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In amyotrophic lateral sclerosis (ALS), non-invasive ventilation (NIV) improves survival; however, the phenotype of patients who are most likely to benefit remains unclear. This study aimed to identify predictors of survival at initiation of NIV.

A retrospective review (September 2018 - June 2022) of patients with ALS initiated on NIV at a tertiary referral centre was conducted. Survival analysis was performed with a Cox regression model.

105 patients (56% male) with ALS (69% limb-onset, 31% bulbar-onset) received new NIV treatment. Age (mean±SD) at initiation was 66±11 yrs, BMI was 23±6 kg/m2. Daytime CO₂ at initiation was 5.7±0.8 kPa, sniff nasal inspiratory pressure (SNIP) was 25±19 cmH2O, and peak cough flow (PCF) was 197±100 L/min. At 3-months following initiation, median (IQR) NIV adherence was 1.0 (0-5.5) hours per night. 28% were using NIV for more than four hours per night, while 43% used it for …