TY - JOUR
T1 - The Impact of Respiratory Function on Functionality and Mortality in ALS Patients
AU - Maciel, Ana Cristina de Medeiros Garcia
AU - Resqueti, Vanessa Regiane
AU - Costa, Lariza Maria da
AU - Silva, Ana Aline Marcelino da
AU - Fonseca, Jéssica Danielle Medeiros da
AU - Vieira, Rayane Grayce da Silva
AU - Pondofe, Karen de Medeiros
AU - Otto-Yáñez, Matías
AU - Vilaró, Jordi
AU - Torres-Castro, Rodrigo
AU - Vera-Uribe, Roberto
AU - Ribeiro-Samora, Giane Amorim
AU - Nagem, Danilo
AU - Valentim, Ricardo Alexsandro
AU - Dourado Júnior, Mario Emílio Teixeira
AU - Fregonezi, Guilherme
N1 - Publisher Copyright:
© 2025 by the authors.
PY - 2025/10
Y1 - 2025/10
N2 - 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.
AB - 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.
KW - amyotrophic lateral sclerosis
KW - functionality
KW - mortality
KW - respiratory function
UR - https://www.scopus.com/pages/publications/105018829141
U2 - 10.3390/jcm14196702
DO - 10.3390/jcm14196702
M3 - Article
AN - SCOPUS:105018829141
SN - 2077-0383
VL - 14
JO - Journal of clinical medicine
JF - Journal of clinical medicine
IS - 19
M1 - 6702
ER -