A Cochrane review [Abstract] 1 to examine the efficacy of mechanical ventilation in improving survival, on disease progression and quality of life in amyotrophic lateral sclerosis (ALS) included 2 studies with a total of 54 subjects. The results of the review were based on a single study of 41 participants. The overall median survival in the whole cohort after initiation of assisted ventilation was significantly different between the non-invasive ventilation and standard care groups (p=0.0062) with a median survival for the non-invasive ventilation group patients of 48 days longer than the standard care group participants. Non-invasive ventilation significantly improved survival and quality of life in the subgroup with normal to moderately impaired bulbar function. Non-invasive ventilation did not prolong survival in patients with poor bulbar function although it showed significant improvement in the mean symptoms domain of the sleep apnoea quality-of-life index but not in the Short Form-36 quality of life mental component summary score.
Comment: The quality of evidence is downgraded by imprecise results (few patients).
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