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Evidence summaries

Mechanical Ventilation for Amyotrophic Lateral Sclerosis

Non-invasive mechanical ventilation appears to significantly prolong survival and improve or maintain quality of life in people with amyotrophic lateral sclerosis who have normal or only moderately impaired bulbar function. Sleep-related symptoms appear to significantly improve in those who have severe bulbar impairment. Level of evidence: "B"

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).

    References

    • Radunovic A, Annane D, Rafiq MK et al. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2017;(10):CD004427. [PubMed]

Primary/Secondary Keywords