抗髓鞘少突胶质细胞糖蛋白G抗体相关疾病8例临床特点及康复结局研究

A study of clinical characteristic and rehabilitation outcome in an eight-case series with anti-myelin oligodendrocyte glycoprotein IgG associated disorders

目的探讨抗髓鞘少突胶质细胞糖蛋白G抗体(MOG-IgG)相关疾病(MOGAD)的临床特点和预后。方法回顾8例符合MOGAD诊断标准的病例,分析其人口统计学、临床表现、实验室及影像学检查、治疗及预后等情况。采用扩展的残疾状态量表(EDSS)和改良Rankin量表(mRS)对患者神经功能残疾进行动态评估。结果8例患者中男女比例1∶1,中位起病年龄34岁,中位病程42 m。最常见首发症状是视神经炎(ON,占50%),其次是脑膜/脑炎(25%)。50%的病例出现复发,血清MOG-IgG滴度高者(≥1:32)复发率60%。脑膜/脑炎或长节段脊髓炎者其脑脊液白细胞数常>100×106/L,高于其它表型。有6例(75%)患者出现不同的MRI异常,包括视神经及眶内组织T2/Flair高信号伴强化,无症状的间脑/丘脑T2及强化病灶,也可出现类似多发性硬化的典型MRI特征。脊髓炎病例在MRI上可呈短节段偏心病灶、亦可出现长节段中心病灶。脑膜/脑炎病例在颅脑MRI上呈单侧的皮质高T2/Flair病灶,强化不一。经免疫调节药物治疗及康复治疗,该病例组高峰期EDSS为(5±1.069)分,末访时为(2.19±1.689)分;mRS在高峰时(3.25±1.165)分,末访时为(1.25±1.035)分,差异均有统计学意义。结论MOGAD是一类罕见的、表现多样的中枢神经脱髓鞘疾病,该病可治疗性很强。充分认识该病的临床特点,尽早综合检查检验资料作出诊断,及时启动免疫及康复治疗,大多数患者可以获得较佳结局。

Objective To investigate the clinical features and prognosis of anti-myelin oligodendrocyte glycoprotein-IgG(MOG-IgG)associated disorders(MOGAD).Methods Eight cases met the diagnostic criteria of MOGAD were reviewed,and the demographics,clinical manifestations,laboratory and imaging results,treatment and prognosis were analyzed.The Expanded Disability Status Scale(EDSS)and the modified Rankin Scale(mRS)were used to dynamically evaluate the neurological disability.Results The ratio of male to female in this 8-case series was 1∶1,the median onset age was 34 years old,and the median disease duration was 42 months.The most common initial symptom is optic neuritis(ON,50%),followed by meningitis and/or encephalitis(25%).Recurrence occurs in 50%of cases,and the recurrence rate of patients with high serum MOG-IgG titer(≥1∶32)is 60%.The white blood cells in the cerebrospinal fluid in cases with meningitis/encephalitis or longitudinally extensive myelitis is often>100×106/L,which is higher than other phenotypes.There are 6 cases(75%)of patients with different MRI abnormalities,including hypertensity on T2/Flair image with enhancement at affected optic nerve and orbital tissue,asymptomatic diencephalon/thalamus lesions on T2 and contrasted image,and MRI findings mimic typical multiple sclerosis may also be present.Cases with myelitis can show short-segment eccentric lesions or long-segment central lesions on MRI.Cases with meningitis/encephalitis showed unilateral cortical high T2/Flair lesions on cranial MRI with various enhancements.After medication and rehabilitation,the peak EDSS of this case series was 5±1.069,and 2.19±1.689 points at the last visit;mRS was 3.25±1.165 points at the peak and 1.25±1.035 points at the last visit.The difference was statistically significant.Conclusions MOGAD is a rare type of demyelinating disease in central nervous with various manifestations,which is highly treatable.Fully understanding of the clinical characteristics,making a early diagnosis through comprehensive tests,and initiating immunization and rehabilitation treatment in time,will contribute to better prognosis for patients.