儿童抗髓鞘少突胶质细胞糖蛋白IgG相关疾病诊断及治疗进展

Progress on diagnosis and treatment of anti-myelin oligodendrocyte glycoprotein-IgG associated disorders in children

抗髓鞘少突胶质细胞糖蛋白IgG相关疾病(anti-myelin oligodendrocyte glycoprotein-IgG associated disorders,MOGAD)是儿童特发性中枢神经系统脱髓鞘中最常见的一类疾病。儿童MOGAD可为单相性或多相性病程。常见临床表型包括急性播散性脑脊髓炎、视神经炎、横贯性脊髓炎、视神经脊髓炎谱系疾病等,少见表型包括脑炎、重叠综合征和颅神经炎等,对于多相性病程患儿,上述同一表型可反复或不同表型先后组合。目前推荐转染细胞法检测血清MOG-IgG作为诊断依据,血清MOG-IgG的持续存在是多相性病程的主要危险因素。多相性MOGAD的疾病修正治疗尚缺乏高质量临床研究证据,可选择的药物包括硫唑嘌呤、吗替麦考酚酯、利妥昔单抗和每月维持静脉注射免疫球蛋白等。本病的预后取决于临床表型、病程以及治疗等因素。MOGAD正在被逐渐认识,尚有很多问题亟待解决。

Anti-myelin oligodendrocyte glycoprotein-IgG associated disorders(MOGAD)are the most common disorders among children with idiopathic central nervous system demyelination.MOGAD in children can be monophasic or multiphasic.Common clinical phenotypes include acute disseminated encephalomyelitis,optic neuritis,transverse myelitis,and optic neuromyelitis spectrum disorders,etc.Additionally,while rare phenotypes include encephalitis,overlapping syndrome,and cranial neuritis.For children with multiphasic MOGAD,the same phenotypes mentioned above can be repeated or combined with other phenotypes.At present,the cell-based assay is recommended to detect serum MOG-IgG level as the diagnostic basis,and the persistent presence of serum MOG-IgG level is a major risk factor for recurrence.There is a lack of high-quality clinical evidence for the treatment of disease modification in multiphasic MOGAD.Alternative drugs include azathioprine,mycophenolate mofetil,rituximab,and maintenance of intravenous immunoglobulin.The prognosis depends on the clinical phenotype,course of disease,and treatment.MOGAD is being recognized,and there are still many problems needed to be solved.