Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2002 Jun;72(6):752–756. doi: 10.1136/jnnp.72.6.752

Outcome of ventilatory support for acute respiratory failure in motor neurone disease

M Bradley 1, R Orrell 1, J Clarke 1, A Davidson 1, A Williams 1, D Kullmann 1, N Hirsch 1, R Howard 1
PMCID: PMC1737909  PMID: 12023419

Abstract

Objectives: To review the outcome of acute ventilatory support in patients presenting acutely with respiratory failure, either with an established diagnosis of motor neurone disease (MND) or with a clinical event where the diagnosis of MND has not yet been established.

Methods: Outcome was reviewed in 24 patients with respiratory failure due to MND who received endotracheal intubation and intermittent positive pressure ventilation either at presentation or as a result of the unexpected development of respiratory failure. Patients presenting to local hospitals with acute respiratory insufficiency and requiring tracheal intubation, ventilatory support, and admission to an intensive therapy unit (ITU) before transfer to a regional respiratory care unit were selected. Clinical features of presentation, management, and outcome were studied.

Results: 24 patients with MND were identified, all being intubated and ventilated acutely within hours of presentation. 17 patients (71%) were admitted in respiratory failure before the diagnosis of MND had been made; the remaining seven patients (29%) were already known to have MND but deteriorated rapidly such that intubation and ventilation were initiated acutely. Seven patients (29%) died on ITU (between seven and 54 days after admission). 17 patients (71%) were discharged from ITU. 16 patients (67%) received long term respiratory support and one patient required no respiratory support following tracheal extubation. The daily duration of support that was required increased gradually with time.

Conclusion: When a patient with MND is ventilated acutely, with or without an established diagnosis, independence from the ventilator is rarely achieved. Almost all of these patients need long term ventilatory support and the degree of respiratory support increases with time as the disease progresses. The aim of management should be weaning the patient to the minimum support compatible with symptomatic relief and comfort. Respiratory failure should be anticipated in patients with MND when the diagnosis has been established.

Full Text

The Full Text of this article is available as a PDF (123.6 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aboussouan L. S., Khan S. U., Meeker D. P., Stelmach K., Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997 Sep 15;127(6):450–453. doi: 10.7326/0003-4819-127-6-199709150-00006. [DOI] [PubMed] [Google Scholar]
  2. Albert S. M., Murphy P. L., Del Bene M. L., Rowland L. P. A prospective study of preferences and actual treatment choices in ALS. Neurology. 1999 Jul 22;53(2):278–283. doi: 10.1212/wnl.53.2.278. [DOI] [PubMed] [Google Scholar]
  3. Albert S. M., Murphy P. L., Del Bene M. L., Rowland L. P. Prospective study of palliative care in ALS: choice, timing, outcomes. J Neurol Sci. 1999 Oct 31;169(1-2):108–113. doi: 10.1016/s0022-510x(99)00227-0. [DOI] [PubMed] [Google Scholar]
  4. Annane D., Korach J. M., Templier F., Durand M. C., Dinet-Busso N., Le Corre A., de Lattre J., Gajdos P. Diaphragmatic paralysis preceding amyotrophic lateral sclerosis. Lancet. 1993 Oct 16;342(8877):990–991. doi: 10.1016/0140-6736(93)92037-t. [DOI] [PubMed] [Google Scholar]
  5. Borasio G. D., Voltz R., Miller R. G. Palliative care in amyotrophic lateral sclerosis. Neurol Clin. 2001 Nov;19(4):829–847. doi: 10.1016/s0733-8619(05)70049-9. [DOI] [PubMed] [Google Scholar]
  6. Brooks B. R. Natural history of ALS: symptoms, strength, pulmonary function, and disability. Neurology. 1996 Oct;47(4 Suppl 2):S71–S82. doi: 10.1212/wnl.47.4_suppl_2.71s. [DOI] [PubMed] [Google Scholar]
  7. Carrey Z., Gottfried S. B., Levy R. D. Ventilatory muscle support in respiratory failure with nasal positive pressure ventilation. Chest. 1990 Jan;97(1):150–158. doi: 10.1378/chest.97.1.150. [DOI] [PubMed] [Google Scholar]
  8. Chen R., Grand'Maison F., Strong M. J., Ramsay D. A., Bolton C. F. Motor neuron disease presenting as acute respiratory failure: a clinical and pathological study. J Neurol Neurosurg Psychiatry. 1996 Apr;60(4):455–458. doi: 10.1136/jnnp.60.4.455. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. David W. S., Bundlie S. R., Mahdavi Z. Polysomnographic studies in amyotrophic lateral sclerosis. J Neurol Sci. 1997 Oct;152 (Suppl 1):S29–S35. doi: 10.1016/s0022-510x(97)00241-4. [DOI] [PubMed] [Google Scholar]
  10. Durelli L., Bongioanni M. R., Ferrero B., Ferri R., Imperiale D., Bradac G. B., Bergui M., Geuna M., Bergamini L., Bergamasco B. Interferon alpha-2a treatment of relapsing-remitting multiple sclerosis: disease activity resumes after stopping treatment. Neurology. 1996 Jul;47(1):123–129. doi: 10.1212/wnl.47.1.123. [DOI] [PubMed] [Google Scholar]
  11. Evangelista T., Carvalho M., Pinto A., Luís M. de L. Phrenic nerve conduction in amyotrophic lateral sclerosis. J Neurol Sci. 1995 May;129 (Suppl):35–37. doi: 10.1016/0022-510x(95)00057-9. [DOI] [PubMed] [Google Scholar]
  12. Fallat R. J., Jewitt B., Bass M., Kamm B., Norris F. H., Jr Spirometry in amyotrophic lateral sclerosis. Arch Neurol. 1979 Feb;36(2):74–80. doi: 10.1001/archneur.1979.00500380044004. [DOI] [PubMed] [Google Scholar]
  13. Gelinas D. F., O'Connor P., Miller R. G. Quality of life for ventilator-dependent ALS patients and their caregivers. J Neurol Sci. 1998 Oct;160 (Suppl 1):S134–S136. doi: 10.1016/s0022-510x(98)00212-3. [DOI] [PubMed] [Google Scholar]
  14. Hadjikoutis S., Eccles R., Wiles C. M. Coughing and choking in motor neuron disease. J Neurol Neurosurg Psychiatry. 2000 May;68(5):601–604. doi: 10.1136/jnnp.68.5.601. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Hardiman O. Symptomatic treatment of respiratory and nutritional failure in amyotrophic lateral sclerosis. J Neurol. 2000 Apr;247(4):245–251. doi: 10.1007/s004150050578. [DOI] [PubMed] [Google Scholar]
  16. Haverkamp L. J., Appel V., Appel S. H. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain. 1995 Jun;118(Pt 3):707–719. doi: 10.1093/brain/118.3.707. [DOI] [PubMed] [Google Scholar]
  17. Hayashi H., Kato S., Kawada A. Amyotrophic lateral sclerosis patients living beyond respiratory failure. J Neurol Sci. 1991 Sep;105(1):73–78. doi: 10.1016/0022-510x(91)90121-m. [DOI] [PubMed] [Google Scholar]
  18. Hayashi H., Kato S. Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci. 1989 Oct;93(1):19–35. doi: 10.1016/0022-510x(89)90158-5. [DOI] [PubMed] [Google Scholar]
  19. Hayashi H. Ventilatory support: Japanese experience. J Neurol Sci. 1997 Oct;152 (Suppl 1):S97–100. doi: 10.1016/s0022-510x(97)00254-2. [DOI] [PubMed] [Google Scholar]
  20. Hill N. S. Noninvasive positive pressure ventilation in neuromuscular disease. Enough is enough! Chest. 1994 Feb;105(2):337–338. doi: 10.1378/chest.105.2.337. [DOI] [PubMed] [Google Scholar]
  21. Hillberg R. E., Johnson D. C. Noninvasive ventilation. N Engl J Med. 1997 Dec 11;337(24):1746–1752. doi: 10.1056/NEJM199712113372407. [DOI] [PubMed] [Google Scholar]
  22. Howard R. S., Wiles C. M., Loh L. Respiratory complications and their management in motor neuron disease. Brain. 1989 Oct;112(Pt 5):1155–1170. doi: 10.1093/brain/112.5.1155. [DOI] [PubMed] [Google Scholar]
  23. Kimura K., Tachibana N., Kimura J., Shibasaki H. Sleep-disordered breathing at an early stage of amyotrophic lateral sclerosis. J Neurol Sci. 1999 Mar 15;164(1):37–43. doi: 10.1016/s0022-510x(99)00044-1. [DOI] [PubMed] [Google Scholar]
  24. Kleopa K. A., Sherman M., Neal B., Romano G. J., Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999 Mar 15;164(1):82–88. doi: 10.1016/s0022-510x(99)00045-3. [DOI] [PubMed] [Google Scholar]
  25. Lyall R. A., Donaldson N., Polkey M. I., Leigh P. N., Moxham J. Respiratory muscle strength and ventilatory failure in amyotrophic lateral sclerosis. Brain. 2001 Oct;124(Pt 10):2000–2013. doi: 10.1093/brain/124.10.2000. [DOI] [PubMed] [Google Scholar]
  26. Melo J., Homma A., Iturriaga E., Frierson L., Amato A., Anzueto A., Jackson C. Pulmonary evaluation and prevalence of non-invasive ventilation in patients with amyotrophic lateral sclerosis: a multicenter survey and proposal of a pulmonary protocol. J Neurol Sci. 1999 Oct 31;169(1-2):114–117. doi: 10.1016/s0022-510x(99)00228-2. [DOI] [PubMed] [Google Scholar]
  27. Miller R. G., Rosenberg J. A., Gelinas D. F., Mitsumoto H., Newman D., Sufit R., Borasio G. D., Bradley W. G., Bromberg M. B., Brooks B. R. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology. 1999 Apr 22;52(7):1311–1323. doi: 10.1212/wnl.52.7.1311. [DOI] [PubMed] [Google Scholar]
  28. Pinto A. C., Evangelista T., Carvalho M., Alves M. A., Sales Luís M. L. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci. 1995 May;129 (Suppl):19–26. doi: 10.1016/0022-510x(95)00052-4. [DOI] [PubMed] [Google Scholar]
  29. Polkey M. I., Lyall R. A., Green M., Nigel Leigh P., Moxham J. Expiratory muscle function in amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 1998 Sep;158(3):734–741. doi: 10.1164/ajrccm.158.3.9710072. [DOI] [PubMed] [Google Scholar]
  30. Ringel S. P., Murphy J. R., Alderson M. K., Bryan W., England J. D., Miller R. G., Petajan J. H., Smith S. A., Roelofs R. I., Ziter F. The natural history of amyotrophic lateral sclerosis. Neurology. 1993 Jul;43(7):1316–1322. doi: 10.1212/wnl.43.7.1316. [DOI] [PubMed] [Google Scholar]
  31. Schiffman P. L., Belsh J. M. Pulmonary function at diagnosis of amyotrophic lateral sclerosis. Rate of deterioration. Chest. 1993 Feb;103(2):508–513. doi: 10.1378/chest.103.2.508. [DOI] [PubMed] [Google Scholar]
  32. Stambler N., Charatan M., Cedarbaum J. M. Prognostic indicators of survival in ALS. ALS CNTF Treatment Study Group. Neurology. 1998 Jan;50(1):66–72. doi: 10.1212/wnl.50.1.66. [DOI] [PubMed] [Google Scholar]
  33. de Carvalho M., Matias T., Coelho F., Evangelista T., Pinto A., Luís M. L. Motor neuron disease presenting with respiratory failure. J Neurol Sci. 1996 Aug;139 (Suppl):117–122. doi: 10.1016/0022-510x(96)00089-5. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES