中华眼底病杂志

中华眼底病杂志

16岁以下的儿童脱髓鞘性视神经炎临床特征分析

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目的 观察16岁以下的儿童脱髓鞘性视神经炎(DON)的临床特征。 方法 回顾性分析。临床确诊为DON并住院治疗的16岁以下儿童患者42例77只眼纳入研究。其中,男性22例,占52.4%;女性20例,占47.6%。年龄3~15岁,平均年龄(9.5±2.3)岁。单眼发病7例,双眼发病35例。发病前有前驱症状27例,占64.3%。所有患儿均行最佳矫正视力(BCVA)、眼底彩色照相、视觉诱发电位(VEP)、面对面视野及MRI等视功能和神经影像检查。所有患儿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(AQP4)抗体;24例患儿同时行髓鞘少突胶质细胞糖蛋白(MOG)抗体检测。统计分析患儿临床症状、眼底和视功能检查特征、神经影像检查特征及血清学特异性抗体检查结果。所有患儿均行糖皮质激素冲击治疗。治疗后平均随访时间为(1.17±0.42)年。随访期间对BCVA≥0.3且能配合检查者行Humphery自动视野计检查,观察其视野改变。同时,观察末次随访时患眼的BCVA。 结果 23.8%的患儿首发症状为双眼视神经炎(ON);83.3%的患儿最终症状累及双眼。64.2%的患儿呈复发性ON。发病2周内,87.0%的患眼BCVA≤0.1;77.9%的患眼出现视盘水肿。所有患眼均存在视野缺损和VEP异常,后者主要表现为P100波或P2波潜伏期延迟,伴不同程度的振幅降低。全部患儿中,AQP4抗体检测结果阳性2例,占4.7%。行MOG抗体检测的24例患儿中,阳性5例,占20.8%。AQP4、MOG抗体阳性患儿均呈复发性ON。眼眶MRI检查发现,视神经呈长T2信号40例,占95.2%;呈大于1/2视神经长度的长节段病变5例,占11.9%。视神经长节段病变的5例患儿中,AQP4抗体阳性2例,MOG抗体阳性3例。合并脑脱髓鞘病变4例,其中MOG抗体阳性3例。合并脊髓脱髓鞘病变3例,其中AQP4抗体阳性2例,MOG抗体阳性1例。经糖皮质激素治疗后,患眼视力均有改善,其中84.4%的患眼BCVA≥0.5。经治疗后BCVA≥0.3且能配合Humphery自动视野计检查的26例48只眼,均存在中心暗点、旁中心暗点、盲中心暗点、弥漫视野缺损等与视神经损害相关的视野异常。 结论 16岁以下的儿童DON可出现严重视力障碍和较高复发倾向,易累及视盘,对糖皮质激素治疗反应良好。AQP4或MOG抗体阳性者易并发其他中枢神经系统炎性脱髓鞘改变。

Objective To observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16. Methods A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis. Results 23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage. Conclusions Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.

关键词: 视神经炎; 脱髓鞘疾病; 儿童; 疾病特征

Key words: Optic neuritis; Demyelinating diseases; Child; Disease attributes

引用本文: 赵颖, 徐全刚, 魏世辉, 赵杰, 滕达. 16岁以下的儿童脱髓鞘性视神经炎临床特征分析. 中华眼底病杂志, 2017, 33(5): 472-475. doi: 10.3760/cma.j.issn.1005-1015.2017.05.008 复制

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