Medical Surgical Unit of Respiratory Diseases, Hospital Virgen del Rocío, Av. Manuel Siurot S/N, 41013, Seville, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Respiratorias (CIBERES), Carlos III Health Institute, Madrid, Spain.
Sci Rep. 2023 Jul 26;13(1):12074. doi: 10.1038/s41598-023-39147-w.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative rare disease characterized by symptoms and signs in the upper and lower motor neurons, leading to progressive neuro-degeneration and muscle atrophy. Our objective was to analyse the quality of life (QoL) in patients with ALS and compare with general population and with patients with cancer. Prospective study from consecutive ALS patients in one center. In order to assess quality of life, during the first visit three questionnaires were administered: Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R), Short Form-36 (SF-36) and EuroQoL 5D (EQ-5D). We compared SF-36 of ALS patients with a reference population (n = 9151), and we compared the EQ-5D index score of ALS patients versus patients with cancer in the same area and in the same period (2015-2018). Between June 2015 and September 2017, 23 were included. The mean age was 65.1 ± 12.6 years and 56.5% were women. Compared with the general population, patients with ALS showed lowest QoL (p < 0.05) in all the dimensions, with a very important impairment in physical function (median: 0; p25-75: 0-10) and physical role (median: 0; p25-75: 0-6.25). In EQ-5D questionnaire, patients with ALS presented an EQ-5D index score of 0.21 ± 0.39 (mean ± standard deviation) with a visual analog scale (VAS) score of 0.32 ± 0.24. Compared with an oncological population, patients with ALS had a worse EQ-5D index score both clinically and statistically (0.21 ± 0.39 vs. 0.77 ± 0.27; p < 0.05). We demonstrate a poorer quality of life in patients with ALS is poor, and clinically and statistically worse than in patients with cancer or general population. New studies need to evaluate the impact of strategies in this population to improve the quality of life.
肌萎缩侧索硬化症（ALS）是一种神经退行性罕见疾病，其特征为上下运动神经元的症状和体征，导致进行性神经退行性变和肌肉萎缩。我们的目的是分析肌萎缩侧索硬化症患者的生活质量（QoL），并与普通人群和癌症患者进行比较。这是一项对一家中心的连续肌萎缩侧索硬化症患者进行的前瞻性研究。为了评估生活质量，在首次就诊时，我们给患者使用了三种问卷：肌萎缩侧索硬化症功能评定量表（ALSFRS-R）、健康调查简表 36 项（SF-36）和欧洲五维健康量表（EQ-5D）。我们将肌萎缩侧索硬化症患者的 SF-36 与参考人群（n=9151）进行比较，并将同一地区和同一时期（2015-2018 年）的肌萎缩侧索硬化症患者与癌症患者的 EQ-5D 指数得分进行比较。2015 年 6 月至 2017 年 9 月期间，共纳入 23 名患者。患者的平均年龄为 65.1±12.6 岁，56.5%为女性。与普通人群相比，肌萎缩侧索硬化症患者在所有维度上的生活质量均较低（p<0.05），身体功能（中位数：0；p25-75：0-10）和身体角色（中位数：0；p25-75：0-6.25）方面的损害尤其严重。在 EQ-5D 问卷中，肌萎缩侧索硬化症患者的 EQ-5D 指数得分为 0.21±0.39（平均值±标准差），视觉模拟量表（VAS）评分为 0.32±0.24。与肿瘤患者相比，肌萎缩侧索硬化症患者的 EQ-5D 指数评分在临床和统计学上均较差（0.21±0.39 比 0.77±0.27；p<0.05）。我们证明肌萎缩侧索硬化症患者的生活质量较差，且临床和统计学上均比癌症患者和普通人群差。需要开展新的研究来评估该人群中改善生活质量的策略的影响。
