Disease progression impacts health-related quality of life in amyotrophic lateral sclerosis

Objectives: To determine the impact of disease progression on health-related quality of life in amyotrophic lateral sclerosis (ALS). Methods: A total of 161 patients with ALS were enrolled. Assessments included the revised ALS Functional Rating Scale and the ALS Assessment Questionnaire (ALSAQ-40). Data analysis comprised linear regression and multivariate analyses. Results: ALSFRS-R score (β = 0.75, p < 0.001), depression (β = 0.08, p < 0.001), pain (β = 0.07, p < 0.001), hopelessness (β = 0.07, p = 0.001), and progression rate (β = 0.02, p = 0.02) explained 76% of the ALSAQ-40 summary index variance. Progression rate alone explained 7% of the ALSAQ-40 summary index variance. The subdomains of emotional well-being, followed by ADL, and finally communication and eating were most strongly influenced by progression rate. Conclusion: Our study demonstrates the importance of physical health for emotional well-being. In particular, slower disease progression is associated with higher levels of emotional well-being in ALS.