Ventilation treatment in patients with amyotrophic lateral sclerosis

Authors: S. Dostálová;  D. Kemlink;  I. Příhodová;  M. Srp;  M. Miler;  K. Šonka
Authors‘ workplace: Neurologická klinika a centrum klinických, neurověd 1. LF UK a VFN v Praze
Published in: Cesk Slov Neurol N 2021; 84/117(3): 232-236
Category: Review Article
doi: 10.48095/cccsnn2021232


Amyotrophic lateral sclerosis is a neurodegenerative disease affecting both upper and lower motoneurones, which leads to progressive weakness of muscles. Respiratory failure caused by muscle weakness is the major cause of death in this disease. As respiratory complications can be improved by treatment, it is necessary to pay signifi cant attention to respiratory functions at each patient review. Symptoms related to respiratory muscle weakness (dyspnea, tachypnea, orthopnea, use of auxiliary respiratory muscles at rest, paradoxical respiration, disturbed sleep, morning headache, daytime fatigue, excessive daytime sleepiness) should be observed, and respiratory functions require monitoring, especially forced vital capacity and sniff nasal pressure. An important dia gnostic feature is the early detection of nocturnal hypoventilation with the help of polysomnography or polygraphy, which are supplemented with blood gas analysis, ideally using transcutaneous capnography. Timely use of carefully monitored ventilation therapy improves the health status, quality of life, and survival of patients with amyotrophic lateral sclerosis. The essential treatment modality is non-invasive ventilation through a nasal or full-face mask allowing the achievement of the target tidal volume, backup respiratory rate, and automatic pressure adjustment. In order to ensure sufficient cough eff ectiveness cough-assisting devices are applied.


Amyotrophic lateral sclerosis – hypoventilation – capnography – noninvasive ventilation


1. Andersen PM, Abrahams S, Borasio GD et al. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS)-revised report of an EFNS task force. Eur J Neurol 2012; 19(3): 360–375. doi: 10.1111/ j.1468- 1331.2011.03501.x.

2. Štětkářová I, Matěj R, Ehler E. Nové poznatky v diagnostice a léčbě amyotrofické laterální sklerózy. Cesk Slov Neurol N 2018; 81/ 114(5): 546–554. doi: 10.14735/ amcsnn2018546.

3. Valko K, Ciesla L. Amyotrophic lateral sclerosis. Prog Med Chem 2019; 58: 63–117. doi: 10.1016/ bs.pmch. 2018.12.001.

4. Brooks BR, Miller RG, Swash M et al. El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 2000; 1(5): 293–299. doi: 10.1080/ 146608200300079536.

5. de Carvalho M, Dengler R, Eisen A et al. Electrodia g - nostic criteria for dia gnosis of ALS. Clin Neurophysiol 2008; 119(3): 497–503. doi: 10.1016/ j.clinph.2007.09.143.

6. Miller RG, Mitchell JD, Moore DH. Riluzole for amyotrophic lateral sclerosis (ALS)/ motor neuron disease (MND). Cochrane Database Syst Rev 2012; 2012(3): CD001447. doi: 10.1002/ 14651858.CD001447.pub3.

7. Fang T, Al Khleifat A, Meurgey JH et al. Stage at which riluzole treatment prolongs survival in patients with amyotrophic lateral sclerosis: a retrospective analysis of data from a dose-ranging study. Lancet Neurol 2018; 17(5): 416–422. doi: 10.1016/ S1474-4422(18)30054-1.

8. Berlowitz DJ, Howard ME, Fiore JF Jr et al. Identifying who will benefi t from non-invasive ventilation in amyotrophic lateral sclerosis/ motor neurone disease in a clinical cohort. J Neurol Neurosurg Psychiatry 2016; 87(3): 280–286. doi: 10.1136/ jnnp-2014-310055.

9. Botiková D, Vlčková E, Voháňka S. Dechová rehabilitace u pacientů s amyotrofickou laterální sklerózou. Cesk Slov Neurol N 2020; 83/ 116(5): 491–498. doi: 10.14735/ amcsnn2020491.

10. Arnulf I, Similowski T, Salachas F et al. Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis. Am J Respir Crit Care Med 2000; 161(3): 849–856. doi: 10.1164/ ajrccm.161.3.9805008.

11. Boentert M, Glatz C, Helmle C et al. Prevalence of sleep apnoea and capnographic detection of nocturnal hypoventilation in amyotrophic lateral sclerosis. J Neurol Sci 2018; 89(4): 418–424. doi: 10.1136/ jnnp-2017- 316515.

12. Pinto S, Carvalho M. Breathing new life into treatment advances for respiratory failure in amyotrophic lateral sclerosis patients. Neurodegener Dis Manag 2014; 4(1): 83–102. doi: 10.2217/ nmt.13.74.

13. Bourke SC, Tomlinson M, Williams TL et al. Eff ects 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. doi: 10.1016/ S1474-4422(05)70326-4.

14. Spittel S, Maier A, Kettemann D et al. Non-invasive and tracheostomy invasive ventilation in ALS: utilisation and survial rates in a cohort study over 12 years in Germany. Eur J Neurol 2020; 28(4): 1160–1171. doi: 10.1111/ ene.14647.

15. Lechtzin N, Scott Y, Busse AM et al. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler 2007; 8(3): 185–188. doi: 10.1080/ 17482960701262392.

16. Hirose T, Kimura F, Tani H et al. Clinical characteristics of long-term survival with non-invasive ventilation and factors aff ecting the transition to invasive ventilation in ALS. Muscle Nerve 2018; 58(6): 770–776. doi: 10.1002/ mus.26149.

17. Sancho J, Martinez D, Bures E et al. Bulbar impairment score and survival of stable amyotrophic lateral sclerosis patients after noninvasive ventilation initiation. ERJ Open Res 2018; 4(2): 00159–2017. doi: 10.1183/ 23120541.00159-2017.

18. Hayashi N, Atsuta N, Yokoi D et al. Prognosis of amyotrophic lateral sclerosis patients undergoing tracheostomy invasive ventilation therapy in Japan. J Neurol Neurosurg Psychiatry 2020; 91(3): 285–290. doi: 10.1136/ jnnp-2019-322213.

19. Vitacca M, Montini A, Lunetta C et al. Impact of an early respiratory care programme with non-invasive ventilation adaptation in patients with amyotrophic lateral sclerosis. Eur J Neurol 2018; 25(3): 556-e33. doi: 10.1111/ ene.13547.

20. Johns MW. A new method for measuring daytime sleepiness: the Epworth sleepiness scale. Sleep 1991; 14(6): 540–545.doi: 10.1093/ sleep/ 14.6.540.

21. Pinto S, De Carvalho M. Correlation between forced vital capacity and slow vital capacity for the assessment of respiratory involvement in amyotrophic lateral sclerosis: a prospective study. Amyotroph Lateral Scler Frontotemporal Degener 2017; 18(1–2): 86–91. doi: 10.1080/ 216 78421.2016.1249486.

22. Carratu P, Cassano A, Gadaleta F et al. Association between low sniff nasal-inspiratory pressure (SNIP) and sleep disordered breathing in amyotrophic lateral sclerosis: preliminary results. Amyotroph Lateral Scler 2011; 12(6): 458–463. doi: 10.3109/ 17482968.2011.593038.

23. Srp M, Gál O, Korteová R et al. Hygiena dýchacích cest u pacientů s Parkinsonovou nemocí – přehled problematiky a možnosti fyzioterapeutické intervence. Cesk Slov Neurol N 2020; 83/ 116(4): 375–381. doi: 10.14735/ amcsnn2020375.

24. Windisch W, Geiseler J, Simon K et al. German national guideline for treating chronic respiratory failure with invasive and non-invasive ventilation – revised edition 2017: Part 2. Respiration 2018; 96(2): 171–203. doi: 10.1159/ 000488667.

25. Tilanus TB, Groothuis JT, TenBroek-Pastoor JM et al. The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis. Respir Res 2017; 18(1): 144. doi: 10.1186/ s12931-017- 0624-8.

26. Prell T, Ringer TM, Wullenkord K et al. Assessment of pulmonary function in amyotrophic lateral sclerosis: when can polygraphy help evaluate the need for non-invasive ventilation? J Neurol Neurosurg Psychiatry 2016; 87(9): 1022–1026. doi: 10.1136/ jnnp-2015-312 185.

27. Berry RB, Brooks R, Gamaldo CE et al. for the American Academy of Sleep Medicine. The AASM Manual for the scoring of sleep and associated events: rules, terminology and technical specifications, Version 2.2. Darien, Il: American Academy of Sleep Medicine 2015.

28. Dostálová S, Šonka K. Vyšetřování poruch spánku a bdění. In: Nevšímalová S, Šonka K (eds). Poruchy spánku a bdění, třetí vydání. Praha: Galén 2020: 51–69.

29. Georges M, Nguyen-Baranoff D, Griff on L et al. Usefulness of transcutaneous PCO2 to assess nocturnal hypoventilation in restrictive lung disorders. Respirology 2016; 21(7): 1300–1306. doi: 10.1111/ resp.12812.

30. Dorst J, Ludolph AC. Non-invasive ventilation in amyotrophic lateral sclerosis. Ther Adv Neurol Disord 2019; 12: 1756286419857040. doi: 10.1177/ 1756286419857040.

31. Gelinas DF, O’Connor P, Miller RG. Quality of life for ventilator-dependent ALS patients and their caregivers. J Neurol Sci 1998; 160 (Suppl 1): S134–S136. doi: 10.1016/ s0022-510x(98)00212-3.

32. Mustfa N, Walsh E, Bryant V et al. The eff ect of noninvasive ventilation on ALS patients and their caregivers. Neurology 2006; 66(8): 1211–1217. doi: 10.1212/ 01. wnl.0000208957.88534.11.

33. Boentert M, Brenscheidt I, Glatz C et al. Eff ects of noninvasive ventilation on objective sleep and nocturnal respiration in patients with amyotrophic lateral sclerosis. J Neurol 2015; 262(9): 2073–2082. doi: 10.1007/ s00415- 015-7822-4.

34. Vrijsen B, Buyse B, Belge C et al. Noninvasive ventilation improves sleep in amyotrophic lateral sclerosis: a prospective polysomnographic study. J Clin Sleep Med 2015; 11(5): 559–566. doi: 10.5664/ jcsm.4704.

35. Jacobs TL, Brown DL, Baek J et al. Trial of early noninvasive ventilation for ALS: a pilot placebo-controlled study. Neurology 2016; 87(18): 1878–1883. doi: 10.1212/ WNL.0000000000003158.

36. Honnerová M, Kemlink D, Lněnička J et al. Indikační kritéria pro domácí neinvazivní ventilační podporu (NIV). [online]. Dostupné z URL: http:/ / www.sleepsociety. cz/ doporucene-postupy/ Indikace_NIV.pdf 2013.

37. NICE – National Institut for Health and Care Excellence. Non-invasive ventilation. [online]. Available from URL: https:/ / guidance/ NG42/ chapter/ recommendations#non-invasive-ventilation.

38. Bourke SC, Bullock RE, Williams TL et al. Noninvasive ventilation in ALS: indications and eff ect on quality of life. Neurology 2003; 61(2): 171–177. doi: 10.1212/ 01. wnl.0000076182.13137.38.

39. Vandenberghe N, Vallet AE, Petitjean T et al. Absence of airway secretion accumulation predicts tolerance of noninvasive ventilation in subjects with amyotrophic lateral sclerosis. Respir Care 2013; 58(9): 1424–1432. doi: 10.4187/ respcare.02103.

40. Gonzalez Calzada N, Prats Soro E, Mateu Gomez L et al. Factors predicting survival in amyotrophic lateral sclerosis patients on non-invasive ventilation. Amyotroph Lateral Scler Frontotemporal Degener 2016; 17(5–6): 337– 342. doi: 10.3109/ 21678421.2016.1165256.

41. Georges M, Attali V, Golmard JL et al. Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation. J Neurol Neurosurg Psychiatry 2016; 87(10): 1045–1050. doi: 10.1136/ jnnp-2015-312606.

42. Markovic N, Povitz M, Smith J et al. Patterns of non-invasive ventilation in amyotrophic lateral sclerosis. Can J Neurol Sci 2018; 45(4): 445–450. doi: 10.1017/ cjn.2018.25.

43. Spataro R, Bono V, Marchese S et al. Tracheostomy mechanical ventilation in patients with amyotrophic lateral sclerosis: clinical features and survival analysis. J Neurol Sci 2012; 323(1–2): 66–70. doi: 10.1016/ j.jns.2012.08. 011.

44. Matějek J. Dříve vyslovená přání pacienta. Komentář k zákonné úpravě. Praktický lékař 2012; 92(5): 286–289.

45. Neumannová K, Doušová T, Sedlák V et al. Doporučený postup České pneumologické a ftizeologické společnosti a České společnosti dětské pneumologie pro dlouhodobou domácí léčbu poruch expektorace pomocí přístroje CoughAssist. Cesk Slov Neurol N 2017; 80/ 113(4): 480–484. doi: 10.14735/ amcsnn 2017480.

46. Winck JC, Goncalves MR, Lourenco C et al. Effects of mechanical insuffl ation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest 2004; 126(3): 774–780. doi: 10.1378/ chest.126.3.774.

47. Sancho J, Servera E, Chiner E et al. Noninvasive respiratory muscle aids during PEG placement in ALS patients with severe ventilatory impairment. J Neurol Sci 2010; 297: 55–59. doi: 10.1016/ j.jns.2010.06.022.

48. Thompson AG, Blackwell V, Marsden R et al. A risk stratifying tool to facilitate safe late-stage percutaneous endoscopic gastrostomy in ALS. Amyotroph Lateral Scler Frontotemporal Degener 2017;18(3–4): 243– 248. doi: 10.1080/ 21678421.2016.1274330.

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