Neurology Department, American University of Beirut Medical Center, Beirut, Lebanon.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov;21(7-8):614-619. doi: 10.1080/21678421.2020.1788095. Epub 2020 Jul 7.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder primarily manifesting as motor deficits. It is caused by motor neuron death and leads to progressive disability and demise. It can present at any age, manifest as several phenotypes, and may have a variable progression pattern. : This retrospective study is based on chart review of subjects presenting to the American University of Beirut Medical Center from June 2015 till March 2020. It aims to describe the characteristics of ALS in Lebanon. : Out of 140 subjects identified, 113 had classical ALS. The mean age in classical and atypical ALS were 55.5 and 55.6 years, male gender was predominant in both groups, and the mean duration from disease onset to diagnosis was 10 months in classic ALS compared to 22 months in atypical ALS. The median survival in subjects with classical ALS was 31 months which was significantly lower than atypical ALS phenotypes of 41.5 months. Additionally, more than half of patients (57%) were found to have a moderate ALSFRS-R progression rate. : The study is the first to report the characteristics of amyotrophic lateral sclerosis in Lebanon. Moreover, we were able to categorize patients with classical phenotype according to disease progression using the ALSFRS-R score which can be a useful tool in determining disease prognosis at an early stage.
肌萎缩侧索硬化症（ALS）是一种主要表现为运动功能障碍的神经退行性疾病。它是由运动神经元死亡引起的，并导致进行性残疾和死亡。它可以在任何年龄出现，表现为几种表型，并且可能具有不同的进展模式。
本回顾性研究基于 2015 年 6 月至 2020 年 3 月在美国贝鲁特大学医学中心就诊的患者的图表回顾。它旨在描述黎巴嫩 ALS 的特征。
在确定的 140 名患者中，有 113 名患有经典 ALS。经典和非典型 ALS 的平均年龄分别为 55.5 岁和 55.6 岁，两组均以男性为主，从疾病发作到诊断的平均时间为经典 ALS 的 10 个月，而非典型 ALS 的 22 个月。经典 ALS 患者的中位生存期为 31 个月，明显低于非典型 ALS 表型的 41.5 个月。此外，超过一半的患者（57%）发现疾病进展的 ALSFRS-R 进展率中等。
这项研究是第一个报告黎巴嫩肌萎缩侧索硬化症特征的研究。此外，我们能够根据 ALSFRS-R 评分对经典表型患者进行分类，这可以作为早期确定疾病预后的有用工具。
