The Impact of Respiratory Function on Functionality and Mortality in ALS Patients

Maciel, ACDG; Resqueti V.R.; Da Costa L.M.; Da Silva, AAM; da Fonseca, JDM; Vieira, RGD; Pondofe, KD; Otto-Yáñez, M; Vilaró, J.; Torres-Castro R.; Vera-Uribe R.; Ribeiro-Samora, GA; Nagem, D; Valentim, RA; Dourado, MET Jr; et. al.

Keywords: functionality, mortality, amyotrophic lateral sclerosis, Respiratory function

Abstract

Objective: To investigate the relationship between respiratory function, functionality, and mortality in amyotrophic lateral sclerosis (ALS) patients and to determine which respiratory parameters show the strongest correlation with functionality and mortality. Methods: The study was conducted in Rio Grande do Norte, Northeast Brazil, between January 2018 and December 2023. This was a retrospective cohort, following individuals with ALS who were evaluated at the University Laboratory. Results: A total of 74 ALS patients were included in the analysis, with a mean age of 55.7 ± 13.5 years. Most were male (66.2%) and predominantly presented with spinal-onset ALS (51.3%). Respiratory variables (except peak expiratory flow (PEF)) showed a weak but significant inverse correlation with mortality (FVC% predicted (rpb = −0.260; p < 0.001), SNIP (rpb = −0.235; p = 0.001), MEP (rpb = −0.207; p = 0.007), MIP (rpb = −0.198; p = 0.009), and PEF% predicted (rpb = −0.156; p = 0.013)). When analyzing their correlation with ALSFRS-R, all variables showed a significant positive correlation (ranging from weak to moderate) with functionality. A reduction of one unit in the respiratory variables PEF% of predicted, maximal inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) increased the risk of death by an average of 300% (OR = 2.99; 95% CI: 2.05–4.35), 2% (OR = 1.02; 95% CI: 1.01–1.03), and 1% (OR = 1.01; 95% CI: 1.00–1.02), respectively. Conclusions: Our findings suggest that direct measurements of respiratory function and muscle strength, particularly PEF and SNIP, may serve as more useful markers to guide early interventions such as non-invasive ventilation, thereby improving quality of life and potentially prolonging survival. © 2025 by the authors.

Más información

Título según WOS: The Impact of Respiratory Function on Functionality and Mortality in ALS Patients
Título de la Revista: Journal of Clinical Medicine
Volumen: 14
Número: 19
Editorial: Multidisciplinary Digital Publishing Institute (MDPI)
Fecha de publicación: 2025
Idioma: English
DOI:

10.3390/jcm14196702

Notas: ISI