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韩国多中心回顾性研究:晚发性视神经脊髓炎谱系疾病的临床特征。

Clinical characteristics of late-onset neuromyelitis optica spectrum disorder: A multicenter retrospective study in Korea.

机构信息

Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea/Neuroscience Center, Samsung Medical Center, Seoul, Korea.

Department of Neurology, Bucheon St. Mary's Hospital, The Catholic University of Korea College of Medicine, Bucheon, Korea.

出版信息

Mult Scler. 2017 Nov;23(13):1748-1756. doi: 10.1177/1352458516685416. Epub 2017 Jan 6.

Abstract

BACKGROUND

There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD).

OBJECTIVE

We aimed to describe the characteristic features of patients with LO-NMOSD in Korea.

METHODS

Anti-aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder (NMOSD) from nine tertiary hospitals were reviewed retrospectively. The patients were divided into two groups based on age of onset: LO-NMOSD (⩾50 years of age at onset) versus early-onset neuromyelitis optica spectrum disorder (EO-NMOSD) (<50 years of age at onset). Clinical, laboratory, and magnetic resonance imaging (MRI) parameters were investigated.

RESULTS

Among a total of 147 patients (125 female; age of onset, 39.4 ± 15.2 years), 45 patients (30.6%) had an age of onset of more than 50 years. Compared to patients with EO-NMOSD, patients with LO-NMOSD had more frequent isolated spinal cord involvement at onset (64.4% vs 37.2%, p = 0.002), less frequent involvement of the optic nerve (40.0% vs 67.7%, p = 0.002), and less frequent brain MRI lesions (31.1% vs 50.0%, p = 0.034). Furthermore, there was a significant positive correlation between age of onset and Expanded Disability Status Scale (EDSS) score at last follow-up ( r = 0.246, p = 0.003).

CONCLUSION

Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.

摘要

背景

目前关于晚发性视神经脊髓炎谱系疾病(LO-NMOSD)的研究较少。

目的

我们旨在描述韩国 LO-NMOSD 患者的特征。

方法

回顾性分析了来自 9 家三级医院的抗水通道蛋白 4 抗体阳性的视神经脊髓炎谱系疾病(NMOSD)患者。根据发病年龄将患者分为两组:晚发性 NMOSD(发病年龄 ⩾50 岁)与早发性 NMOSD(发病年龄 <50 岁)。研究了临床、实验室和磁共振成像(MRI)参数。

结果

在总共 147 名患者(125 名女性;发病年龄为 39.4 ± 15.2 岁)中,45 名患者(30.6%)的发病年龄超过 50 岁。与 EO-NMOSD 患者相比,LO-NMOSD 患者发病时更常出现孤立性脊髓受累(64.4% vs 37.2%,p = 0.002),视神经受累较少(40.0% vs 67.7%,p = 0.002),脑 MRI 病变较少(31.1% vs 50.0%,p = 0.034)。此外,发病年龄与最后一次随访时的扩展残疾状态量表(EDSS)评分呈显著正相关(r = 0.246,p = 0.003)。

结论

发病年龄可能是 NMOSD 患者病变部位和临床病程的重要预测因素。

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