Vol 14, No 1 (2020)
Case report
Published online: 2020-05-08

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How to manage exacerbation of respiratory failure in a patient diagnosed with amyotrophic lateral sclerosis

Katarzyna Anna Blacharska-Krzanowska12, Kartarzyna Anna Strzępek12, Jarosław Woroń3, Teresa Weber-Lipiec2
Palliat Med Pract 2020;14(1):44-50.


Amyotrophic lateral sclerosis (ALS) is a debilitating neurodegenerative disease of unclear pathogenesis, which leads to the damage of motor neurons. The majority of patients survive two to five years after a diagnosis is made. Despite numerous clinical trials and some progress in understanding ALS pathology, no treatment that effectively inhibits the disease progression has been developed. The aim of this paper is to review a patient’s disease history from diagnosis, through the occurrence of respiratory failure features, until the start of non-invasive ventilation (NIV), including the assessment of the efficacy of this therapy during respiratory failure exacerbation. ALS is an incurable disease, but many of its symptoms may be relieved, and therapy should be aimed at quality of life improvement and maintaining the patient’s independence and comfort as long as possible. Better quality of life and longer survival are currently well-established advantages of NIV. Palliat Med Pract 2020; 14, 1: 44–50

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  1. Shaw PJ. Molecular and cellular pathways of neurodegeneration in motor neurone disease. J Neurol Neurosurg Psychiatry. 2005; 76(8): 1046–1057.
  2. Liu ZJ, Lin HX, Wei Q, et al. Genetic Spectrum and Variability in Chinese Patients with Amyotrophic Lateral Sclerosis. Aging Dis. 2019; 10(6): 1199–1206.
  3. Wei Q, Chen X, Zheng Z, et al. The predictors of survival in Chinese amyotrophic lateral sclerosis patients. Amyotroph Lateral Scler Frontotemporal Degener. 2015; 16(3-4): 237–244.
  4. Coan G, Mitchell CS. An Assessment of Possible Neuropathology and Clinical Relationships in 46 Sporadic Amyotrophic Lateral Sclerosis Patient Autopsies. Neurodegener Dis. 2015; 15(5): 301–312.
  5. Hollinger SK, Okosun IS, Mitchell CS, et al. Antecedent Disease is Less Prevalent in Amyotrophic Lateral Sclerosis. Neurodegener Dis. 2015; 15(2): 109–113.
  6. Hollinger SK, Okosun IS, Mitchell CS. Antecedent Disease and Amyotrophic Lateral Sclerosis: What Is Protecting Whom? Front Neurol. 2016; 7: 47.
  7. de Carvalho M, Dengler R, Eisen A, et al. Electrodiagnostic criteria for diagnosis of ALS. Clin Neurophysiol. 2008; 119(3): 497–503.
  8. Zoccolella S, Beghi E, Palagano G, et al. Analysis of survival and prognostic factors in amyotrophic lateral sclerosis: a population based study. Journal of Neurology, Neurosurgery & Psychiatry. 2008; 79(1): 33–37.
  9. Geevasinga N, Howells J, Menon P, et al. Amyotrophic lateral sclerosis diagnostic index: Toward a personalized diagnosis of ALS. Neurology. 2019; 92(6): e536–e547.
  10. Iłżecka J, Iłżecka J. Mechanizmy patogenetyczne stwardnienia bocznego zanikowego. Aktualn Neurol. 2012; 12: 222–235.
  11. Wijesekera LC, Leigh PN, Wijesekera LC, et al. Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 2009; 4: 3.
  12. Brooks BR, Miller RG, Swash M, et al. World Federation of Neurology Research Group on Motor Neuron Diseases, World Federation of Neurology Research Group on Motor Neuron Diseases. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000; 1(5): 293–299.
  13. Beeldman E, Raaphorst J, Klein Twennaar M, et al. The cognitive profile of ALS: a systematic review and meta-analysis update. J Neurol Neurosurg Psychiatry. 2016; 87(6): 611–619.
  14. Crockford C, Newton J, Lonergan K, et al. ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS. Neurology. 2018; 91(15): e1370–e1380.
  15. Woolley SC, Strong MJ, Woolley SC, et al. Frontotemporal Dysfunction and Dementia in Amyotrophic Lateral Sclerosis. Neurol Clin. 2015; 33(4): 787–805.
  16. Balendra R, Isaacs AM. C9orf72-mediated ALS and FTD: multiple pathways to disease. Nat Rev Neurol. 2018; 14(9): 544–558.
  17. Testa D, Lovati R, Ferrarini M, et al. Survival of 793 patients with amyotrophic lateral sclerosis diagnosed over a 28-year period. Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders. 2009; 5(4): 208–212.
  18. Miller RG, Mitchell JD, Moore DH, et al. Riluzole for amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND). Cochrane Database Syst Rev. 2000; 4(2): CD001447–206.
  19. Aboussouan LS, Khan SU, Banerjee M, et al. Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis. Muscle Nerve. 2001; 24(3): 403–409, doi: 10.1002/1097-4598(200103)24:3<403::aid-mus1013>3.0.co;2-3.
  20. Kleopa KA, Sherman M, Neal B, et al. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999; 164(1): 82–88.
  21. Davidson AC, Banham S, Elliott M, et al. BTS Standards of Care Committee Member, British Thoracic Society/Intensive Care Society Acute Hypercapnic Respiratory Failure Guideline Development Group, On behalf of the British Thoracic Society Standards of Care Committee. BTS/ICS guideline for the ventilatory management of acute hypercapnic respiratory failure in adults. Thorax. 2016; 71 Suppl 2: ii1–i35.
  22. Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006; 5(2): 140–147.