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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.
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
URL: http://dx.doi.org/10.1016/S1474-4422(05)70326-4
DOI: 10.1016/S1474-4422(05)70326-4
PubMed id: 16426990
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