抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病的临床特征分析  

Clinical features of myelin oligodendrocyte glycoprotein-IgG-associated disorders

作　　者：刘曌 王丽君 万东君 LIU Zhao;WANG Lijun;WAN Dongjun(Department of Neurology,The 940 th Hospital of Joint Logistics Support Force of People’s Liberation Army,Lanzhou 730050,China)

机构地区：[1]解放军联勤保障部队第940医院神经内科,甘肃兰州730050

出　　处：《中风与神经疾病杂志》2025年第3期233-238,共6页Journal of Apoplexy and Nervous Diseases

基　　金：甘肃省卫生行业科研计划项目(GSWSKY-2019-64);甘肃省青年科技基金计划项目(20JR5RA590);联勤保障部队第九四〇医院专项培育项目(2021yxky018)。

摘　　要：目的探讨抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病(MOGAD)的临床特征。方法回顾性分析解放军联勤保障部队第940医院神经内科收治的9例MOGAD的临床资料。结果9例MOGAD患者(5例男性,4例女性),中位发病年龄29.0岁。主要临床症状包括:视神经炎5例、大脑症状(含精神症状)5例、癫痫发作4例、脑干症状3例、锥体束征3例、小脑症状3例、直肠/膀胱功能症状3例、脊髓炎2例、感觉异常2例、合并自身免疫性脑炎1例。患者血清MOG-IgG均阳性,脑脊液MOG-IgG阳性2例。MRI显示脑部病变多累及中线结构和深部灰质,其中中脑、脑桥、延髓4例(44.4%),第四脑室4例(44.4%);脊髓病变为长节段(≥3个节段),其中颈段脊髓2例,胸段脊髓1例。9例患者在急性期均接受了免疫治疗,其神经功能评估采用扩展残疾状态量表(EDSS)评分平均值为(3.94±2.28)。随访(中位时间:36.0个月)中发现3例复发(2例为脊髓炎)。结论MOGAD的患病率无明显性别差异,发病年龄早于多发性硬化(MS)和视神经脊髓炎谱系疾病(NMOSD);视神经炎是最主要的临床表型;有脊髓炎表型的MOGAD患者均为脊髓纵向长节段病变。MOGAD不同的临床表型可能与复发率相关。Objective To investigate the clinical features of myelin oligodendrocyte glycoprotein-IgG-associated disorders(MOGAD).Methods A retrospective analysis was performed for the clinical data of nine patients with MOGAD who were admitted to our department.Results Among the nine patients with MOGAD,there were five male patients and four female patients,with a median age of onset of 29.0 years.The main clinical symptoms included optic neuritis in five patients,cerebral symptoms(including mental symptoms)in five patients,seizures in four patients,brainstem symptoms in three patients,pyramidal signs in three patients,cerebellar symptoms in three patients,rectal and bladder dysfunction in three patients,myelitis in two patients,paresthesia in two patients,and comorbidity with autoimmune encephalitis in one patient.All patients tested positive for serum MOG-IgG,and two patients tested positive for MOG-IgG in cerebrospinal fluid.MRI showed that cerebral lesions mainly involved the midline structure and deep gray matter,and there were four patients(44.4%)with lesions in the midbrain,the pons,and the medulla oblongata and four patients(44.4%)with lesions in the fourth ventricle;spi⁃nal lesions often involved long segments(≥3 segments),and there were two patients with lesions in cervical spinal cord and one patient with lesions in the thoracic spinal cord.All nine patients received immunotherapy in the acute stage,with a mean Expanded Disability Status Scale score of(3.94±2.28)for neurological function assessment.Three patients experienced recurrence during the median follow-up time of 36.0 months,among whom two patients had myelitis.Conclusion There was no significant sex difference in the prevalence rate of MOGAD,and the age of onset of MOGAD is earlier than that of multiple sclerosis and neuromyelitis optica spectrum disorder.Optic neuritis is the most important clinical phenotype of MOGAD,and MOGAD patients with myelitis phenotype all have longitudinal long-segment lesions of the spinal cord.The different clinical phen

关 键 词：MOG抗体相关疾病 中枢神经系统脱髓鞘 临床表型 预后 

分 类 号：R744.5[医药卫生—神经病学与精神病学]