17例髓鞘少突胶质细胞糖蛋白抗体相关疾病患者的临床分析

The clinical features of 17 patients with the myelin oligodendrocyte glycoprotien antibody-associated disease

目的探讨髓鞘少突胶质细胞糖蛋白抗体相关疾病患者的临床特点。方法回顾性分析2017年1月-2019年6月就诊于中山大学附属第一医院神经内科17例MOGAD患者临床资料,总结其临床及影像学特点。结果17例MOGAD患者中男性11例,女性6例,发病年龄为(27.76±12.22)岁。10例出现ON症状,9例出现脊髓炎症状,10例表现为脑部受累症状,1例表现典型ADEM。17例患者血清均检测出MOG抗体,抗体滴度为(1∶10~1∶320),其中7例CSF检测出MOG抗体,抗体滴度为(1∶1~1∶32),部分患者可合并其他自身免疫性抗体阳性。17例患者MRI可见病灶,为T2WI、T2FLAIR序列斑片状高信号影,部分出现边缘强化。17例患者入院均予激素冲击治疗,13例同时予IVIG治疗。16例患者症状在1个月内均有改善,1例患者症状改善不明显,予血浆置换后症状改善。随访1 y内,6例出现复发。结论MOGAD临床表现多样,主要表现为ON、脊髓炎、脑炎、脑膜脑炎等,大多数患者急性期免疫治疗有效,部分可出现复发。

Objective To investigate the clinical features of the myelin oligodendrocyte glycoprotien antibody-associated disease(MOGAD).Methods The clinical data of 17 MOGAD patients who were hospitalized in The First Affiliated Hospital of Sun Yat-sen University from January 2017 to June 2019 were retrospectively analyzed.Then we summarized the clinical and imagine features of the MOGAD.Results Seventeen patients,including 11 males and 6 females,were recruited in this study,and the average age of onset was(27.76±12.22)years old.Ten patients presented as ON,9 cases manifested as myelitis,10 cases presented as the symptoms of brain damage,and 1 case as the ADEM.The MOG antibody in serum of 17 patients were positive,and the MOG antibody titers were from 1∶10 to 1∶320.Among them,the MOG antibody in cerebrospinal fluid of 7 patients were positive,and the antibody titers were from 1∶1 to 1∶32.Other autoimmune antibodies were positive in a small group of these patients.Magnetic resonance imaging(MRI)scan are abnormal in all the patients at onset.The majority lesions appear as poorly demarcated hyperintensities on T2-weighted and T2FLAIR images,some of them showed edge enhancement.All patients were treated with intravenous methylprednisolone,and 13 patients were treated with IVIG.The symptoms of 16 cases were improved within a month,and another case improved after the plasma exchange.And 6 cases recurred within a year.Conclusion The clinical features of the MOGAD were various,mainly including ON,myelitis,encephalitis,meningoencephalitis i.e.Early immunotherapy can result in having a complete or almost complete recovery.However,some patients had the risk of relapse.