抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病临床及影像特征

doi:10.6040/j.issn.1671-7554.0.2021.1149

摘要/Abstract

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)的临床及影像学特征。方法选取2018年9月至2021年6月在山东第一医科大学附属省立医院确诊的MOGAD的临床及影像学资料,进行回顾性总结分析。结果 19例MOGAD患者中男9例,女10例,中位年龄10岁。患者临床表型为视神经脊髓炎谱系疾病者7例,急性播散性脑脊髓炎者6例,脑炎者4例［其中1例合并抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎］,脊髓炎者1例,脑干脑炎者1例。17例(17/19)患者颅脑磁共振成像(MRI)检查发现异常信号,病变可累及脑组织任何部位,表现为多灶性、边界不清的点片状T₂WI高信号。4例(4/13)患者脊柱MRI检查发现脊髓异常信号。4例(4/9)患者眼眶MRI检查发现异常信号,表现为视神经T₂WI高信号;2例病变累及视交叉、视束。19例患者血清 MOG-IgG 抗体均为阳性,其中1例合并NMDAR抗体阳性,1例合并甲状腺过氧化物酶抗体阳性。结论 MOGAD多见于儿童青少年,临床表型多样且与年龄相关。病变可累及视神经、脊髓、颅脑等多个部位,影像学表现多样。

关键词: 髓鞘少突胶质细胞糖蛋白抗体, 视神经脊髓炎谱系疾病, 多发性硬化, 磁共振成像

Abstract: Objective To investigate the clinical and imaging features of anti-myelin oligodendrocyte glycoprotein-IgG antibody associated disorders(MOGAD). Methods The clinical and imaging data of 19 MOGAD patients confirmed in Shandong Provincial Hospital Affiliated to Shandong First Medical University during Sep. 2018 and Jun. 2021 were retrospectively analyzed. Results The patients included 9 males and 10 females, with a median age of 10 years. Seven cases manifested asaquaporin4(AQP4)negative neuromyelitis optica spectrum disorder(NMOSD); 6 cases presented with acute disseminated encephalomyelitis(ADEM); 4 cases presented with encephalitis, 1 of which complicated with anti-N-methyld-aspartate receptor(NMDAR)encephalitis; 1 case presented with transverse myelitis; 1 case presented with brainstem encephalitis. Abnormal signals were detected with brain MRI in 17 cases(17/19), and the lesions could be involved in any part of the brain, presenting multifocal ill-defined T₂WI hyperintensity. Spinal MRI found abnormal signals in 4 cases(4/13). Orbital MRI detected abnormal signals in 4 cases(4/9), presenting T₂WI hyperintensity in optic nerves, 2 of which(2/4)showed optic crossover and optic tract. The serum MOG-IgG antibody of all patients was positive; 1 case was NMDAR antibody positive; 1 case was thyroidperoxidase antibody positive. Conclusion The MOGAD clinical phenotype is associated with age, more common in children and adolescents, and prone to relapse. Lesions can involve the optic nerves, spinal cord, intracranial and other parts. The imaging performance is varied.

Key words: Myelin oligodendrocyte glycoprotein antibody, Neuromyelitis optica spectrum disorder, Multiple sclerosis, Magnetic resonance imaging

中图分类号:

R445.2

冯宝民,王舟,徐晗,李佳存,于乔文,修建军. 抗髓鞘少突胶质细胞糖蛋白IgG抗体相关疾病临床及影像特征[J]. 山东大学学报 (医学版), 2022, 60(3): 45-50.

FENG Baomin, WANG Zhou, XU Han, LI Jiacun, YU Qiaowen, XIU Jianjun. Clinical and imaging features of 19 cases of anti-myelin oligodendrocyte glycoprotein-IgG antibody associated disorders[J]. Journal of Shandong University (Health Sciences), 2022, 60(3): 45-50.

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