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.
Mult Scler. 2017 Nov;23(13):1748-1756. doi: 10.1177/1352458516685416. Epub 2017 Jan 6.
There are currently few studies regarding late-onset neuromyelitis optica spectrum disorder (LO-NMOSD).
We aimed to describe the characteristic features of patients with LO-NMOSD in Korea.
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.
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).
Age of onset could be an important predictor of lesion location and clinical course of patients with NMOSD.
目前关于晚发性视神经脊髓炎谱系疾病（LO-NMOSD）的研究较少。
我们旨在描述韩国 LO-NMOSD 患者的特征。
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。
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）。
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。
