical encephalitis in Tianjin Huanhu Hospital from January 2021 to January 2025. The male-to-female ratio was 1∶1, with a mean age of (32.13 ± 13.21) years old. The initial symptoms were primarily headache, fever and epileptic seizures. All patients tested positive for serum anti-MOG antibodies. Cerebrospinal fluid (CSF) analysis showed elevated pressure in 6 cases, and an increased white blood cell count in 6 cases. No definitive evidence of neoplasms was identified in all patient. MRI showed unilateral (5 cases) or bilateral (3 cases) diffuse cortical FLAIR hyperintensity in 8 patients. The lesions involved the frontal lobes in 6 cases, temporal lobes in 6 cases, parietal lobes in 7 cases, and occipital lobes in 2 cases. All patients improved after treatment with glucocorticoid or intravenous immunoglobulin (IVIg). During follow-up, the relapse occurred in 3 patients, who subsequently recovered well after retreatment with glucocorticoid or IVIg, and experienced no further recurrence. Conclusions: The clinical manifestations of MOG antibody-associated cortical encephalitis are non-specific. Head MRI shows unilateral or bilateral cortical abnormal signals, which are often misdiagnosed as viral encephalitis. Glucocorticoid is effective in combination with immunotherapy. Although some patients may experience recurrence, the symptoms of recurrence are mild. For patients with cortical encephalitis who have poor response to anti-infection treatment, it is necessary to be vigilant about MOG antibody-associated cortical encephalitis, identify it early and provide combination hormone and immunotherapy in a timely manner.

Key words: Myelin-oligodendrocyte glycoprotein, Autoantibodies, Encephalitis, Cerebral cortex, Glucocorticoids, Immunotherapy

摘要：

目的: 总结抗髓鞘少突胶质细胞糖蛋白（MOG）抗体相关皮质脑炎的临床特征。方法与结果: 回顾2021年1月至2025年1月天津市环湖医院收治的8例抗MOG抗体相关皮质脑炎患者的诊断与治疗过程，男女比例1∶1，平均年龄（32.13 ± 13.21）岁；首发症状以头痛、发热和癫痫发作为主；血清抗MOG抗体均呈阳性，6例脑脊液压力升高、6例脑脊液白细胞计数增加，均未发现明确的肿瘤证据；头部MRI表现为单侧（5例）或双侧（3例）大脑皮质弥漫性FLAIR高信号，病变累及额叶（6例）、颞叶（6例）、顶叶（7例）、枕叶（2例）；均经糖皮质激素或联合静脉注射免疫球蛋白治疗后好转，3例复发，再次经糖皮质激素或联合静脉注射免疫球蛋白治疗后好转，未再复发。结论: 抗MOG抗体相关皮质脑炎临床表现无特异性，头部MRI表现为单侧或双侧大脑皮质弥漫性异常信号，易误诊为病毒性脑炎；激素联合免疫治疗有效，仍有部分患者复发但症状较轻微。对于抗感染治疗效果欠佳的皮质脑炎患者，应警惕抗MOG抗体相关皮质脑炎，早期诊断，及时予以激素联合免疫治疗。

关键词: 髓鞘少突胶质细胞糖蛋白, 自身抗体, 脑炎, 大脑皮质, 糖皮质激素类, 免疫疗法

Zhi-hong ZHAO, Zi-kai XIN, Ya-qi LI, Yu-meng JIANG, Zi-long ZHU. Clinical characteristics of myelin oligodendrocyte glycoprotein antibody-associated cortical encephalitis[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2025, 25(12): 1128-1135.

赵志红, 辛子凯, 李雅琪, 蒋雨萌, 朱子龙. 抗髓鞘少突胶质细胞糖蛋白抗体相关皮质脑炎临床特征分析[J]. 中国现代神经疾病杂志, 2025, 25(12): 1128-1135.

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URL: https://journal11.magtechjournal.com/cjcnn/EN/10.3969/j.issn.1672-6731.2025.12.006

https://journal11.magtechjournal.com/cjcnn/EN/Y2025/V25/I12/1128

Figures/Tables 5

References 32

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序号	性别	年龄（岁）	病程（d）	临床症状	实验室检查		影像学检查	治疗	转归
序号	性别	年龄（岁）	病程（d）	临床症状	血清抗MOG抗体	脑脊液	影像学检查	治疗	转归
1	女性	57	16	头痛、发热、言语表达及理解费力、行走不稳、右眼视物不清	1∶100	无异常，抗MOG抗体阴性	左侧基底节区点状DWI高信号，左侧颞顶枕叶皮质FLAIR稍高信号伴强化	初始予阿司匹林、阿托伐他汀治疗；确诊后予甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙500 mg/d（× 3 d）、250 mg（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持6个月停药	无复发
2	男性	33	27	发热、癫痫发作	1∶ 10	压力160 mm H₂O，白细胞计数30 ×10⁶/L，抗MOG抗体阴性	双侧额顶叶大脑镰旁皮质FLAIR信号增高，脑沟内FLAIR高信号	甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙250 mg/d（× 3 d），后为甲泼尼龙60 mg/d口服，每2周减量4 mg，减量至4 mg/d维持7个月停药	无复发
3	男性	20	14	头痛、发热、肢体乏力、嗜睡	1∶100	压力330 mm H₂O，白细胞计数450 ×10⁶/L，抗MOG抗体滴度1∶1	左侧额颞叶、右侧额叶脑沟略变浅，脑沟内FLAIR稍高信号伴轻度线样强化	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗、500 mg（× 3 d），后为甲泼尼龙60 mg/d口服，每2周减量4 mg，减至4 mg/d维持7个月后停药	无复发
4	男性	44	13	发作性头晕头痛、肢体乏力，癫痫发作	1∶ 10	压力310 mm H₂O，白细胞计数126 ×10⁶/L，抗MOG抗体阴性	双侧额顶叶皮质及脑沟内FLAIR稍高信号，未见强化改变	脑卒中样发病早期予静脉溶栓；确诊后予甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗、250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每2周减量4 mg，减至4 mg/d维持6个月后停药	无复发
5	女性	31	10	头痛、发热	1∶ 10	压力200 mm H₂O，白细胞计数360 ×10⁶/L，抗MOG抗体阴性	左侧额颞顶枕叶肿胀呈FLAIR高信号，相应区域脑沟内线样FLAIR高信号，未见强化改变	初始予更昔洛韦抗病毒治疗；确诊后予地塞米松10 mg/d（× 2 d）静脉滴注，后为甲泼尼龙40 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减至8 mg/d维持6个月停药	10个月后复发，予以甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗联合吗替麦考酚酯500 mg/次（2次/d）口服1年，甲泼尼龙250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量8 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持6个月停药
6	女性	32	9	癫痫发作	1∶ 10	压力300 mm H₂O，白细胞计数4 ×10⁶/L，抗MOG抗体滴度1∶1	右侧额颞顶叶和岛叶脑沟内FLAIR高信号伴明显强化	甲泼尼龙80 mg/d（× 5 d）静脉滴注治疗，后为甲泼尼龙40 mg/d口服，每周减量4 mg，减至32 mg/d时每2周减量4 mg，减至4 mg/d维持6个月后停药	无复发
7	男性	23	15	头痛、发热、行为异常、反应迟钝、发作性言语不清、表达困难	1∶100	压力190 mm H₂O，白细胞计数100 ×10⁶/L，抗MOG抗体滴度1∶1	左侧颞枕顶叶脑沟内FLAIR高信号，相应区域脑实质略肿胀	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙1000 mg/d（× 5 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙500 mg/d（× 3 d）、250 mg/d（×3 d），后为甲泼尼龙48 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减至8 mg/d维持1年停药	1年8个月后复发，予甲泼尼龙1000 mg/d（× 5 d）静脉滴注冲击治疗联合利妥昔单抗500 mg静脉滴注（仅1次），后为甲泼尼龙48 mg/d口服，每2周减量4 mg，6个月后停药
8	女性	17	26	头痛、发热、癫痫发作、肢体乏力	1∶100	压力280 mm H₂O，白细胞计数20 ×10⁶/L，抗MOG抗体滴度1∶1	左侧额颞顶叶皮质FLAIR高信号伴线样强化，脑回肿胀、脑沟变浅	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗，250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量8 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持1年停药	1年2个月后复发，予IVIg 0.40 g/（kg·d）5 d，口服甲泼尼龙8 mg/d和吗替麦考酚酯1 g/次（2次/d），6个月后停药

序号	性别	年龄（岁）	病程（d）	临床症状	实验室检查		影像学检查	治疗	转归
序号	性别	年龄（岁）	病程（d）	临床症状	血清抗MOG抗体	脑脊液	影像学检查	治疗	转归
1	女性	57	16	头痛、发热、言语表达及理解费力、行走不稳、右眼视物不清	1∶100	无异常，抗MOG抗体阴性	左侧基底节区点状DWI高信号，左侧颞顶枕叶皮质FLAIR稍高信号伴强化	初始予阿司匹林、阿托伐他汀治疗；确诊后予甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙500 mg/d（× 3 d）、250 mg（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持6个月停药	无复发
2	男性	33	27	发热、癫痫发作	1∶ 10	压力160 mm H₂O，白细胞计数30 ×10⁶/L，抗MOG抗体阴性	双侧额顶叶大脑镰旁皮质FLAIR信号增高，脑沟内FLAIR高信号	甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙250 mg/d（× 3 d），后为甲泼尼龙60 mg/d口服，每2周减量4 mg，减量至4 mg/d维持7个月停药	无复发
3	男性	20	14	头痛、发热、肢体乏力、嗜睡	1∶100	压力330 mm H₂O，白细胞计数450 ×10⁶/L，抗MOG抗体滴度1∶1	左侧额颞叶、右侧额叶脑沟略变浅，脑沟内FLAIR稍高信号伴轻度线样强化	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙1000 mg/d（× 3 d）静脉滴注冲击治疗、500 mg（× 3 d），后为甲泼尼龙60 mg/d口服，每2周减量4 mg，减至4 mg/d维持7个月后停药	无复发
4	男性	44	13	发作性头晕头痛、肢体乏力，癫痫发作	1∶ 10	压力310 mm H₂O，白细胞计数126 ×10⁶/L，抗MOG抗体阴性	双侧额顶叶皮质及脑沟内FLAIR稍高信号，未见强化改变	脑卒中样发病早期予静脉溶栓；确诊后予甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗、250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每2周减量4 mg，减至4 mg/d维持6个月后停药	无复发
5	女性	31	10	头痛、发热	1∶ 10	压力200 mm H₂O，白细胞计数360 ×10⁶/L，抗MOG抗体阴性	左侧额颞顶枕叶肿胀呈FLAIR高信号，相应区域脑沟内线样FLAIR高信号，未见强化改变	初始予更昔洛韦抗病毒治疗；确诊后予地塞米松10 mg/d（× 2 d）静脉滴注，后为甲泼尼龙40 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减至8 mg/d维持6个月停药	10个月后复发，予以甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗联合吗替麦考酚酯500 mg/次（2次/d）口服1年，甲泼尼龙250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量8 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持6个月停药
6	女性	32	9	癫痫发作	1∶ 10	压力300 mm H₂O，白细胞计数4 ×10⁶/L，抗MOG抗体滴度1∶1	右侧额颞顶叶和岛叶脑沟内FLAIR高信号伴明显强化	甲泼尼龙80 mg/d（× 5 d）静脉滴注治疗，后为甲泼尼龙40 mg/d口服，每周减量4 mg，减至32 mg/d时每2周减量4 mg，减至4 mg/d维持6个月后停药	无复发
7	男性	23	15	头痛、发热、行为异常、反应迟钝、发作性言语不清、表达困难	1∶100	压力190 mm H₂O，白细胞计数100 ×10⁶/L，抗MOG抗体滴度1∶1	左侧颞枕顶叶脑沟内FLAIR高信号，相应区域脑实质略肿胀	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙1000 mg/d（× 5 d）静脉滴注冲击治疗联合IVIg 0.40 g/（kg·d）5 d，甲泼尼龙500 mg/d（× 3 d）、250 mg/d（×3 d），后为甲泼尼龙48 mg/d口服，每周减量4 mg，减至24 mg/d时每2周减量4 mg，减至8 mg/d维持1年停药	1年8个月后复发，予甲泼尼龙1000 mg/d（× 5 d）静脉滴注冲击治疗联合利妥昔单抗500 mg静脉滴注（仅1次），后为甲泼尼龙48 mg/d口服，每2周减量4 mg，6个月后停药
8	女性	17	26	头痛、发热、癫痫发作、肢体乏力	1∶100	压力280 mm H₂O，白细胞计数20 ×10⁶/L，抗MOG抗体滴度1∶1	左侧额颞顶叶皮质FLAIR高信号伴线样强化，脑回肿胀、脑沟变浅	初始予更昔洛韦抗病毒治疗；确诊后予甲泼尼龙500 mg/d（× 3 d）静脉滴注冲击治疗，250 mg/d（× 3 d），后为甲泼尼龙48 mg/d口服，每周减量8 mg，减至24 mg/d时每2周减量4 mg，减量至8 mg/d维持1年停药	1年2个月后复发，予IVIg 0.40 g/（kg·d）5 d，口服甲泼尼龙8 mg/d和吗替麦考酚酯1 g/次（2次/d），6个月后停药

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