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性别对水通道蛋白 4 免疫球蛋白 G 相关视神经脊髓炎谱系疾病的影响。

Gender effect on neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G.

机构信息

Department of Neurology, Seoul National University College of Medicine, Seoul, Korea.

Nuffield Department of Clinical Neurosciences, Neuroimmunology Group, Oxford, UK.

出版信息

Mult Scler. 2017 Jul;23(8):1104-1111. doi: 10.1177/1352458516674366. Epub 2016 Oct 19.

Abstract

BACKGROUND

Neuromyelitis optica spectrum disorder with aquaporin4-immunoglobulin G (NMOSD-AQP4) is an inflammatory disease characterised by a high female predominance. However, the effect of gender in patients with NMOSD-AQP4 has not been fully evaluated.

OBJECTIVE

The aim of this study was to determine the effect of gender in clinical manifestations and prognosis of patients with NMOSD-AQP4.

METHODS

The demographics, clinical and radiological characteristics, pattern reversal visual evoked potential (VEP) test results, and prognosis of 102 patients (18 males) with NMOSD-AQP4 were assessed.

RESULTS

Male patients had a higher age at onset (48.7 vs 41 years, p = 0.037) and less optic neuritis as the onset attack (17% vs 44%, p = 0.026), higher tendency to manifest as isolated myelitis over the follow-up period (67% vs 28%, p = 0.005), fewer optic neuritis attacks per year (0.08 vs 0.27, p < 0.001), and shorter relative P100 latency on VEP testing (97.1% vs 108.3%, p = 0.001). Moreover, male gender was significantly associated with the absence of optic neuritis attacks over the follow-up period independent of their age of onset.

CONCLUSION

In NMOSD-AQP4 patients, gender impacts on disease onset age and site of attack. This may be an important clue in identifying NMOSD-AQP4 patients with limited manifestations as well as in predicting their clinical courses.

摘要

背景

水通道蛋白 4 免疫球蛋白 G 相关视神经脊髓炎谱系疾病(NMOSD-AQP4)是一种以女性高发为特征的炎症性疾病。然而,性别因素对 NMOSD-AQP4 患者的影响尚未得到充分评估。

目的

本研究旨在确定性别对 NMOSD-AQP4 患者临床表现和预后的影响。

方法

评估了 102 例 NMOSD-AQP4 患者(18 例男性)的人口统计学、临床和影像学特征、反转视觉诱发电位(VEP)测试结果和预后。

结果

男性患者的发病年龄更高(48.7 岁比 41 岁,p=0.037),首发症状为视神经炎的比例更低(17%比 44%,p=0.026),在随访期间更倾向于表现为孤立性脊髓炎(67%比 28%,p=0.005),视神经炎发作次数更少(每年 0.08 次比 0.27 次,p<0.001),VEP 检测的相对 P100 潜伏期更短(97.1%比 108.3%,p=0.001)。此外,性别独立于发病年龄,与随访期间无视神经炎发作显著相关。

结论

在 NMOSD-AQP4 患者中,性别影响疾病的发病年龄和发病部位。这可能是识别 NMOSD-AQP4 患者临床表现有限以及预测其临床病程的重要线索。

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