髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床、影像及预后特点  

Clinical and imaging features and prognoses of myelin oligodendrocyte glycoprotein antibody associated disorders

作　　者：刘慧勤[1] 崔红培 张弥兰[1] 庞瑞 马伟锋 滕勇士 张杰文[1] 黄月[1] 袁丽品[1] 李玮[1] Liu Huiqin;Cui Hongpei;Zhang Milan;Pang Rui;Ma Weifeng;Teng Yongshi;Zhang Jiewen;Huang Yue;Yuan Lipin;Li Wei(Department of Neurology,Henan Provincial People's Hospital,Zhengzhou 450003,China;Department of Ophthalmology,Henan Provincial People's Hospital,Zhengzhou 450003,China)

机构地区：[1]河南省人民医院神经内科,郑州450003 [2]河南省人民医院眼科,郑州450003

出　　处：《中华神经医学杂志》2022年第2期164-171,共8页Chinese Journal of Neuromedicine

基　　金：河南省科技发展计划项目(212102310833);河南省医学科技攻关计划联合共建项目(LHGJ20200027)。

摘　　要：目的探讨髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的临床、影像特点及预后。方法选择河南省人民医院神经内科自2018年1月至2021年4月收治的39例MOGAD患者进入研究,收集患者急性发作期(首次发作或复发)临床、影像学资料及随访结果并分析其特点。结果39例MOGAD患者中20例(51.3%)为单向病程,19例(48.7%)为复发病程。共收集到39名患者55次发作的临床及影像学资料,其中临床表型包括:视神经炎27次(49.1%),脑炎10次(18.2%),脑干脑炎8次(14.5%),脑膜脑炎2次(14.5%),脊髓炎3次(5.5%),脑脊髓炎1次(1.8%),视神经脊髓炎1次(1.8%),视神经炎+脑膜脑炎2次(3.6%),视神经炎+脑炎1次(1.8%)。实验室检查结果:36例患者中4例(11.1%)抗核抗体(ANA)阳性。22例患者28次脑脊液检查结果中,19次(67.9%)脑脊液白细胞升高,数值为(54.89±67.70)×10^(6)/L。影像学表现:收集到19例患者27次急性发作期的头颅MRI,1次MRI完全正常;余26次MRI中单个病灶6次,2个病灶5次,3个及以上病灶15次。分布范围方面,脑干及其邻近结构9次,间脑及深部灰质7次,幕上白质13次,皮层病灶13次。4次头颅增强MRI可见脑膜强化(4/20)。脊髓病变可表现为长节段或短节段脊髓炎,颈、胸、圆锥均可受累,横断面可见H征。所有患者均在急性期接受激素治疗并序贯减量。治疗3个月后38例(97.4%)患者预后良好(改良Rankin量表评分≤2分)。结论MOGAD是一类广泛累及中枢神经系统白质、灰质及脑膜的疾病,临床表现为视神经炎、脑炎、脑干脑炎、脑膜脑炎、脊髓炎或以上组合,多数患者免疫治疗有效,但复发率高,部分患者需长程免疫治疗。Objective To explore the clinical and imaging features and prognoses of myelin oligodendrocyte glycoprotein antibody associated disorders(MOGAD).Methods Thirty-nine MOGAD patients,admitted to our hospital from January 2018 to April 2021,were chosen in our study.The clinical and imaging data and follow-up results of these patients at acute attack period(first-onset or relapse)were collected and their features were analyzed.Results In these 39 patients with MOGAD,20 patients(51.3%)had non-reversing course,and 19 patients(48.7%)had relapsing course.The clinical and imaging data of 55 episodes of these 39 patients were collected.In these 55 episodes,optic neuritis was noted in 27 episodes(49.1%),encephalitis was noted in 10 episodes(18.2%),brainstem encephalitis was noted in 8 episodes(14.5%),meningoencephalitis in 2 episodes(14.5%),myelitis in 3 episodes(5.5%),encephalomyelitis in 1 episode(1.8%),optic neuromyelitis in 1 episode(1.8%),optic neuritis+meningoencephalitis in 2 episodes(3.6%),and optic neuritis+encephalitis in 1 episode(1.8%).The positive rate of antinuclear antibody(ANA)was 11.1%(4/36);the cerebrospinal fluid results of 28 samples were collected from 22 patients,and CSF pleocytosis occurred in 67.9%of the samples with value of 54.89±67.70×10^(6)/L.Twenty-seven brain MRIs of 19 patients at the acute episode were collected;one completely normal MRI was recorded;among the remaining 26 MRIs,6 were with one single lesion,5 were with 2 lesions,and 15 were with 3 or more lesions;in terms of distribution,lesions involving brainstem and its adjacent structures were found in 9 MRIs,lesions involving diencephalon and deep gray matter were found in 7 MRIs,supratentorial white matter lesions were found in 13 MRIs,and cortical lesions were found in 13 MRIs.Meningeal enhancement were found in 4 contrast-enhanced brain MRIs(4/20).Long or short segmental myelitis in the spinal MRIs was noted in spinal lesions,involving cervical spinal cord,thoracic spinal cord and conus,and the"H"sign could be seen in the cross secti

关 键 词：髓鞘少突胶质细胞糖蛋白抗体相关疾病 炎性脱髓鞘疾病 脑脊液 

分 类 号：R744.5[医药卫生—神经病学与精神病学]