• Nimish J Thakore, Brittany R Lapin, Erik P Pioro, and Loutfi S Aboussouan.
• From the Department of Neurology/Neuromuscular Center, Neurological Institute (N.J.T., E.P.P.), Quantitative Health Sciences/Neurological Institute Center for Outcomes Research and Evaluation (NICORE) (B.R.L.), Department of Neurosciences, Lerner Research Institute (E.P.P.), and Department of Pulmonary Medicine, Respiratory Institute (L.S.A.), Cleveland Clinic, OH. thakorn@ccf.org.
• Neurology. 2019 Jul 16; 93 (3): e306-e316.

ObjectiveWe sought to examine prevalence and predictors of noninvasive ventilation (NIV) in a composite cohort of patients with amyotrophic lateral sclerosis (ALS) followed in a clinical trials setting (Pooled Resource Open-Access ALS Clinical Trials database).MethodsNIV initiation and status were ascertained from response to question 12 of the revised ALS Functional Rating Scale (ALSFRS-R). Factors affecting NIV use in patients with forced vital capacity (FVC) ≤50% of predicted were examined. Predictors of NIV were evaluated by Cox proportional hazard models and generalized linear mixed models.ResultsAmong 1,784 patients with 8,417 simultaneous ALSFRS-R and FVC% measures, NIV was used by 604 (33.9%). Of 918 encounters when FVC% ≤50%, NIV was reported in 482 (52.5%). Independent predictors of NIV initiation were lower FVC% (hazard ratio [HR] 1.27, 95% confidence interval [CI] 1.17-1.37 for 10% drop), dyspnea (HR 2.62, 95% CI 1.87-3.69), orthopnea (HR 4.09, 95% CI 3.02-5.55), lower bulbar and gross motor subscores of ALSFRS-R (HRs 1.09 [95% CI 1.03-1.14] and 1.13 [95% CI 1.07-1.20], respectively, per point), and male sex (HR 1.73, 95% CI 1.31-2.28). Adjusted for other variables, bulbar onset did not significantly influence time to NIV (HR 0.72, 95% CI 0.47-1.08). Considerable unexplained variability in NIV use was found.ConclusionNIV use was lower than expected in this ALS cohort that was likely to be optimally managed. Absence of respiratory symptoms and female sex may be barriers to NIV use. Prospective exploration of factors affecting adoption of NIV may help bridge this gap and improve care in ALS.© 2019 American Academy of Neurology.

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