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髓鞘少突胶质细胞糖蛋白抗体相关脊髓炎的临床特征

[Clinical characteristics of myelin oligodendrocyte glycoprotein antibody associated myelitis].

作者信息

Zhangbao B J Z, Zhou L, Wang L, Huang W J, Zhao C B, Lu J H, Quan C

机构信息

Department of Neurology, Huashan Hospital, Fudan University, Shanghai 200040, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2020 Feb 11;100(5):334-338. doi: 10.3760/cma.j.issn.0376-2491.2020.05.004.

Abstract

To evaluate the clinical characteristics of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) associated myelitis in a cohort of Chinese Han adults. From January 2016 to December 2017, 70 patients with MOG-IgG associated disorders (MOGAD) and 120 patients with aquaporin 4 antibody (AQP4-IgG) positive neuromyelitis optica spectrum disorders (NMOSD) visited the NMO/MS clinic or the neurology ward of Huashan Hospital, and the neurophthalmology clinic of Eye and ENT hospital, Shanghai Medical College, Fudan University were enrolled. The clinical and paraclinical data of the patients were retrospectively reviewed. The characteristics of MOG-IgG associated myelitis were further clarified. Sixteen of the 70 patients with MOGAD had ever experienced myelitis. The frequency of myelitis was 18.6% at the first attack and 22.9% throughout the disease duration. The onset age of MOG-IgG associated myelitis was 9-57(30±11) years, and the female to male ratio was 0.6∶1. Compared with AQP4-IgG positive myelitis attacks, MOG-IgG associated myelitis attacks were more common to be accompanied by feverish prodromal symptom (30.8%) while less common to exhibit painful tonic (12.5%). Longitudinally extensive myelitis (>3 vertebral segments) was less frequent (56.3%), and short-segment myelitis and multiple short-segment myelitis could also be seen. MRI showed that MOGAD patients had more lower spinal cord lesions (20%), fewer cervical cord lesions (40%) and less transverse lesions (52%). Axial H sign was a distinct feature (36%). MOG-IgG associated myelitis attack also demonstrated a lower EDSS score after treatment. MOG-IgG associated myelitis should be recognized as an important clinical component of MOGAD.

摘要

评估中国汉族成年人群中髓鞘少突胶质细胞糖蛋白抗体(MOG-IgG)相关脊髓炎的临床特征。2016年1月至2017年12月,70例MOG-IgG相关疾病(MOGAD)患者和120例水通道蛋白4抗体(AQP4-IgG)阳性的视神经脊髓炎谱系障碍(NMOSD)患者就诊于复旦大学附属眼耳鼻喉科医院神经眼科门诊、华山医院NMO/MS门诊或神经内科病房,并纳入研究。对患者的临床和副临床资料进行回顾性分析,进一步明确MOG-IgG相关脊髓炎的特征。70例MOGAD患者中有16例曾发生过脊髓炎。首次发作时脊髓炎的发生率为18.6%,疾病全程发生率为22.9%。MOG-IgG相关脊髓炎的发病年龄为9 - 57(30±11)岁,男女比例为0.6∶1。与AQP4-IgG阳性脊髓炎发作相比,MOG-IgG相关脊髓炎发作更常伴有发热前驱症状(30.8%),而较少出现痛性强直(12.5%)。纵向广泛脊髓炎(>3个椎体节段)较少见(56.3%),也可见短节段脊髓炎和多发短节段脊髓炎。MRI显示,MOGAD患者脊髓下段病变较多(20%),颈段脊髓病变较少(40%),横贯性病变较少(52%)。轴位H征是一个显著特征(36%)。MOG-IgG相关脊髓炎发作经治疗后扩展残疾状态量表(EDSS)评分也较低。MOG-IgG相关脊髓炎应被视为MOGAD的一个重要临床组成部分。

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