Giving Breath to Motor Neurons: Noninvasive Mechanical Ventilation Slows Disease Progression in Amyotrophic Lateral Sclerosis.

Autor: Grassano M; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Koumantakis E; Department of Public Health and Pediatrics, University of Turin, Turin, Italy.; Post Graduate School of Medical Statistics, University of Turin, Turin, Italy., Manera U; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurologia 1U, Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino, Turin, Italy., Canosa A; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurologia 1U, Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino, Turin, Italy., Vasta R; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Palumbo F; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Fuda G; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Salamone P; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Marchese G; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Casale F; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Charrier L; Department of Public Health and Pediatrics, University of Turin, Turin, Italy., Mora G; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy., Moglia C; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurologia 1U, Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino, Turin, Italy., Calvo A; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurologia 1U, Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino, Turin, Italy., Chiò A; 'Rita Levi Montalcini' Department of Neuroscience, University of Turin, Turin, Italy.; Neurologia 1U, Azienda Ospedaliero Universitaria Città della Salute e della Scienza di Torino, Turin, Italy.; Institute of Cognitive Sciences and Technologies, National Council of Research, Rome, Italy.
Jazyk: angličtina
Zdroj: Annals of neurology [Ann Neurol] 2024 Apr; Vol. 95 (4), pp. 817-822. Date of Electronic Publication: 2024 Jan 29.
DOI: 10.1002/ana.26875
Abstrakt: Objective: Noninvasive mechanical ventilation (NIMV) improves amyotrophic lateral sclerosis (ALS) quality of life and survival. However, data about its effect on disease progression are still lacking. Here, we test whether NIMV use changed the rate of functional decline among ALS patients.
Methods: In this retrospective observational study, we included 448 ALS patients followed up at the ALS Center in Turin, Italy, who underwent NIMV during the disease course. The primary outcome was the change in functional decline after NIMV initiation adjusting for covariates. Functional decline was based on the nonrespiratory items of the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R).
Results: NIMV initiation resulted in a slower functional decline (mean improvement = 0.16 points per month, 95% confidence interval = 0.12-0.19, p < 0.001), with consistent effects observed across various demographic factors, including sex, age at diagnosis, and disease duration before NIMV initiation. This finding was replicated using the PRO-ACT (Pooled Resource Open-Access ALS Clinical Trials) dataset. The favorable impact of NIMV on ALSFRS-R progression was evident independently of disease stages. Notably, NIMV benefits were not dose-dependent but were particularly prominent for nighttime respiratory support.
Interpretation: NIMV significantly influences the rate of motor progression in ALS, and this effect is not determined by the nonlinearity of ALSFRS-R trajectory. The functional decline slowed following NIMV initiation, independently of the site of disease onset or disease severity at the time of NIMV initiation. Our findings underscore the importance of timely NIMV initiation for all ALS patients and highlight the need to consider NIMV-induced slowing of disease progression when evaluating clinical trial outcomes. ANN NEUROL 2024;95:817-822.
(© 2024 The Authors. Annals of Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)
Databáze: MEDLINE
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