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Abstract Details

Non-Invasive Mechanical Ventilation slows motor decline in Amyotrophic Lateral Sclerosis: a population-based study
Neuromuscular and Clinical Neurophysiology (EMG)
P10 - Poster Session 10 (8:00 AM-9:00 AM)
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.
To test whether Non-Invasive Mechanical Ventilation (NIMV) use improved the rate of motor decline among ALS patients.
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.

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. 

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.

Maurizio Grassano, MD (Dept. of Neuroscience, University of Turin)
Dr. Grassano has received research support from American Brain Foundation, ALS Association and American Academy of Neurology.
Emanuele Koumantakis, MD (Università degli Studi di Torino) Dr. Koumantakis has nothing to disclose.
Cristina Moglia (University of Torino) Cristina Moglia has nothing to disclose.
Antonio Canosa Antonio Canosa has nothing to disclose.
Umberto Manera, MD (Department of Neuroscience "Rita Levi Montalcini" - University of Torino) Dr. Manera has nothing to disclose.
Rosario Vasta, MD (University of Turin, Department of Neurosciences) Dr. Vasta has nothing to disclose.
Francesca Palumbo Francesca Palumbo has nothing to disclose.
Stefano Callegaro Mr. Callegaro has nothing to disclose.
Giulia Marchese, Other (SLA Center Turin) Dr. Marchese has nothing to disclose.
Paolina Salamone Paolina Salamone has nothing to disclose.
Giuseppe Fuda, Other Dr. Fuda has nothing to disclose.
Federico Casale Federico Casale has nothing to disclose.
Andrea Calvo, MD, PhD, FAAN (Dept. of Neuroscience, University of Turin) Dr. Calvo has nothing to disclose.
Gabriele Mora, MD Dr. Mora has nothing to disclose.
Adriano Chio, MD, FAAN (Dept. of Neuroscience, University of Turin) 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.