髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎的临床特征分析

Clinical characteristics of myelin oligodendrocyte glycoprotein antibody-positive optic neuritis

目的观察髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性视神经炎(MOG-ON)的临床特征和预后。方法回顾性系列病例研究。选取2016年1月至2017年10月在解放军总医院眼科临床确诊为MOG-ON的65例住院患者的临床及随访资料。患者入院后行最佳矫正视力(BCVA)、眼底彩色照相、相干光层析成像术和MRI等检查。所有患者均采用基于细胞的间接荧光免疫法检测血清水通道蛋白4抗体和MOG抗体。随访时间为14~217个月。分析患者临床特征、神经影像学特征及预后转归情况。连续变量的比较采用独立样本t检验或Mann-WhitneyU检验,分类变量的比较采用χ2检验或Fisher精确检验。结果儿童患者(<18岁)35例,成人患者(≥18岁)30例,女性37例,男性28例,发病年龄(23±16)岁(3~61岁)。首次发病时单眼发病40例,双眼发病25例。首次发病时伴有眼痛55例,占84.6%,儿童患者眼痛发生率明显低于成人患者[74.3%(26/35)与96.7%(29/30)比较,P=0.016];同时合并自身抗体异常13例,占20.0%;47.8%(43/90)的患眼出现视盘水肿。60.0%(39/65)的患者呈复发性视神经炎,73.8%(48/65)的患者最终症状累及双眼。63例行眼眶MRI检查者中发现视神经呈长T2信号62例,占98.4%。19例行颅脑MRI检查者中发现脑部脱髓鞘病变12例。发病2周以内,78.9%(71/90)的患眼BCVA≤0.1,儿童与成人患者发病视力均损伤严重,二者间差异无统计学意义(P=0.650)。经糖皮质激素治疗后患眼视力均有改善。首次发病恢复视力:95.6%(86/90)的患眼BCVA≥0.5,儿童与成人患者首次发病恢复视力均较好,二者间差异无统计学意义(P=0.061)。末次随访视力:86.7%(98/113)的患眼BCVA≥0.5,儿童患者末次随访视力明显好于成人患者(最小分辨角对数视力0.10±0.35与0.32±0.53比较,t=-2.526,P=0.013)。儿童患者中3例(8.6%)发病时伴有急性播散性脑脊髓膜炎。首次发病6个月后视盘周围视网膜神经纤维层厚度为(69.90±12.49)μm,黄斑区节细胞层+内丛状层厚度为(59.58±7.91)μm,均出现不同程度萎缩变薄现象,儿童与成人患者之间差异均无统计学意义(P=0.606、0.223)。

Objective To investigate the clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein(MOG) antibody-positive optic neuritis (MOG-ON). Methods Retrospective case series study. A total of 65 patients diagnosed with MOG-ON at the Department of Ophthalmology of the Chinese People′s Liberation Army General Hospital during January 2016 and October 2017 were selected. The patients underwent examinations including best corrected visual acuity (BCVA), fundus color photography, optical coherence tomography (OCT) and magnetic resonance imaging (MRI). The patients were tested for serum levels of antibodies for aquaporin 4 and MOG with a cell-based assay. The follow-up duration ranged from 14 to 217 months. Statistical analyses of patients′ clinical features, neuroimaging features and prognosis were conducted. The independent-sample t test or Mann-Whitney U test for continuous variable data and the χ^2 test or Fisher exact test for classified variable data and rates were used. Results There were 35 children (<18 years) and 30 adults (≥18 years) patients. Thirty-seven patients were female and 28 patients were male. The mean age of onset was (23±16) years (range of 3-61 years). There were 40 unilateral patients and 25 bilateral patients at the first onset. Fifty-five patients (84.6%) suffered from eye pain at the first onset and the incidence in children was significantly lower than that in adult [74.3%(26/35) and 96.7%(29/30), P=0.016]. Abnormal autoimmune antibodies were found in 13 patients (20.0%).And 47.8%(43/90) eyes had optic disc edema. Sixty percent (39/65) were recurrent optic neuritis and 73.8%(48/65) were bilateral diseases at final visit. Orbital MRI scans showed T2 hyperintensity of optic nerve in 98.4% patients (62/63). Head MRI scans showed demyelinating lesions of the brain in 12/19 patients. Within 2 weeks of onset, 78.9%(71/90) of the affected eyes had BCVA ≤ 0.1. The visual impairment of the children and adult was serious, and there was no significant difference (P=0.650). After treatment with corticosteroid, visual acuity improved in all affect eyes. After the initial attack, 95.6%(86/90) of the affected eyes had BCVA ≥ 0.5. The children and adult had good visual recovery, and there was no significant difference (P=0.061). At the final visit, 86.7%(98/113) of the eyes had BCVA ≥ 0.5, the children had better visual recovery than adult [logarithm of the minimum angle of resolution, 0.10±0.35 vs. 0.32±0.53, t=-2.526, P=0.013].Three children (8.6%) were diagnosed with acute disseminated encephalomyelitis. The average thickness of peripapillary retinal nerve fiber layers (pRNFL) and macular ganglion cell-inner plexiform layers (mGCIPL) were (69.90±12.49)μm and (59.58±7.91)μm at least 6 months after optic neuritis attack. There were different degrees atrophy and not statistically different in pRNFL and mGCIPL between children and adult (P=0.606, 0.223). Conclusions The clinical characteristics of MOG-ON are diverse. The children patients are more common and have better recovery of visual acuity. Additionally, some patients have demyelinating lesions in the brain.