Algahtani Hussein, Shirah Bader, Ibrahim Badr, Malik Yaser Al, Makkawi Seraj
King Abdulaziz Medical City/ King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
King Abdullah International Medical Research Center / King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.
Mult Scler Relat Disord. 2021 Jan;47:102668. doi: 10.1016/j.msard.2020.102668. Epub 2020 Dec 2.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune astrocytopathy that affects several regions of the central nervous system (CNS) with a predilection for the optic nerves and spinal cord. Epidemiological studies of NMOSD are uncommon in the Middle East and up-to-date, there are no such data from Saudi Arabia. In this study, we aim to study the clinical pattern of NMOSD patients in Saudi Arabia.
A retrospective multi-center observational study was conducted at King Abdulaziz Medical City in Jeddah and Riyadh, Saudi Arabia. The inclusion criteria consisted of all the patients with either neuromyelitis optica according to the 2006 criteria or NMOSD according to the 2015 criteria. The study period was 20 years.
A total of 23 patients were included in the study. Four were males (17.4%) and 19 were females (82.6%). The attack type was optic neuritis in 3 patients (13.0%), transverse myelitis in 15 patients (65.2%), and both in 5 patients (21.7%). All patients (100%) received pulse steroid therapy (intravenous methylprednisolone 1 g for 5 days) at the onset of the disease. Fifteen patients had plasma exchange therapy (65.2%). All patients received maintenance immunosuppressive treatment except 1 (4.3%).
NMOSD is a rare, broad-spectrum, polyphasic, rare disorder primarily affecting the optic pathway and the spinal cord either in isolation or simultaneously. Unfortunately, there are no adequate studies that assess NMOSD cohorts in Saudi Arabia despite the increased number of diagnosed cases. In addition, there is no registry for this disorder with only a few specialized centers dealing with its management. It is time to establish specialized demyelinating disease centers and build expertise in both common and rare diseases in this category.
视神经脊髓炎谱系障碍（NMOSD）是一种罕见的自身免疫性星形细胞病，可累及中枢神经系统（CNS）的多个区域，尤其好发于视神经和脊髓。NMOSD的流行病学研究在中东地区并不常见，截至目前，沙特阿拉伯尚无此类数据。在本研究中，我们旨在研究沙特阿拉伯NMOSD患者的临床模式。
在沙特阿拉伯吉达和利雅得的阿卜杜勒阿齐兹国王医疗城进行了一项回顾性多中心观察性研究。纳入标准包括所有符合2006年标准的视神经脊髓炎患者或符合2015年标准的NMOSD患者。研究期限为20年。
共有23例患者纳入研究。男性4例（17.4%），女性19例（82.6%）。发作类型为视神经炎3例（13.0%），横贯性脊髓炎15例（65.2%），两者均有的5例（21.7%）。所有患者（100%）在疾病发作时均接受了脉冲类固醇治疗（静脉注射甲泼尼龙1 g，共5天）。15例患者接受了血浆置换治疗（65.2%）。除1例（4.3%）外，所有患者均接受了维持性免疫抑制治疗。
NMOSD是一种罕见的、广谱的、多相性疾病，主要孤立或同时累及视神经通路和脊髓。遗憾的是，尽管确诊病例数有所增加，但沙特阿拉伯尚无足够的研究评估NMOSD队列。此外，这种疾病没有登记系统，只有少数专门中心处理其管理。现在是时候建立专门的脱髓鞘疾病中心，并积累此类常见和罕见疾病的专业知识了。
