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Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: A randomised controlled trial

Lookup NU author(s): Professor Stephen BourkeORCiD, Dr Timothy Williams, Dr Robert Bullock, Professor Pamela Shaw, Emeritus Professor John Gibson


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Background: Few patients with amyotrophic lateral sclerosis currently receive non-invasive ventilation (NIV), reflecting clinical uncertainty about the role of this intervention. We aimed to assess the effect of NIV on quality of life and survival in amyotrophic lateral sclerosis in a randomised controlled trial. Methods: 92 of 102 eligible patients participated. They were assessed every 2 months and randomly assigned to NIV (n=22) or standard care (n=19) when they developed either orthopnoea with maximum inspiratory pressure less than 60% of that predicted or symptomatic hypercapnia. Primary validated quality-of-life outcome measures were the short form 36 mental component summary (MCS) and the sleep apnoea quality-of-life index symptoms domain (sym). Both time maintained above 75% of baseline (TiMCS and Tisym) and mean improvement (μMCS and μsym) were measured. Findings: NIV improved T iMCS, Tisym, μMCS, μsym, and survival in all patients and in the subgroup with better bulbar function (n=20). This subgroup showed improvement in several measures of quality of life and a median survival benefit of 205 days (p=0·006) with maintained quality of life for most of this period. NIV improved some quality-of-life indices in those with poor bulbar function, including μsym (p=0·018), but conferred no survival benefit. Interpretation: In patients with amyotrophic lateral sclerosis without severe bulbar dysfunction, NIV improves survival with maintenance of, and improvement in, quality of life. The survival benefit from NIV in this group is much greater than that from currently available neuroprotective therapy. In patients with severe bulbar impairment, NIV improves sleep-related symptoms, but is unlikely to confer a large survival advantage.

Publication metadata

Author(s): Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ

Publication type: Article

Publication status: Published

Journal: The Lancet Neurology

Year: 2006

Volume: 5

Issue: 2

Pages: 140-147

Print publication date: 01/02/2006

ISSN (print): 1474-4422

ISSN (electronic): 1474-4465

Publisher: The Lancet Publishing Group


DOI: 10.1016/S1474-4422(05)70326-4

PubMed id: 16426990


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