This randomised controlled trial allocated patients with amyotrophic lateral sclerosis to either standard care (n = 19) or non-invasive ventilation (NIV, n = 22). Patients were randomised when they developed orthopnoea with a maximum inspiratory pressure of <60% or symptomatic daytime hypercapnia. They were assessed with the SF-36 and sleep apnoea quality of life index (SAQLI) scores. The authors found that quality of life scores were maintained at 75% above baseline for longer in the patients randomised to NIV (192 v 46 days, p = 0.0013). Furthermore, in the patients with good bulbar function there was a median survival benefit of 205 days (p = 0.006). The subgroup with poor bulbar function had improved sleep related symptoms but no survival benefit.

This study confirms that, rather than prolonging suffering, NIV extends survival in motor neuron disease with improved quality of life in those with good bulbar function, and improves sleep symptoms in those with poor bulbar function.