未成年人髓鞘少突胶质细胞糖蛋白抗体相关视神经炎的临床特点、治疗及预后分析

Clinical characteristics,treatment and prognosis of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis in children

目的回顾性分析未成年人髓鞘少突胶质细胞糖蛋白(MOG)抗体相关的视神经炎的临床特点、治疗及预后。方法收集2016年1月1日至2018年12月31日在广州市妇女儿童医疗中心诊断为MOG抗体相关的视神经炎患者的临床资料、实验室检查、最佳矫正视力、眼底检查、神经电生理及影像学结果、治疗及预后资料,进行回顾性分析。结果(1)临床特点:16例患者共29眼受累,男∶女为1∶1,起病年龄为(7.0±2.9)岁,7/16患者存在前驱感染,单侧∶双侧为3∶13,复发性视神经炎2例。治疗前视力≤0.1共19眼(65.5%),其中无光感4眼,光感5眼,眼前手动5眼,眼前指动5眼;0.1~0.5共10眼(34.5%)。治疗后随访视力:0.1~0.5共4眼(13.8%),0.5~1.0共5眼(17.2%),>1.0共20眼(69.0%)。(2)辅助检查:12/16患者急性期眼底检查可见视神经乳头炎,所有患者视觉诱发电位检查均有不同程度P100波潜伏期延长及振幅降低,13/16患者MRI T2WI可见视神经肿胀、增粗。12/16患者MRI可见颅内病变,3/16患者有长节段脊髓病变。(3)治疗及预后:13/16急性期给予静脉甲泼尼龙冲击联合静脉输注人免疫球蛋白治疗有效,2例复发视神经炎的患者维持期给予霉酚酸脂口服无复发,1例激素不敏感患者给予利妥昔单抗治疗病情无进展。患者随访时间(16±9)个月,随访期间无复发,10/16治愈,4/16明显好转,2/16无明显改善。结论未成年人MOG抗体相关的视神经炎患者发病率无明显的性别差异,双侧受累多见,急性期视力受损严重。多数患者急性期静脉甲泼尼龙冲击联合人免疫球蛋白治疗反应好。多数患者预后好,少数患者有神经系统后遗症。

Objective To investigate the clinical characteristics,treatment and prognosis of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis(MOG-ON)in pediatric patients.Methods Clinical data,laboratory examination,the initial best corrected visual acuity(BCVA),fundus,neuroelectrophysiological results,MRI imaging,treatment and prognosis of children diagnosed with MOG-ON from 2016 to 2019 were retrospectively analyzed.Results A total of 29 eyes from 16 children were involved,with a male/female ratio of 1∶1,onset age of(7.0±2.9)years.Seven of 16 patients had prodromal infection,with a unilateral/bilateral ratio of 3∶13,and 2 cases had recurrent optic neuritis.Before treatment,BCVA of 19 eyes(65.5%)was≤0.1,among them,4 had no sense of light,5 had light sense,5 with sense of hands in front of eyes,and 5 with sense of fingers in front of eyes.There were 10 eyes(34.5%)with BCVA of 0.1-0.5.After treatment,there were 4 eyes(13.8%),5 eyes(17.2%)and 20 eyes(69.0%)in groups with BCVA of 0.1-0.5,0.5-1.0,and>1.0,respectively.Twelve of 16 patients had optic papillitis in fundus examination during acute phase.The latency was prolonged and the amplitude was decreased in P100 wave of all the children.Thirteen out of 16 children showed swelling and thickening of optic nerve in MRI T2WI.MRI images exhibited intracranial demyelinating lesions in 12 of 16 children and long segment spinal cord lesions in 3 of 16 children.Thirteen of 16 patients showed effective results after intravenous methylprednisolone(IVMP)and intravenous immunoglobulin(IVIG)treatment.There was no relapse after administration of mycophenolate mofetil in 2 recurrent children.No progression after administration of rituximab was found in 1 child with corticosteroid insensitivity.The average follow-up time was(16±9)months and no recurrence occurred.Ten of 16 patients had full recovery,4 had significant improvement,and 2 showed no significant improvement.Conclusions There is no significant gender difference in the incidence of pediatric MOG-ON.Bilateral involvement and severe visual impairment are common in acute phase.Most patients have good response to IVMP combined with IVIG treatment and hence have a good prognosis.Only a few of them have neurological sequelae.