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

Ana Cristina de Medeiros Garcia Maciel, Vanessa Regiane Resqueti, Lariza Maria da Costa, Ana Aline Marcelino da Silva, Jéssica Danielle Medeiros da Fonseca, Rayane Grayce da Silva Vieira, Karen de Medeiros Pondofe, Matías Otto-Yáñez, Jordi Vilaró, Rodrigo Torres-Castro, Roberto Vera-Uribe, Giane Amorim Ribeiro-Samora, Danilo Nagem, Ricardo Alexsandro Valentim, Mario Emílio Teixeira Dourado Júnior, Guilherme Fregonezi*

*Autor corresponent d’aquest treball

Producció científica: Article en revista indexadaArticleAvaluat per experts

Resum

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.

Idioma originalAnglès
Número d’article6702
Nombre de pàgines13
RevistaJournal of clinical medicine
Volum14
Número19
DOIs
Estat de la publicacióPublicada - d’oct. 2025

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