Non invasive ventilation for people with amyotrophic lateral sclerosis or motor neuron disease

Management of amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND), has evolved rapidly in the last ten years and although still incurable, ALS is not untreatable. In this review we examined the evidence from two randomised trials, involving 54 participants in total, of non-invasive ventilation (using a face or nasal mask and a small portable ventilator) in people with ALS. Complete data were only available from a single trial of 41 participants. The results of this trial indicate that non-invasive ventilation significantly prolongs survival and improves or maintains quality of life. The survival benefit from non-invasive ventilation was shown to be much greater than that of riluzole in those people with ALS who had normal or only moderately impaired bulbar function (impairments to the muscles used for speaking, chewing and swallowing). Sleep-related symptoms significantly improved in those people with severe bulbar impairment but NIV did not prolong survival. Neither trial reported on adverse effects due to the intervention. Future studies should examine the health economics of non-invasive ventilation and factors influencing access to non-invasive ventilation.