Non-Invasive Mechanical Ventilation slows motor decline in Amyotrophic Lateral Sclerosis: a population-based study (P10-8.003)

Abstract

<h3>Objective:</h3> To test whether Non-Invasive Mechanical Ventilation (NIMV) use improved the rate of motor decline among ALS patients. <h3>Background:</h3> 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. <h3>Design/Methods:</h3> 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. <h3>Results:</h3> NIMV was associated with a ~30% reduction in the ΔALSFRS-R (post-NIMV difference in ΔALSFRS-R −0.46, 97.5% CI −0.43 to −0.49, p&lt;0.0001). The NIMV-related slowing was present on bulbar symptoms (post-NIMV difference in ΔALSFRS-R −0.08, 97.5% CI −0.065 to −0.010, p &lt; 0.0001) and even greater on spinal function (post-NIMV difference in ΔALSFRS-R −0.42, 97.5% CI −0.39 to −0.46, p &lt; 0.0001). All patients benefitted from NIMV usage, regardless of age, site of symptoms onset and presence of cognitive impairment. <h3>Conclusions:</h3> 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. <b>Disclosure:</b> Mr. Grassano has nothing to disclose. Dr. Koumantakis has nothing to disclose. Cristina Moglia has nothing to disclose. Antonio Canosa has nothing to disclose. Dr. Manera has nothing to disclose. Dr. Vasta has nothing to disclose. Francesca Palumbo has nothing to disclose. Mr. Callegaro has nothing to disclose. Dr. Marchese has nothing to disclose. Paolina Salamone has nothing to disclose. Dr. Fuda has nothing to disclose. Federico Casale has nothing to disclose. Dr. Calvo has nothing to disclose. Dr. Mora has nothing to disclose. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cytokinetics. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi. Dr. Chio has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen.