Non-Invasive Mechanical Ventilation slows motor decline in Amyotrophic Lateral Sclerosis: a population-based study
Maurizio Grassano1, Emanuele Koumantakis1, Cristina Moglia1, Antonio Canosa1, Umberto Manera1, Rosario Vasta1, Francesca Palumbo1, Stefano Callegaro1, Giulia Marchese1, Paolina Salamone1, Giuseppe Fuda1, Federico Casale1, Andrea Calvo1, Gabriele Mora1, Adriano Chio1
1"Rita Levi Montalcini" Department of Neuroscience, University of Turin
Objective:
To test whether Non-Invasive Mechanical Ventilation (NIMV) use improved the rate of motor decline among ALS patients.
Background:
The efficacy of NIMV in improving Amyotrophic Lateral Sclerosis (ALS) survival and quality of life is well established. However, data regarding its effect on disease progression are still lacking.
Design/Methods:
This population-based cohort study included 630 patients with ALS from the Piemonte and Valle d’Aosta Register for ALS (PARALS) who were followed up after NIV initiation. Motor function was evaluated with the ALS Functional Rating Score – Revised (ALSFRS-R). Mixed-effect models for longitudinal data were performed to assess change in the rate of functional decline after NIMV initiation.
Results:
NIMV was associated with a ~30% reduction in the ∆ALSFRS-R (post-NIMV difference in ∆ALSFRS-R -0.46, 97.5% CI -0.43 to -0.49, p<0.0001). The NIMV-related slowing was present on bulbar symptoms (post-NIMV difference in ∆ALSFRS-R -0.08, 97.5% CI -0.065 to -0.010, p < 0.0001) and even greater on spinal function (post-NIMV difference in ∆ALSFRS-R -0.42, 97.5% CI -0.39 to -0.46, p < 0.0001). All patients benefitted from NIMV usage, regardless of age, site of symptoms onset and presence of cognitive impairment.
Conclusions:
We demonstrate that NIMV significantly improves the rate of disease progression in ALS, regardless of age at symptoms onset, disease duration, presence of bulbar dysfunction or cognitive impairment. Our results reinforce the importance of not delaying NIMV initiation. In addition, NIMV-induced slowing of disease progression should also be accounted for when evaluating clinical trial outcomes. Further studies are required to optimize the effectiveness of respiratory care on disease progression in ALS.