Objective

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. Methods: 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. Results: 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. Conclusions: 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.

中文翻译：

---

黎巴嫩肌萎缩侧索硬化的特征——图表回顾。

客观的

肌萎缩侧索硬化 (ALS) 是一种神经退行性疾病，主要表现为运动障碍。它是由运动神经元死亡引起的，并导致进行性残疾和死亡。它可以出现在任何年龄，表现为多种表型，并且可能具有可变的进展模式。方法：这项回顾性研究基于对 2015 年 6 月至 2020 年 3 月在贝鲁特美国大学医学中心提交的受试者的图表回顾。旨在描述黎巴嫩 ALS 的特征。结果：在确定的 140 名受试者中，113 名患有经典 ALS。典型和非典型 ALS 的平均年龄分别为 55.5 和 55.6 岁，两组均以男性为主，典型 ALS 从发病到诊断的平均持续时间为 10 个月，而非典型 ALS 为 22 个月。经典 ALS 受试者的中位生存期为 31 个月，显着低于非典型 ALS 表型的 41.5 个月。此外，发现超过一半的患者 (57%) 具有中等的 ALSFRS-R 进展率。结论：该研究首次报道了黎巴嫩肌萎缩侧索硬化的特征。此外，我们能够使用 ALSFRS-R 评分根据疾病进展对具有经典表型的患者进行分类，这可以成为确定早期疾病预后的有用工具。