水通道蛋白-4抗体阳性儿童视神经炎的临床特点及预后

Q4 Medicine

中华眼底病杂志 Pub Date : 2020-04-25 DOI:10.3760/CMA.J.CN511434-20200207-00043

Honglu Song, Mo Yang, M. Sun, Huanfen Zhou, Quangang Xu, S. Wei

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引用次数: 0

摘要

目的观察水通道蛋白4抗体阳性儿童视神经炎（AQP4-PON）的临床、影像学特点及预后。方法回顾性分析一系列病例。本研究纳入了2015年1月至2018年12月在中国人民解放军总医院第一医学中心眼科临床确诊的14名AQP4-PON患儿的23只眼睛。所有患者均接受了BCVA、眼底彩色摄影和磁共振成像（MRI）检查。对10例患者的15眼进行了OCT，并测量了受影响眼睛的乳头周围视网膜神经纤维层（pRNFL）、黄斑神经节细胞内丛状层（mGCIPL）厚度。采用细胞间接荧光免疫法检测血清AQP4抗体和髓鞘少突胶质细胞糖蛋白抗体。随访时间28～59个月。对患儿的临床、神经影像学特点及预后进行分析。结果14例患儿中，男性2例（14.3%），女性12例（85.7%），平均发病年龄13.3±3.0岁。第一次就诊时，有10名单侧患者和4名双侧患者。首发表现为视神经炎11例（78.6%），后极综合征2例（14.3%），脊髓炎1例（7.1%），其中眼痛10例（71.4%），合并自身抗体阳性5例（35.7%）。首次发病时间小于2周时，眼底检查显示7眼（38.9%）椎间盘水肿。3个月后，15眼OCT检查的平均pRNFL和mGCIPL厚度分别为62.33±11.07和54.17±5.42μm。眼眶MRI显示，14名患者（100.0%）和11名T1强化病变患者（78.6%）的视神经显示长T2信号。当首次发病不到2周时，糖皮质激素治疗后，16眼（88.9%）BCVA≤0.1，7眼（38.9%）BCVA≤0.1，9眼（50.0%）BCVA≥0.5。11名患者在随访期间出现复发，并接受了免疫抑制剂治疗。在最后一次就诊时，14名患者中，9只眼睛（64.3%）受累于双眼，5名患者（35.7%）进展为视神经脊髓炎；BCVA≥0.5者8眼，占34.8%。结论AQP4-PON患者多见于女性，视功能严重受损，易复发，部分患者会发展为视神经脊髓炎。关键词：水通道蛋白4；视神经炎；视神经脊髓炎；儿童

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Clinical features and prognosis of aquaporin-4 antibody positive pediatric optic neuritis

Objective To observe the clinical, radiographic features and prognosis of aquaporin-4 antibody positive pediatric optic neuritis (AQP4-PON). Methods A retrospective case series. Twenty-three eyes of 14 children with AQP4-PON who were clinically confirmed in the Department of Ophthalmology of the First Medical Center of the Chinese PLA General Hospital from January 2015 to December 2018 were included in the study. All patients underwent BCVA, fundus color photography, and magnetic resonance imaging (MRI). OCT was performed on 15 eyes of 10 patients, and the peripapillary retinal nerve fiber layers (pRNFL), macular ganglion cell-inner plexiform layers (mGCIPL) thickness of the affected eyes were measured. Cell-based indirect fluorescent immunoassay was used to detect serum AQP4 antibodies and myelin oligodendrocyte glycoprotein antibodies. The follow-up time ranged from 28 to 59 months. The clinical, neuroimaging characteristics and prognosis of the children were analyzed. Results Among 14 children, 2 were male (14.3%) and 12 were female(85.7%). The mean age of onset was 13.3±3.0 years. On the first visit, there were 10 unilateral patients and 4 bilateral patients. The first manifestations were 11 patients of optic neuritis (78.6%), 2 patients of posterior pole syndrome (14.3%), and 1 patient of myelitis (7.1%). There were 10 patients (71.4%) with eye pain, and 5 patients(35.7%) combined with autoantibodies positive. When the first onset time was less than 2 weeks, fundus examination revealed disc edema in 7 eyes (38.9%). After 3 months, the average pRNFL and mGCIPL thickness of 15 eyes underwent OCT examination were 62.33±11.07 and 54.17±5.42 μm, respectively. Orbital MRI showed that the optic nerve showed a long T2 signal in 14 patients (100.0%) and 11 patients (78.6%) with T1 intensive lesions. When the first onset was less than 2 weeks, 16 eyes (88.9%) had BCVA≤0.1, and 7 eyes(38.9%) had BCVA≤0.1 and 9 eyes (50.0%) with BCVA≥0.5 after glucocorticoid treatment. Recurrence occurred in 11 patients during follow-up and was treated with immunosuppressive agents. At the last visit, in 14 patients, 9 eyes (64.3%) were involved in both eyes, and 5 patients (35.7%) progressed to neuromyelitis optica; in 23 eyes, 8 eyes (34.8%) had BCVA≥0.5. Conclusions AQP4-PON patients are more common in women, severely impaired visual function, easy to relapse, and some patients will progress to neuromyelitis optica. Key words: Aquaporin 4; Optic neuritis; Neuromyelitis optica; Child

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来源期刊

中华眼底病杂志 Medicine-Ophthalmology

CiteScore

0.40

自引率

0.00%

发文量

5383

期刊介绍： Chinese Journal of Ocular Fundus Diseases is the only scientific journal in my country that focuses on reporting fundus diseases. Its purpose is to combine clinical and basic research, and to give equal importance to improvement and popularization. It comprehensively reflects the leading clinical and basic research results of fundus disease disciplines in my country; cultivates professional talents in fundus disease, promotes the development of fundus disease disciplines in my country; and promotes academic exchanges on fundus disease at home and abroad. The coverage includes clinical and basic research results of posterior segment diseases such as retina, uveal tract, vitreous body, visual pathway, and internal eye diseases related to systemic diseases. The readers are medical workers and researchers related to clinical and basic research of fundus diseases. According to the journal retrieval report of the Chinese Institute of Scientific and Technological Information, the comprehensive ranking impact factor and total citation frequency of the Chinese Journal of Ocular Fundus Diseases have been among the best in the disciplines of ophthalmology, otolaryngology, and ophthalmology in my country for many years. The papers published have been included in many important databases at home and abroad, such as Scopus, Peking University Core, and China Science Citation Database (CSCD).