儿童MOG抗体阳性的中枢神经系统脱髓鞘病变12例临床特点分析

Clinical characteristics of 12 cases of children with MOG-IgG associated central nervous system demyelination

目的探讨髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的儿童中枢神经系统脱髓鞘病变的临床特点。方法回顾性分析2016年1月至2018年12月广东三九脑科医院神经内科收治的12例儿童MOG抗体阳性的中枢神经系统脱髓鞘病患者的临床症状、影像学特点、实验室检查、预后及随诊情况。结果 12例患者中男4例,女8例,起病年龄5~10岁,平均(8.0±3.1)岁。首发症状以意识水平下降、癫痫大发作等急性播散性脑脊髓膜炎(ADEM)样症状最多见(8例,8/12),其次为视力下降(5例,5/12),其中1例以ADEM样症状和视力下降同时起病。另有3例以ADEM样症状首发,起病后1周左右出现视力下降。12例患者行头颅MRI检查,11例(11/12)可见颅内异常病灶,以皮层下白质(10例,10/11)、脑干(7例,7/11)、丘脑(6例,6/11)、视神经(5例,5/11)受累为主,小脑(4例,4/11)、胼胝体(1例,1/11)亦可受累。9例患者行全脊髓MRI检查,5例(5/9)有异常病灶,均累及颈髓(5例,5/5),3例(3/5)累及胸髓,累及胸髓的病灶均为>3个椎体节段的长节段脊髓受累。12例患者行血清MOG抗体检测,11例(11/12)阳性,另1例(1/12)血清抗体阴性而脑脊液抗体阳性;8例患者行脑脊液MOG抗体检测,3例(3/8)阳性。所有患者经糖皮质激素及免疫球蛋白治疗,预后均良好,3例患者出现复发。结论MOG抗体相关的儿童中枢神经系统脱髓鞘病变以ADEM、视神经脊髓炎谱系疾病(NMOSD)最常见,往往以意识水平下降、癫痫大发作或视力下降起病,其预后相对较好,糖皮质激素治疗对缓解临床症状及预防复发有重要作用。

Objective To investigate the clinical characteristics of 12 cases of children with MOG-IgG associated central nervous system demyelination.Methods The clinical data, MRI studies, MOG antibody status,prognosis,and follow-up were retrospectively analyzed in 12 children with inflammatory central nervous system disorders in gd999 brain hospital from January 2016 to December 2018.Results There were 4 males and 8 females in this study,the average age was 8.0±3.1 years.The most common initial clinical symptoms were acute disseminated encephalomyelitis(ADEM)-like symptoms(8/12),followed by visual impairment(5/12),one of which presented as ADEM-like symptoms and visual impairment simultaneously,3 cases had decreased vision following ADEM-like symptoms.MRI results showed that subcortical white matter,brainstem,thalamus,optic nerve,cerebellum and corpus callosum were often affected.The most common lesions of the spinal cord located in the cervical cord and the lesions involving the thoracic cord were all longer than three vertebral segments.11(11/12)cases had positive MOG antibody in the serum and 3(3/8)in the CSF,one case was negative in the serum while positive in the CSF.All the symptoms were relieved by intravenous glucocorticoid therapy and immunoglobulin,clinical relapse occurred in some patients.Conclusions ADEM and neuromyelitis optica spectrum disorders are most common inflammatory demyelinating disorders of central nervous system that are associated with MOG-IgG in children.They often present with ADEM-like symptoms or visual impairment.Most of the patients have a good prognosis.Glucocorticoid therapy plays an important role in relieving acute clinical symptoms and preventing recurrence.