8例髓鞘少突胶质细胞糖蛋白抗体相关疾病患者的临床和影像学表现分析并文献复习  

Analysis of clinical and imaging manifestations in 8 patients with MOGAG and literature review

作　　者：林霞[1,2] 高超 黄沛 王刚[2] 林国珍[3] 任汝静[2] LIN Xia;GAO Chao;HUANG Pei;WANG Gang;LIN Guozhen;REN Rujing(Department of Neurology,Rujin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;Department of Psychiatry,Rujin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China;Department of Neurology,First People's Hospital of Tianshui,Gansu Province,Gansu Tianshui 741000,China)

机构地区：[1]甘肃省天水市第一人民医院神经内一科,甘肃天水741000 [2]上海交通大学医学院附属瑞金医院神经内科,上海200025 [3]上海交通大学医学院附属瑞金医院临床心理科,上海200025

出　　处：《诊断学理论与实践》2022年第5期606-612,共7页Journal of Diagnostics Concepts & Practice

基　　金：上海市医苑新星杰出青年基金(2018)。

摘　　要：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�Objective: To analyze the clinical manifestations, imaging features and prognosis of the patients with antimyelin 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 ob-served 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 for3 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 wer

关 键 词：髓鞘少突胶质细胞糖蛋白抗体相关疾病 临床特征 脑脊液 脑电图 脑诱发电位 头颅磁共振 

分 类 号：R744.5[医药卫生—神经病学与精神病学]