16岁以下髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎患儿临床特征分析

Clinical characteristics of myelin oligodendrocyte glycoprotein antibody-positive optic neuritis in children under 16 years old

背景髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎(myelin oligodendrocyte glycoprotein antibody-positive optic neuritis,MOG-ON)作为新的视神经炎类型写进了《中国脱髓鞘性视神经炎诊断与治疗循证指南(2021年)》,但国内对于儿童MOG-ON的临床研究还比较少。目的研究16岁以下儿童MOG-ON的临床特点和预后。方法回顾性分析2017年1月-2020年12月解放军第942医院眼科和宁夏回族自治区人民医院眼科医院诊断的MOG-ON患儿33例(57只眼)的临床资料。患儿发病年龄3~15(9.9±3.7)岁,男性患儿16例。收集患儿的临床特点和治疗后视力恢复情况。结果14例患儿(42.4%)首发表现为双眼视神经炎,18例患儿(54.5%)复发,24例患儿(72.7%)末次随访时双眼发病。首次发病时24例患儿(72.7%)伴眼痛,23只患眼(48.9%)发生视盘水肿。随访3个月后检查OCT可见视盘周围神经纤维层厚度平均为(70.66±11.11)μm,黄斑区节细胞层+内丛状层厚度平均为(60.71±7.14)μm,显示出不同程度的萎缩变薄。眼眶MRI检查可见视神经长T2信号改变32例(97.0%),部分伴有视神经强化或视神经周围强化,累及视交叉4例(12.1%)。初次发病两周之内,37只眼(78.7%)最佳矫正视力(best corrected visual acuity,BCVA)≤0.1,经过静滴甲泼尼龙治疗后视功能出现不同程度的好转,46只眼(97.9%)BCVA≥0.5。平均随访时间(34.06±17.60)个月,末次随访时54只眼(94.7%)BCVA≥0.5。结论16岁以下的MOG-ON患儿临床表现多样,对糖皮质激素冲击治疗敏感,多数预后较好。

Background Myelin oligodendrocyte glycoprotein antibody-positive optic neuritis(MOG-ON)has been included in the Evidence-Based Guidelines for Diagnosis and Treatment of Demyelinating Optic Neuritis in China(2021)as a new type of optic neuritis,but there are few domestic clinical studies on MOG-ON in children.Objective To study the clinical characteristics and prognosis of MOG-ON in children under 16 years old.Methods Clinical data about 33 children(57 eyes)with MOG-ON diagnosed by the Department of Ophthalmology of the 942 Hospital of Chinese people's Liberation Army and the Eye Hospital of People's Hospital of Ningxia Hui Autonomous Region from January 2017 to December 2020 were analyzed retrospectively.The age of onset was 3-15(9.9±3.7)years old,and 16 cases were male.The clinical characteristics of the children and the recovery of visual acuity after treatment were collected.Results There were 14 children(42.4%)with binocular optic neuritis as the first manifestation,18 children(54.5%)had recurrence,and 24 children(72.7%)had bilateral onset at the last follow-up.At the first onset,24 children(72.7%)accompanied with eye pain,and 23 eyes(48.9%)had optic disc edema.After 3 months of follow-up,OCT showed that the average thickness of peripheral optic disc nerve fiber layer(pRNFL)was(70.66±11.11)μm,and the average thickness of ganglion cell layer+inner plexus layer(mGCIPL)in macular region was(60.71±7.14)μm,showing varying degrees of atrophic thinning.Orbital MRI showed that the long T2 signal of the optic nerve was changed in 32 cases(97.0%),some of which were accompanied by optic nerve enhancement or perioptic nerve enhancement,and the optic chiasm was involved in 4 cases(12.1%).Within two weeks of the first onset,37 eyes(78.7%)had best corrected visual acuity(BCVA)≤0.1.After intravenous methylprednisolone treatment,visual function improved to varying degrees,and 46 eyes(97.9%)had BCVA≥0.5.The mean follow-up time was(34.06±17.60)months.At the last follow-up,54 eyes(94.7%)had BCVA≥0.5.Conclusion The clinical manifestations of MOG-ON in children under 16 years old are diverse.They are sensitive to corticosteroids pulse therapy,and most of them have a good prognosis.