目的：分析髓鞘少突胶质细胞糖蛋白抗体相关疾病（anti-myelin oligodendrocyte glycoprotein-IgG associated disorders, MOGAD）患者的临床和影像学表现及预后。方法：对我院2018年1月至2021年12月确诊的8例MOGAD患者的发病诱因、起病形式、临床表现及辅助检查进行分析，观察患者治疗效果及预后，并结合相关文献探讨。结果：8例MOGAD患者中，男性6例，女性2例，平均年龄为27.37岁，均为亚急性、慢性起病，临床表现以肢体无力、肢体抽搐、视物模糊常见。8例患者从出现症状到确诊的时间为2周至4年不等，首诊原因包括脊髓炎、脑炎、多发性硬化、颅内占位性病变。误诊原因与患者临床表现、影像学表现不典型，临床医师对MOGAD认识不足有关。脊髓穿刺检查示，脑脊液压力为95~225 mmH₂O，其中脑脊液压力增高3例，脑脊液中细胞数增高5例，脑脊液蛋白增高4例，而脑脊液中糖、氯化物均正常；血液、脑脊液MOG抗体阳性患者6例；脑诱发电位异常5例，脑电图异常4例；头颅MRI示受累范围较广，包括视神经、脑桥臂、丘脑、侧脑室旁、胼胝体、额叶、顶叶、枕叶、皮层下、小脑等多部位不同程度异常信号，7例为颅内病灶，仅1例患者表现为颈、胸髓长节段异常信号。8例患者均使用大剂量甲泼尼龙冲击治疗，甲泼尼龙联合丙种球蛋白治疗者3例，甲泼尼龙联合环磷酰胺治疗1例。规律治疗后，随访3年，7例患者症状缓解，1例仍有反复发作。检索中国知网、万方医学网、维普数据库、PubMed医学文献数据库，共纳入MOGAD患者161例，回顾相关文献发现，MOGAD亚急性、慢性起病多见，临床表现多样，影像学提示全脑、脊髓均可受累，容易反复，复发率为30.00%~43.39%（本研究8例患者的复发率为49.69%），对激素、免疫治疗敏感。本研究报道的MOGAD临床和影像学表现及治疗预后均与既往报道一致。结论：MOGAD以亚急性、慢性起病多见，年青患者居多，临床表型广泛，脑脊液常规检验无特异性，影像学表现全脑、脊髓均可受累。患者对激素、免疫治疗敏感，疗效及预后较好，但易复发。对疑似患者应尽早行髓鞘少突胶质细胞糖蛋白（myelin oligodendrocyte glycoprotein，MOG）抗体测定，必要时可多次检测，避免漏诊。

Objective: To analyze the clinical manifestations, imaging features and prognosis of the patients with anti-myelin oligodendrocyte glycoprotein-IgG associated disorders (MOGAD). Methods: The data of eight MOGAD patients diagnosed in our hospital from January 2018 to December 2021 was analyzed, including the pathogenic factors, onset forms, clinical manifestations and auxiliary examinations. The therapeutic effect and prognosis of the patients were observed and discussed, and the relevant literatures were reviewed. Results: Among 8 patients with MOGAD, 6 were male and 2 were female, and the average age was 27.37 years. The most common clinical manifestations were limb weakness, limb twitching and blurred vision. The spinal puncture showed that the CSF pressure of the patients were 95-225 mmH₂O. It revealed that 3 cases had increased CSF pressure, 5 cases had increased CSF cell count and 4 cases had increased CSF protein, while the CSF sugar and chloride were all normal. Six patients had positive MOG antibody in blood and cerebrospinal fluid. Five cases were found to have abnormal brain evoked potentials, and 4 cases had abnormal electroencephalogram. The brain MRI showed that the wide area of brain was affected, including different degree of abnormal signals in optic nerve, pons arm, thalamus, paraventricular, corpus callosum, frontal lobe, parietal lobe, occipital lobe, subcortical, cerebellum and other parts. Seven patients showed intracranial lesions, while only one patient showed abnormal signals in the long segment of cervical and thoracic spinal cord. All 8 patients were treated with high-dose methylprednisolone pulse therapy, in which 3 cases were given methylprednisolone combined with gamma globulin, and 1 case was treated with methylprednisolone combined with cyclophosphamide. After regular treatment, 7 patients were followed up for 3 years. The symptoms were relieved in 7 patients, while 1 patient still had recurrent attacks. By searching CNKI, CSPD，and CSTJD and PubMed Databases, 161 patients with MOGAD were enrolled. After reviewing the relevant literatures, it revealed that the subacute and chronic onset of MOGAD was common, and its clinical manifestations were diverse. The imaging study showed that the whole brain and spinal cord could be affected, and the recurrence rate was 30.00%-43.39%. The MOGAD patients were sensitive to hormone and immune therapy. The clinical and imaging manifestations and treatment prognosis of 8 MOGAD cases were consistent with previous report. Conclusions: The subacute and chronic onset of MOGAD is common, and most of the patients are young, and the patients show diverse clinical manifestations. In MOGAD patients, the routine examination of cerebrospinal fluid is non-specific, while the imaging of the whole brain and spinal cord are abnormal, and the damage area could be widely. The patients with MOGAD are sensitive to hormone and immune therapy, which presents satisfied therapeutic effects and prognosis. For the suspected patients, MOG should be performed as early as possible, and the multiple tests can be performed to avoid missed diagnosis.