Respiratory rehabilitation in patients with amyotrophic lateral sclerosis

Česká verze

Authors: D. Botiková- ^{1 3}; E. Vlčková ^2,3; S. Voháňka ^2,3
Authors place of work: Rehabilitační oddělení, FN Brno ¹; Neurologická klinika, ERN-EURO NMD, Centrum, FN Brno ²; LF MU, Brno ³
Published in the journal: Cesk Slov Neurol N 2020; 83/116(5): 491-498
Category: Přehledný referát
doi: https://doi.org/10.14735/amcsnn2020491

Summary

Amyotrophic lateral sclerosis (ALS) is a serious neurodegenerative disease, which belongs to a group of conditions affecting the motor neuron system, called motor neuron disease. During the course of the disease, a reduction in the voluntary muscle strength occurs, along with the loss of strength in muscles of respiration. This results in ineffective cough, restrictive changes in ventilation along with dyspnoea. Respiratory muscle weakness progressively leads to respiratory insufficiency, which is the major cause of death in this disease. That is why it is important to monitor respiratory and motor functions in patients with ALS from the moment the diagnosis is given. Functional vital capacity and maximal inspiratory pressure are regarded as the main determinants of respiratory symptomatology and respiratory insufficiency predicting also the life longevity in patients with ALS. Respiratory physiotherapy provides patients with a wide spectrum of techniques, which have a potential to slow down the progress and the severity of respiratory symptoms in case the exercise program is initiated early enough, before the onset of more serious respiratory involvement. The program consists mainly of methods focused on inspiratory and expiratory muscle training, for which special respiratory devices are used. Later in the course of the disease, patients are being provided with techniques which may decrease shortness of breath and which increase effectivity of cough by aiding the manoeuvres. This palliative symptomatic care aims to increase the quality of life in patients with ALS, while being helpful right from the onset as well as during the whole course of the disease.

Keywords:

motor neuron disease – respiratory insuffi ciency – dyspnea – muscle weakness – respiratory muscles – cough – physiotherapy techniques

Zdroje

1. 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

2. Pasparaki E, Bibaki E, Meletis Y et al. Neuromuscular diseases and respiratory system. Pneumon 2017; 30 (3): 176–187. doi: 10.1097/MOP.0000000000000498.

3. Logroscino G, Traynor BJ, Hardiman O et al. Incidence of amyotrophic lateral sclerosis in Europe. J Neurol Neurosurg Psychiatry 2010; 81 (4): 385–390. doi: 10.1136/jnnp.2009.183525.

4. Boentert M, Wenninger S, Sansone VA. Respiratory involvement in neuromuscular disorders. Curr Opin Neurol 2017; 30 (5): 529–537. doi: 10.1097/WCO.0000000000000470.

5. Shoesmith CL, Findlater K, Rowe A et al. Prognosis of amyotrophic lateral sclerosis with respiratory onset. J Neurol Neurosurg Psychiatry 2007; 78 (6): 629–631. doi: 10.1136/jnnp.2006.103564.

6. Hardiman O. Management of respiratory symptoms in ALS. J Neurol 2011; 258 (3): 359–365. doi: 10.1007/s00415-010-5830-y.

7. 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.

8. Benditt JO. Pathophysiology of neuromuscular respiratory diseases. Clin Chest Med 2018; 39 (2): 297–308. doi: 10.1016/j. ccm.2018.01.011.

9. Bergofsky EH. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis 1979; 119 (4): 643–669. doi: 10.1164/arrd.1979.119.4.643.

10. Kapandji IA. The physiology of the joints. Edinburgh: Churchill Livingstone 2007.

11. Troyer AD, Kelly S, Zin WA. Mechanical action of the intercostal muscles on the ribs. Science 1983; 220 (4592): 87–88. doi: 10.1126/science.6828883.

12. Benditt JO. The neuromuscular respiratory system: physiology, pathophysiology, and a respiratory care approach to patients. Respir Care 2006; 51 (8): 829–837. doi: 10.1126/science.6828883.

13. Vondra V. Dušnost: problém mnoha oborů. Praha: Mladá fronta 2017.

14. Neumannová K, Kolek V. Asthma bronchiale a chronická obstrukční plicní nemoc Možnosti komplexní léčby z pohledu fyzioterapeuta. Praha: Mladá fronta 2012.

15. Ayvazian LF. Handbook of physiology: Section 3, The respiratory system, Volume III: Mechanics of breathing, Parts 1 and 2. Am Rev Respir Dis 1987; 136 (3): 796–796. doi: 10.1164/ajrccm/136.3.796c.

16. Park KH, Kim RB, Yang J et al. Reference range of respiratory muscle strength and its clinical application in amyotrophic lateral sclerosis: a single-center study. J Clin Neurol Seoul Korea 2016; 12 (3): 361–367. doi: 10.3988/jcn.2016.12.3.361.

17. Žurková P, Shudeiwa A. Vyšetření funkce plic a respiračních svalů u pacientů s neuromuskulárním onemocněním. Neurol Praxi 2012; 13 (6): 336–340. doi: 10.nnnn/neu.2012.085.

18. Pinto S, Swash M, de Carvalho M. Respiratory exercise in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2012; 13 (1): 33–43. doi: 10.3109/17482968.2011.626052.

19. Sharma R, Wolfe L. Use of non-invasive ventilation in neuromuscular disease. Curr Sleep Med Rep 2017; 3 (4): 290–298. doi: 10.1007/s40675-017-0097-9.

20. Suarez A, Augusto Pessolano F, Monteiro S et al. Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease. Am J Phys Med Rehabil 2002; 81: 506–511. doi: 10.1097/00002060-200207000-00007.

21. Walterspacher S, Pietsch F, Walker DJ et al. Activation of respiratory muscles during respiratory muscle training. Respir Physiol Neurobiol 2018; 247: 126–132. doi: 10.1016/j.resp.2017.10.004.

22. Reffin J, Dewar L, Di Marco M et al. Exercise advice for adults with muscle-wasting conditions. Muscular dystrophy campaign support resour hub. [online]. Available from URL: http: //hub.muscular-dystrophy.org/wp-content/uploads/2014/12/Exercise-advice-for-adults-final-051214.pdf.

23. Eagle M. Report on the muscular dystrophy campaign workshop: exercise in neuromuscular diseases Newcastle, January 2002. Neuromuscul Disord 2002; 12 (10): 975–983. doi: 10.1016/s0960-8966 (02) 00136-0.

24. Pinto S, de Carvalho M. Can inspiratory muscle training increase survival in early-affected amyotrophic lateral sclerosis patients? Amyotroph Lateral Scler Front Degener 2013; 14 (2): 124–126. doi: 10.3109/17482968.2012.726227.

25. Seo K, Hwan PS, Park K. The effects of inspiratory diaphragm breathing exercise and expiratory pursed-lip breathing exercise on chronic stroke patients’ respiratory muscle activation. J Phys Ther Sci 2017; 29 (3): 465–469. doi: 10.1589/jpts.29.465.

26. Ramsook AH, Koo R, Molgat-Seon Y et al. Diaphragm recruitment increases during a bout of targeted inspiratory muscle training. Med Sci Sports Exerc 2016; 48 (6): 1179–1186. doi: 10.1249/MSS.0000000000000881.

27. Nardin R, O’Donnell C, Loring SH et al. Diaphragm training in amyotrophic lateral sclerosis. J Clin Neuromuscul Dis 2008; 10 (2): 56–60. doi: 10.1097/CND.0b013e31818cf6df.

28. Neumannova K. Možnosti využití technik plicní rehabilitace pro léčbu snížené síly dýchacích svalů. Čas Lék čes 2015; 154: 72–78.

29. Neumannová K, Vladimír Koblížek. Doporučený postup plicní rehabilitace 2019. [online]. Dostupné z URL: https: //docplayer.cz/8779489-Standard-plicni-rehabilitace-zakladni-verze-katerina-neumannova-jakub-zatloukal-vladimir-koblizek.html.

30. Lahrmann H, Wild M, Zdrahal F et al. Expiratory muscle weakness and assisted cough in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord 2003; 4 (1): 49–51. doi: 10.1080/14660820310006733.

31. Plowman EK, Tabor-Gray L, Rosado KM et al. Impact of expiratory strength training in amyotrophic lateral sclerosis: results of a randomized, sham-controlled trial. Muscle Nerve 2019; 59 (1): 40–46. doi: 10.1002/mus.26292.

32. Smolíková L, Máček M. Respirační fyzioterapie a plicní rehabilitace. Brno: Národní centrum ošetřovatelství a nelékařských zdravotnických oborů 2010.

33. Ng L, Khan F, Young CA et al. Symptomatic treatments for amyotrophic lateral sclerosis/motor neuron disease. Cochrane Database Syst Rev 2017; 1: CD011776. doi: 10.1002/14651858.CD011776.pub2.

34. Mesquita Montes A, Maia J, Crasto C et al. Abdominal muscle activity during breathing in different postures in COPD ‘Stage 0’ and healthy subjects. Respir Physiol Neurobiol 2017; 238: 14–22. doi: 10.1016/j.resp.2017.01.001.

35. Katz S, Arish N, Rokach A et al. The effect of body position on pulmonary function: a systematic review. BMC Pulm Med 2018; 18 (1): 159. doi: 10.1186/s12890-018-0723-4.

36. Kolář P et al. Rehabilitace v klinické praxi. Praha: Galén 2009.

37. Žurková P, Skřičková J. Přehled dechových pomůcek pro hygienu dýchacích cest v praxi. Medicína Praxi 2012; 9 (5): 250–255. doi: 10.nnnn/med.2012.058.

38. Reid WD, Brown JA, Konnyu KJ et al. Physiotherapy secretion removal techniques in people with spinal cord injury: a systematic review. J Spinal Cord Med 2010; 33 (4): 353–370. doi: 10.1080/10790268.2010.11689714.

39. Bach J. Don’t forget the abdominal thrust. Chest 2004; 126: 1388–1389. doi: 10.1378/chest.126.4.1388-a.

40. Senent C, Golmard JL, Salachas F et al. A comparison of assisted cough techniques in stable patients with severe respiratory insufficiency due to amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2011; 12 (1): 26–32. doi: 10.3109/17482968.2010.535541.

41. Bach JR, Mahajan K, Lipa B et al. Lung insufflation capacity in neuromuscular disease. Am J Phys Med Rehabil 2008; 87 (9): 720–725. doi: 10.1097/PHM.0b013e31817fb26f.

42. Lechtzin N, Wolfe LF, Frick KD. The impact of high-frequency chest wall oscillation on healthcare use in patients with neuromuscular diseases. Ann Am Thorac Soc 2016; 13 (6): 904–909. doi: 10.1513/AnnalsATS.201509-597OC.

43. Stehling F, Bouikidis A, Schara U et al. Mechanical insufflation/exsufflation improves vital capacity in neuromuscular disorders. Chron Respir Dis 2015; 12 (1): 31–35. doi: 10.1177/1479972314562209.

44. Vitacca M, Paneroni M, Trainini D et al. At home and on demand mechanical cough assistance program for patients with amyotrophic lateral sclerosis. Am J Phys Med Rehabil 2010; 89 (5): 401–406. doi: 10.1097/PHM.0b013e3181d89760.

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/amcsnn2017480.

46. Mustfa N, Aiello M, Lyall RA et al. Cough augmentation in amyotrophic lateral sclerosis. Neurology 2003; 61 (9): 1285–1287. doi: 10.1212/01.wnl.0000092018.56823.02.

47. Bach JR. Mechanical insufflation-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest 1993; 104 (5): 1553–1562. doi: 10.1378/chest.104.5.1553.

48. Rafiq MK, Bradburn M, Proctor AR et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Front Degener 2015; 16 (7–8): 448–455. doi: 10.3109/21678421.2015.1051992.

49. Desnuelle C, Bruno M, Soriani MH et al. [What physical therapy techniques can be used to improve airway freedom in amyotrophic lateral sclerosis?] Rev Neurol (Paris) 2006; 162 (Spec No 2): 4S244–4S252.