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Intervention Review

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Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease

  1. Aleksandar Radunovic1,*,
  2. Djillali Annane2,
  3. Muhammad K Rafiq3,
  4. Naveed Mustfa4

Editorial Group: Cochrane Neuromuscular Group

Published Online: 28 MAR 2013

DOI: 10.1002/14651858.CD004427.pub3


How to Cite

Radunovic A, Annane D, Rafiq MK, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database of Systematic Reviews 2013, Issue 3. Art. No.: CD004427. DOI: 10.1002/14651858.CD004427.pub3.

Author Information

  1. 1

    Royal London Hospital, Barts and the London MND Centre, London, UK

  2. 2

    Hôpital Raymond Poincaré, Assistance Publique - Hôpitaux de Paris, Critical Care Department, Garches, Ile de France, France

  3. 3

    University of Sheffield, Academic Neurology Unit, Sheffield, UK

  4. 4

    City General, University Hospital of North Staffordshire, Department of Respiratory Medicine, Stoke-on-Trent, UK

*Aleksandar Radunovic, Barts and the London MND Centre, Royal London Hospital, Whitechapel, London, E1 1BB, UK. Aleksandar.Radunovic@bartshealth.nhs.uk.

Publication History

  1. Publication Status: New search for studies and content updated (no change to conclusions)
  2. Published Online: 28 MAR 2013

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This is not the most recent version of the article. View current version (06 OCT 2017)

References

References to studies included in this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to ongoing studies
  4. Additional references
  5. References to other published versions of this review
Bourke 2006 {published data only}
  • Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurology 2006;5(2):140-7. [PUBMED: 16426990]
Jackson 2001 {published data only}
  • Jackson CE, Rosenfeld J, Moore DH, Bryan WW, Barohn RJ, Wrench M, et al. A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. Journal of the Neurological Sciences 2001;191(1-2):75-8. [PUBMED: 11676995]

References to studies excluded from this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to ongoing studies
  4. Additional references
  5. References to other published versions of this review
Aboussouan 1997 {published data only}
  • Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H. Effect of non invasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Annals of Internal Medicine 1997;127(6):450-3.
Bach 1993 {published data only}
Buhr-Schinner 1999 {published data only}
  • Buhr-Schinner H, Laier-Groeneveld G, Criée CP. Amyotrophic lateral sclerosis and nasal mechanical ventilation. Medizinische Klinik (Munich) 1999;94(1 Spec No):102-4.
Cazzoli 1996 {published data only}
  • Cazzolli PA, Oppenheimer EA. Home mechanical ventilation for amyotrophic lateral sclerosis: nasal compared to tracheostomy-intermittent positive pressure ventilation. Journal of the Neurological Sciences 1996;139(Suppl):123-8.
Cedarbaum 2001 {published data only}
David 1997 {published data only}
Goulon 1989 {published data only}
  • Goulon M, Goulon-Goëau C. Amyotrophic lateral sclerosis and respiratory support [Sclérose latérale amyotrophique et assistance respiratoire]. Revue Neurologique 1989;145(4):293-8.
Kamimoto 1989 {published data only}
  • Kamimoto K, Murakam N, Muroga T, Matsubara M, Yamamoto M. A comparative study between amyotrophic lateral sclerosis patients with and without mechanical ventilation. Clinical Neurology 1989;29(8):989-93.
Kleopa 1999 {published data only}
  • Kleopa KA, Sherman M, Neal B, Romano GJ, Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. Journal of the Neurological Sciences 1999;164(1):82-8.
Lo Coco 2006 {published data only}
Lo Coco 2007 {published data only}
  • Lo Coco D, Marchese S, La Bella V, Piccoli T, Lo Coco A. The amyotrophic lateral sclerosis functional rating scale predicts survival time in amyotrophic lateral sclerosis patients on invasive mechanical ventilation. Chest 2007;132(1):64-9.
Lyall 2001b {published data only}
  • Lyall RA, Donaldson N, Fleming T, Wood C, Newsom-Davis I, Polkey MI, et al. A prospective study of quality of life in ALS patients treated with non-invasive ventilation. Neurology 2001;57(1):153-6.
Newsom-Davis 2001 {published data only}
  • Newsom-Davis IC, Lyall RA, Leigh PN, Moxham J, Goldstein LH. The effect of non-invasive positive pressure ventilation (NIPPV) on cognitive function in ALS: a prospective study. Journal of Neurology, Neurosurgery and Psychiatry 2001;71(4):482-7.
Perez 2003 {published data only}
  • Perez T, Salachas F. Early nasal ventilation in amyotrophic lateral sclerosis: impact on survival and quality of life (the VNP-SLA study). Revue des Maladies Respiratoires 2003;20(4):589-98.
Pinto 1995 {published data only}
  • Pinto AC, Evangelista T, Carvalho M, Alves MA, Sales Luís ML. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. Journal of the Neurological Sciences 1995;129(Suppl):19-26.
Pinto 1999 {published data only}
  • Pinto AC, Alves M, Nogueira A, Evangelista T, Carvalho J, Coelho A, et al. Can amyotrophic lateral sclerosis patients with respiratory insufficiency exercise?. Journal of the Neurological Sciences 1999;169(1-2):69-75.
Pinto 2003 {published data only}
  • Pinto A, de Carvalho M, Evangelista T, Lopes A, Sales-Luis L. Nocturnal pulse oximetry: a new approach to establish the appropriate time for non-invasive ventilation in ALS patients. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders 2003;4(1):31-5.
Saito 1999 {published data only}
  • Saito T. Mechanical ventilation for amyotrophic lateral sclerosis - making a comparison between hospital and home care. Rinsho Shinkeigaku - Clinical Neurology 1999;39(1):70-1.
Shoesmith 2007 {published data only}
  • Shoesmith CL, Findlater K, Rowe A, Strong MJ. Prognosis of amyotrophic lateral sclerosis with respiratory onset. Journal of Neurology, Neurosurgery and Psychiatry 2007;78(6):629-31.
Sivak 1982 {published data only}
Winterholler 2001 {published data only}
  • Winterholler MG, Erbguth FJ, Hecht MJ, Heuss D, Neundörfer B. Survival with artificial respiration at home. An open, prospective study on home ventilation for neuromuscular diseases, in particular, the situation of ALS patients. Nervenarzt 2001;72(4):293-301.

References to ongoing studies

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to ongoing studies
  4. Additional references
  5. References to other published versions of this review
NCT00386464 {published data only}
  • NCT00386464. Noninvasive ventilation in ALS patients with mild respiratory involvement. clinicaltrials.gov/show/NCT00386464 (accessed 10 March 2005).
NCT00537446 {published data only}
  • NCT00537446. Effect of noninvasive positive pressure ventilation on pulmonary function testing in amyotrophic lateral sclerosis. clinicaltrials.gov/show/NCT00537446 (accessed 10 March 2009).
NCT00560287 {published data only}
  • NCT00560287. Non-invasive ventilation in amyotrophic lateral sclerosis. clinicaltrials.gov/show/NCT00560287 (accessed 10 March 2009).
NCT00580593 {published data only}
  • NCT00580593. Pilot placebo-controlled trial of early noninvasive ventilation for amyotrophic lateral sclerosis. clinicaltrials.gov/show/NCT00580593 (accessed 210 March 2009).
NCT01363882 {published data only}
  • NCT01363882. Polysomnography-directed noninvasive ventilation in amyotrophic lateral sclerosis (ALS). clinicaltrials.gov/show/NCT01363882 (accessed 23 January 2013).
NCT01641965 {published data only}
  • NCT01641965. Impact of early non invasive ventilation in amyotrophic lateral sclerosis (ALS) patients. http://clinicaltrials.gov/ct2/show/NCT01641965 (accessed 23 January 2013).

Additional references

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to ongoing studies
  4. Additional references
  5. References to other published versions of this review
Aboussouan 2001
ALSFRS 1996
  • The ALS CNTF (ACTS) phase I-II Study Group. The Amyotrophic Lateral Sclerosis Functional Rating Scale. Assessment of activities of daily living in patients with amyotrophic lateral sclerosis. Archives of Neurology 1996;53(2):141-7.
Annane 2007
Bach 1995
Bach 2002
Borasio 1998
  • Borasio GD, Gelinas DF, Yanagisawa N. Mechanical ventilation in ALS: a cross-cultural perspective. Journal of Neurology 245;Suppl 2:S7-12.
Borasio 2001
  • Borasio GD, Shaw PJ, Hardiman, Ludolph AC, Sales Luis M, Silani V. Standards of palliative care for patients with amyotrophic lateral sclerosis: results of a European survey. Amyotrophic lateral sclerosis and other motor neuron disorders 2001;2(3):159-64.
Bourke 2001
Bourke 2002
  • Bourke SC, Williams TL, Bullock RE, Gibson GJ, Shaw PJ. Non-invasive ventilation in motor neuron disease: current UK practice. Amyotrophic lateral sclerosis and other motor neuron disorders 2002;3(3):145-9.
Bradley 2001
  • Bradley WG, Anderson F, Bromberg M, Gutmann C, Harati Y, Ross M, et al. Current management of ALS: comparison of the ALS CARE Database and the AAN Practice Parameter. The American Academy of Neurology. Neurology 2001;57(3):500-4.
Brooks 1994
  • Brooks BR. El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial 'Clinical limits of amyotrophic lateral sclerosis' workshop contributors. Journal of Neurological Sciences 1994;124(Suppl):96-107.
Brooks 2000
  • Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis and other motor neuron disorders 2000;1(5):293-9.
Carratu 2009
  • Carratù P, Spicuzza L, Cassano A, Maniscalco M, Gadaleta F, Lacedonia D, et al. Early treatment with noninvasive positive pressure ventilation prolongs survival in amyotrophic lateral sclerosis patients with nocturnal respiratory insufficiency. Orphanet Journal of Rare Diseases 2009;4(10). [DOI: 10.1186/1750-1172-4-10]
Cedarbaum 1999
  • Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond E, et al. The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). Journal of the Neurological Sciences 1999;169(1-2):13-21.
Chaudri 2000
  • Chaudri MB, Liu C, Watson L, Jefferson D, Kinnear WJ. Sniff nasal pressure as a marker of respiratory function in motor neuron disease. European Respiratory Journal 2000;15(3):539-42.
Chio 2001
Fitting 1999
Haverkamp 1995
  • Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population.Validation of a scoring system and a model of survival prediction. Brain 1995;118(Pt 3):707-19.
Hayashi 1997
  • Hayashi H. Ventilatory support: Japanese experience. Journal of the Neurological Sciences 152;Suppl 1:S97-100.
Hein 1997
Hein 1999
  • Hein H, Schucher B, Magnussen H. Intermittent assisted ventilation in neuromuscular diseases: course and quality of life. Pneumonologie 1999;Suppl 2:S89-90. [MEDLINE: 20079874]
Higgins 2008
  • Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions. Chichester (UK): John Wiley & Sons, 2008.
Higgins 2011
  • Higgins JPT, Altman DG, Sterne JAC. Chapter 8: Assessing risk of bias in included studies. Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 (updated March 2011). The Cochrane Collaboration, 2011. Available from www.cochrane-handbook.org.
Kawata 2008
  • Kawata A, Mizoguchi K, Hayashi H. A nationwide survey of ALS patients on tracheostomy positive pressure ventilation (TPPV) who developed a totally locked-in state (TLS) in Japan. Rinsho Shinkeigaku 2008;48(7):476-80.
Lechtzin 2002
  • Lechtzin N, Wiener CM, Shade DM, Clawson L, Diette GB. Spirometry in the supine position improves the detection of diaphragmatic weakness in patients with amyotrophic lateral sclerosis. Chest 2002;121(2):436-42.
Lyall 2001a
Mehta 2001
Melo 1999
  • Melo J, Homma A, Iturriaga E, Frierson L, Amato A, Anzueto A, et al. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. Journal of the Neurological Sciences 1999;169(1-2):114-7.
Moss 1993
  • Moss AH, Casey P, Stocking CB, Roos RP, Brooks BR, Siegler M. Home ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family, and physician attitudes. Neurology 1993;43(2):438-43.
Moss 1996
  • Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roose RP, Stocking CB, et al. Patients with amyotrophic lateral sclerosis receiving long-term mechanical ventilation. Advance care planning and outcomes. Chest 1996;110(1):249-55.
NICE 2010
  • National Institute for Health and Clinical Excellence. Motor neurone disease - non-invasive ventilation (CG105). London: National Institute for Health and Clinical Excellence 2010.
Norris 1974
O'Neill 2012
  • O'Neill CL, Williams TL, Peel ET, McDermott CJ, Shaw PJ, Gibson GJ, et al. Non-invasive ventilation in motor neuron disease: an update of current UK practice. Journal of Neurology Neurosurgery and Psychiatry 2012;83(4):371-6.
Parmar 1998
RevMan 2012 [Computer program]
  • The Nordic Cochrane Centre, The Cochrane Collaboration. Review Manager (RevMan). Version 5.2. Copenhagen: The Nordic Cochrane Centre, The Cochrane Collaboration, 2012.
Stambler 1998
Turner 2003
  • Turner MR, Parton MJ, Shaw CE, Leigh PN, Al-Chalabi A. Prolonged survival in motor neuron disease: a descriptive study of the King's Database 1990-2000. Journal of Neurology,Neurosurgery and Psychiatry 2003;74(7):995-7.
Varrato 2001
Vitacca 1997
  • Vitacca M, Clini E, Facchetti D, Pagani M, Polani M, Porta R, et al. Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis. European Respiratory Journal 1997;10(7):1614-21.
Worms 2001
Yamaguchi 2001
  • Yamaguchi M, Hideaki H, Kuniko H. Ventilatory support in Japan: A new life with ALS and a positive approach to living with the disease. Amyotrophic lateral sclerosis and other motor neuron disorders 2001;2(4):209-11.

References to other published versions of this review

  1. References to studies included in this review
  2. References to studies excluded from this review
  3. References to ongoing studies
  4. Additional references
  5. References to other published versions of this review
Leigh 2003
  • Leigh PN, Annane D, Jewitt K, Mustfa N. Mechanical ventilation for amyotrophic lateral sclerosis/motor neuron disease (Protocol). Cochrane Database of Systematic Reviews 2003, Issue 4. [DOI: 10.1002/14651858.CD004427]
Radunovic 2009