儿童MOG抗体阳性与AQP4抗体阳性视神经脊髓炎谱系疾病的临床特征和预后比较

李志超; 万林; 孙于林; 王静; 石秀玉; 周欢粉; 徐全刚; 魏世辉; 杨光

doi:10.3969/j.issn.2095-5227.2022.06.002

儿童MOG抗体阳性与AQP4抗体阳性视神经脊髓炎谱系疾病的临床特征和预后比较

MOG-IgG- versus AQP4-IgG-positive neuromyelitis optica spectrum disorders in children: Clinical characteristics and prognosis

摘要

摘要:
背景视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders，NMOSD)是一种主要累及视神经和脊髓的中枢神经系统炎性脱髓鞘疾病谱，其病因主要与水通道蛋白4抗体(aquaporin-4 immunoglobulin-G，AQP4-IgG)及髓鞘少突胶质糖蛋白抗体(myelin oligodendrocyte glycoprotein immunoglobulin-G，MOG-IgG)有关。儿童NMOSD的发病率远远低于成人，且从临床特点不同，当前关于儿童NMOSD的相关研究较少。
目的比较AQP4-IgG阳性与MOG-IgG阳性的儿童NMOSD的临床表现、影像学特点及预后，为儿童NMOSD提供诊疗思路。
方法选取2008年1月- 2021年7月在解放军总医院第一医学中心就诊的18例AQP4-IgG阳性患儿和17例MOG-IgG阳性患儿为研究样本(起病年龄2 ~ 18岁)，总结分析患儿临床资料、扩展残疾状态量表(expanded disability status scale，EDSS)、年复发率(annualized relapse rate，ARR)、影像学特点。
结果 AQP4-IgG阳性患儿的女性占比较MOG-IgG阳性患儿高(14/18 vs 8/17)，但差异无统计学意义(P＞0.05)。MOG-IgG组较AQP4-IgG组更容易表现为双眼视力下降(14/17 vs 7/18，P=0.015)，而AQP4-IgG组更容易出现单眼或双眼无光感的情况(9/18 vs 0/17，P=0.01)。AQP4-IgG组更容易合并抗核抗体阳性(5/18 vs 0，P=0.045)。MOG-IgG组较AQP4-IgG组颅内病灶更多(17/17 vs 8/18，P<0.001)。在病程最严重时两组患儿的EDSS中位数3.0(4.0，5.0) vs 3.0(4.0，5.0)差异无统计学意义，但末次随访时MOG-IgG组EDSS评分明显低于AQP4-IgG组1.0(1.0，2.0) vs 5.0(4.0，5.0)，P＜0.001。
结论 MOG-IgG阳性的NMOSD患儿更易出现双侧视力下降、颅脑病灶和大脑综合征，但预后相对较好。

Abstract:
Background Neuromyelitis optica spectrum disorder (NMOSD) is one of inflammatory demyelinating diseases of the central nervous system that primarily involves the optic nerve and spinal cord. Its pathogeny is mainly associated with aquaporin-4 immunoglobulin-G antibodies (AQP4-IgG) and myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG). The incidence of NMOSD in children is much lower than that in adults, and the clinical features also varies between them. There are few studies about NMOSD on children.
Objective To analyze and summarize the differences in clinical manifestations, imaging characteristics and prognosis in aquaporin-4 immunoglobulin-G (AQP4-IgG) positive and myelin oligodendrocyte glycoprotein immunoglobulin-G (MOG-IgG) positive pediatric NMOSD, so as to provide diagnosis and treatment ideas for pediatric NMOSD.
Methods From January 2008 to July 2021, clinical data including expanded disability status scale (EDSS), annualized relapse rate (ARR), imaging and laboratory tests about 18 AQP4-IgG-positive and 17 MOG-IgG-positive pediatric NMOSD patients in the First Medical Center of Chinese PLA General Hospital were retrospectively summarized and analyzed.
Results The female rate of AQP4-IgG-positive children was more than MOG-IgG-positive children (14/18 vs 8/17), but the difference was not statistically significant (P＞0.05). The MOG-IgG-positive group was more likely to have binocular vision loss than that of the AQP4-IgG group (14/17 vs 7/18, P=0.015), whereas children in the AQP4-IgG-positive group were more likely to have monocular or binocular absence of light perception (9/18 vs 0/17, P=0.01). AQP4-IgG group was more likely to have ANA positivity (5/18 vs 0, P=0.045). More children in the MOG-IgG-positive group had brain lesions than those in the AQP4-IgG-positive group (17/17 vs 8/18, P<0.001) There was no difference in the median EDSS 3.0 (4.0, 5.0) vs 3.0 (4.0, 5.0) between the two groups at the most severe stage of the disease, but the EDSS score was significantly lower in the MOG-IgG-positive children than that in the AQP4-IgG-positive group at the last follow-up 1.0 (1.0, 2.0) vs 5.0 (4.0, 5.0), P<0.001.
Conclusion Children with MOG-IgG-positive NMOSD are more likely to develop bilateral vision loss, brain lesions and brain syndromes, but have a low disability rate and a relatively good prognosis.

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