Patients with amyotrophic lateral sclerosis (ALS) develop chronic respiratory failure, managed with non-invasive ventilation (NIV) to improve survival and quality of life. We investigated if baseline clinical features can predict outcome. Baseline data of our ALS cohort, initiated on NIV between 01/04/21 and 01/04/22, were included to determine the relationship between baseline clinical characteristics and mortality.  
 
35 patients  were included (57% male; median(IQR) age 68(56-77) yrs); 69% limb-onset and 31% bulbar-onset). Baseline data are shown in Tab 1. Survival time was 389 (308-470) days. 12-month mortality was 42%. In a univariate Cox proportional hazard model, decreasing ALSFRS-R (HR 0.93 [0.89,0.99]; p=0.02), increasing seated Borg dyspnoea scale (HR 1.33 [1.04,1.70]; p=0.02) were associated with shorter survival from NIV initiation.  Although decreasing SNIP (HR 0.96 [0.92,0.99]; p=0.01), PEF (HR 0.99 [0.98,0.99]; p=0.02) and CPEF (HR 0.99 [0.98,0.99]; p=0.02) were associated with reduced survival, the effect size was much less than ALSFRS-R and seated Borg dyspnoea scale.
 
Although measures of respiratory function predict mortality post NIV initiation in ALS, simple measures of dyspnoea whilst seated and functional status may have greater utility at predicting outcome.

Table 1