BACKGROUND: Respiratory failure and dysfunction are common problems in many neurodegenerative conditions. Although physiotherapists manage these problems, it is not known which treatments have been studied and their efficacy. OBJECTIVE: To review evidence on the management of respiratory problems in people with neurodegenerative conditions using the PRISMA approach. DATA SOURCES: Comprehensive searches were conducted using the following electronic databases from inception to May 2010: HUGEnet, SIGLE, British Library Direct, CINAHL, Medline, AMED and Web of Knowledge. Bibliographies of all studies and systematic reviews were searched by hand. STUDY SELECTION: Studies were selected based on: self-ventilating participants with neurodegenerative conditions; interventions aimed at improving respiratory function; and any valid and reliable measures of respiratory function as outcomes. STUDY APPRAISAL: Studies were appraised by one reviewer using the Critical Appraisal Skills Programme. Data were synthesised using a narrative approach. RESULTS: Thirty-five studies were included in the review. The strongest evidence was for the use of non-invasive ventilation for people with amyotrophic lateral sclerosis, although this was weak. The evidence for the use of respiratory muscle training and methods to increase peak cough flow showed a positive effect, but was also weak. CONCLUSION: There is weak evidence for the positive effects of physiotherapeutic interventions for respiratory problems in people with neurodegenerative conditions. Further work is necessary in specific neurodegenerative conditions to identify why respiratory problems occur, and larger scale studies should be undertaken to investigate management of these problems.

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