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髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘疾病临床与预后分析
作者: 董敏睿、陈立杰
单位:郑州大学第一附属医院 450052
关键词: 髓鞘少突胶质细胞糖蛋白、糖皮质激素、复发
分类号:R744.5
出版年·卷·期(页码):2020·46·第 8 期(481-485)
摘要:
目的 探讨髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein,MOG)抗体相关获得性脱髓鞘综合征(acquired demyelinating syndromes,ADS)的临床特点.方法 回顾性分析6例MOG-ADS患者的临床表现、影像学、脑脊液检查以及治疗,用EDSS量表评估预后.结果 6例患者中包括4例女性,2例男性,平均发病年龄29岁(4~64岁),3例表现为视神经炎(optic neuritis,ON)、2例表现为急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM),1例以脑干症状起病;用细胞转染法(cell-based assay,CBA)检测血清MOG抗体滴度在1:100-1:10之间,1例累及视神经眼段,3例头颅MRI累及皮质下白质、脑干,1例且累及大脑皮层、基底节/丘脑,颅内病灶体积较大、边界不清;1例累及脊髓,表现为长节段脊髓炎.所有患者给予激素治疗,均达到部分或完全缓解.平均随访7.8个月,2例复发,2例符合多发性硬化(multiple sclerosis,MS)的诊断标准.结论 MOG-ADS主要表现为ON和ADEM,MRI病灶分布广泛,对激素治疗敏感,部分患者可出现疾病的复发.
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