Hou C, Chen W X, Qiu W, Li X J, Tian Y, Zhu H X, Zeng Y R, Liang H C, Peng B W, Chen L F
Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou 510120, China.
Department of Neurology, the Third Affiliated Hospital of Sun Yat-sen University, Guangzhou 510630, China.
Zhonghua Yi Xue Za Zhi. 2020 Mar 10;100(9):685-689. doi: 10.3760/cma.j.issn.0376-2491.2020.09.008.
To investigate the clinical characteristics, treatment and prognosis of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) in pediatric patients. 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. 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. 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.
探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关视神经炎（MOG-ON）的临床特征、治疗及预后。回顾性分析2016年至2019年诊断为MOG-ON的儿童的临床资料、实验室检查、初始最佳矫正视力（BCVA）、眼底、神经电生理结果、MRI成像、治疗及预后。共纳入16例儿童的29只眼，男/女比例为1∶1，发病年龄为（7.0±2.9）岁。16例患者中有7例有前驱感染，单眼/双眼比例为3∶13，2例有复发性视神经炎。治疗前，19只眼（65.5%）的BCVA≤0.1，其中4只无光感，5只有光感，5只有眼前手动感，5只有眼前指动感。BCVA为0.1 - 0.5的有10只眼（34.5%）。治疗后，BCVA为0.1 - 0.5、0.5 - 1.0及>1.0的组分别有4只眼（13.8%）、5只眼（17.2%）和20只眼（69.0%）。急性期眼底检查16例患者中有12例为视神经乳头炎。所有儿童P100波潜伏期延长、波幅降低。16例儿童中13例在MRI T2WI上显示视神经增粗。16例儿童中有12例MRI图像显示颅内脱髓鞘病变，3例显示长节段脊髓病变。16例患者中有13例在静脉注射甲泼尼龙（IVMP）和静脉注射免疫球蛋白（IVIG）治疗后显示有效。2例复发性儿童在服用霉酚酸酯后未复发。1例对皮质类固醇不敏感的儿童在使用利妥昔单抗后未进展。平均随访时间为（16±9）个月，无复发。16例患者中有10例完全恢复，4例明显改善，2例改善不明显。儿童MOG-ON的发病率无明显性别差异。急性期双侧受累及严重视力损害常见。大多数患者对IVMP联合IVIG治疗反应良好，因此预后良好。只有少数患者有神经后遗症。
