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
作者
刘慧勤
崔红培
张弥兰
庞瑞
马伟锋
滕勇士
张杰文
黄月
袁丽品
李玮
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)
出处
《中华神经医学杂志》
CAS
CSCD
北大核心
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)。
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
Myelin oligodendrocyte glycoprotein antibody associated disorder
Inflammatory demyelinating
Cerebrospinal fluid
