How different domains in ALSFRS-r have a differential ompact on quality of life among ALS patients: A survey study

Rationale: Amyotrophic Lateral Sclerosis (ALS) is a fatal, demyelinating disease which severely impacts the Quality of Life (QoL) of affected patients. Alongside decreased survival rates, the role of disability in QoL among ALS patients is crucial, next to. The revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is the most commonly employed tool for evaluating patients’ disability. Even though ALSFRS-R is widely used in clinical trials, it comes with limitations, as it provides limited information concerning the patients’ experience. In this study PROMIS-10 questionnaire was used in order to overcome this limitation and to integrate patients’ subjective perspectives on their physical and mental health. Objective: This study aims to identify which of the functional disability domains affected by ALS (Gross Motor Function, Fine Motor Function, Respiratory Function and Bulbar Function) are most strongly associated with the decline in quality of life, as it is measured by PROMIS-10 questionnaire. Methods: This was a survey research based on a self-reported, online questionnaire. Responses in the PROMIS-10 questionnaire were obtained from 419 ALS patients, diagnosed at the outpatient clinic of University Medical Center Utrecht. The dataset included demographic characteristics, clinical variables, ALSFRS-R subdomain scores, and PROMIS-10 scores. Statistical analysis involved simple linear correlations, multiple linear regressions, and partial correlations in order to examine the relationship between each ALSFRS-R subdomain and patients’ Mental and Physical Health. Results: The statistical analysis revealed a statistically significant positive correlation between Physical Health and Mental Health. Among the ALSFRS-r subdomains, only Gross Motor Function showed a significant influence on PH, while the other ALSFRS-r subdomains (Fine Motor Function, Bulbar Function, and Respiratory Function) were not significant predictors. Similarly, MH was also significantly influenced by Gross Motor Function, but in a negative direction, suggesting that better Gross Motor Function was associated with poorer Mental Health, while the other ALSFRS-r subdomains did not exhibit a statistically significant impact. Conclusion: These findings highlight the complex and sometimes paradoxical relationship between physical and mental aspects of QoL in ALS. While better motor function enhances physical well-being, it may concurrently relate to poorer mental health, possibly reflecting increased awareness of disease progression or psychological distress in more physically capable patients.