Current Strategies for Assessing Lung Function and Managing Respiratory Dysfunction in Amyotrophic Lateral Sclerosis: a Scoping Review
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Abstract
Introduction: Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease that affects the motor neurons of the central nervous system. Respiratory failure is the main cause of death in these patients.
Objective: To map the recently used strategies (2019–2024) for assessing pulmonary function and managing respiratory dysfunctions in individuals with ALS, identifying emerging advances and their alignment with current clinical guidelines.
Methods: A scoping review was conducted following the recommendations of the Joanna Briggs Institute (JBI) and the PRISMA-ScR extension for scoping reviews. The PubMed, Scopus, and Web of Science databases were searched up to March 2024. Original studies published between 2019 and 2024 were included.
Results: Of 4,427 records, 28 studies met the inclusion criteria. The most frequent strategies were spirometry, maximal respiratory pressures, capnography/blood gas analysis, non-invasive ventilation (NIV), and cough-assist techniques. Emerging tools such as calculated maximal voluntary ventilation (cMVV), diaphragmatic ultrasound, and pressure at 0.1 second (P0.1) measurement showed clinical potential but require validation. The use of telemonitoring stood out as a complement to home-based NIV management.
Conclusions: Although options for respiratory assessment and management in ALS have expanded, international guidelines continue to recommend established methods as the foundation of care. The incorporation of new techniques should be cautious and supported by robust evidence.
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Amato AA, Russell JA. Esclerosis lateral amiotrófica. En: Amato AA, Russell JA, editors. Trastornos neuromusculares. 2nd ed. McGraw-Hill, Burlington, 2016; pp. 174-198.
Quinn C, Elman L. Amyotrophic lateral sclerosis and other motor neuron diseases. Continuum (Minneap Minn) 2020;26(5):1323-1347. https://doi.org/10.1212/CON.0000000000000911
Manera U, Torrieri MC, Moglia C, Canosa A, Vasta R, Palumbo F et al. Calculated maximal voluntary ventilation (cMVV) as a marker of early respiratory insufficiency in amyotrophic lateral sclerosis. Brain Sci 2024;14(2):157. https://doi.org/10.3390/brainsci14020157
Carvalho M, Fernandes SR, Pereira M, Gromicho M, Oliveira Santos M, Alves I et al. Respiratory function tests in amyotrophic lateral sclerosis: the role of maximal voluntary ventilation. J Neurol Sci 2022;434:120143. https://doi.org/10.1016/j.jns.2022.120143
Pellegrino GM, Papa GFS, Centanni S, Corbo M, Kvarnberg D, Tobin MJ et al. Measurement of vital capacity in amyotrophic lateral sclerosis: effects of interfaces and reproducibility. Respir Med 2021;176:106277. https://doi.org/10.1016/j.rmed.2020.106277
Racca F, Del Sorbo L, Mongini T, Vianello A, Ranieri VM. Respiratory management in amyotrophic lateral sclerosis: update and perspectives. Minerva Anestesiol 2010;76(1):33-40.
Lui AJ, Byl NN. A systematic review of the effect of moderate intensity exercise on function and disease progression in amyotrophic lateral sclerosis. J Neurol Phys Ther 2009;33(2):68-87. https://doi.org/10.1097/NPT.0b013e31819912d0
Peters MDJ, Godfrey CM, McInerney P, Soares CB, Khalil H, Parker D. The Joanna Briggs Institute reviewers' manual 2015: methodology for JBI scoping reviews 2015. [Internet]. [Consultado 25 jul 2025]. Disponible en: http://joannabriggs.org/assets/docs/sumari/Reviewers-Manual_Methodology-for-JBI-Scoping-Reviews_2015_v2.pdf
Tricco AC, Lillie E, Zarin W, O’Brien KK, Colquhoun H, Levac D et al. PRISMA extension for scoping reviews (PRISMA-ScR): checklist and explanation. Ann Intern Med 2018;169(7):467-473. https://doi.org/10.1016/j.jclinepi.2015.11.021
Arksey H, O’Malley L. Scoping studies: towards a methodological framework. Int J Soc Res Methodol 2005;8(1):19-32. https://doi.org/10.1080/1364557032000119616
Levac D, Colquhoun H, O’Brien KK. Scoping studies: advancing the methodology. Implement Sci 2010;5:69. https://doi.org/10.1186/1748-5908-5-69
Moola S, Munn Z, Tufanaru C, Aromataris E, Sears K, Sfetcu R et al. Systematic reviews of etiology and risk. En: Aromataris E, Munn Z, editors. JBI Manual for Evidence Synthesis. Adelaide: JBI; 2020. https://doi.org/10.46658/JBIMES-20-06
Sarmento A, Fregonezi G, Dourado-Junior MET, Aliverti A, de Andrade AD, Franco-Parreira V et al. Thoracoabdominal asynchrony and paradoxical motion in middle-stage amyotrophic lateral sclerosis. Respir Physiol Neurobiol 2019;259:16-25. https://doi.org/10.1016/j.resp.2018.06.012
Panchabhai TS, Cabodevila EM, Pioro E, Wang X, Han X, Aboussouan LS. Pattern of pulmonary function decline in patients with amyotrophic lateral sclerosis: implications for timing of noninvasive ventilation. ERJ Open Res 2019;5(3):00044-2019. https://doi.org/10.1183/23120541.00044-2019
Huang X, Du C, Yang Q, Fan D. Comparison of slow and forced vital capacity for evaluating respiratory function in amyotrophic lateral sclerosis with bulbar involvement. Front Neurol 2022;13:938256. https://doi.org/10.3389/fneur.2022.938256
American Thoracic Society/European Respiratory Society. ATS/ERS statement on respiratory muscle testing. Am J Respir Crit Care Med 2002;166(4):518-624. https://doi.org/10.1164/rccm.166.4.518
Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chiò A, Van Damme P 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. https://doi.org/10.1111/j.1468-1331.2011.03501.x
Rajula RR, Saini J, Unnikrishnan G, Vengalil S, Nashi S, Bardhan M et al. Diaphragmatic ultrasound: prospects as a tool to assess respiratory muscle involvement in amyotrophic lateral sclerosis. J Clin Ultrasound 2022;50(1):131-135. https://doi.org/10.1002/jcu.23069
Iguchi N, Mano T, Iwasa N, Ozaki M, Yamada N, Kikutsuji N et al. Thoracic excursion as a biomarker to evaluate respiratory function in amyotrophic lateral sclerosis. Front Neurol 2022;13:853469. https://doi.org/10.3389/fneur.2022.853469
Viccaro F, Lecci A, Baccolini V, Sciurti A, Piamonti D, Inghilleri M et al. Prediction of cough effectiveness in amyotrophic lateral sclerosis patients assessed by ultrasound of the diaphragm during the cough expiration phase. Respir Physiol Neurobiol 2024;327:104299. https://doi.org/10.1016/j.resp.2024.104299
Pinto S, Swash M, de Carvalho M. Mouth occlusion pressure at 100 ms (P0.1) as a respiratory biomarker in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2021;22(1-2):53-60. https://doi.org/10.1080/21678421.2020.1821061
Yoon SY, Kim HK, Kim MJ, Suh JH, Leigh JH. Factors associated with assisted ventilation use in amyotrophic lateral sclerosis: a nationwide population-based study in Korea. Sci Rep 2021;11(1):98990. https://doi.org/10.1038/s41598-021-98990-x
Sarasate M, González N, Córdoba-Izquierdo A, Prats E, Rodriguez Gonzalez-Moro JM, Martí S et al. Impact of early non-invasive ventilation in amyotrophic lateral sclerosis: a multicenter randomized controlled trial. J Neuromuscul Dis 2023;10(4):627-637. https://doi.org/10.3233/JND-221658
Oliver D, Borasio GD, Caraceni A, de Visser M, Grisold W, Lorenzl S et al. A consensus review on the development of palliative care for patients with chronic and progressive neurological disease. Eur J Neurol 2016;23(1):30-38. https://doi.org/10.1111/ene.12889
Volpato E, Vitacca M, Ptacinsky L, Lax A, D’Ascenzo S, Bertella E et al. Home-based adaptation to night-time non-invasive ventilation in patients with amyotrophic lateral sclerosis: a randomized controlled trial. J Clin Med 2022;11(11):3178. https://doi.org/10.3390/jcm11113178
Sancho J, Burés E, Ferrer S, Ferrando A, Bañuls P, Servera E. Unstable control of breathing can lead to ineffective noninvasive ventilation in amyotrophic lateral sclerosis. ERJ Open Res 2019;5(3):00099-2019. https://doi.org/10.1183/23120541.00099-2019
Réginault T, Bouteleux B, Wilbart P, Mathis S, Le Masson G, Pillet O et al. At-home noninvasive ventilation initiation with telemonitoring in amyotrophic lateral sclerosis patients: a retrospective study. ERJ Open Res 2023;9(1):00438-2022. https://doi.org/10.1183/23120541.00438-2022
Niedermeyer S, Murn M, Choi PJ. Respiratory failure in amyotrophic lateral sclerosis. Chest 2019;155(2):401-408. https://doi.org/10.1016/j.chest.2018.06.035
Plowman EK, Tabor-Gray L, Rosado KM, Vasilopoulos T, Robison R, Chapin JL 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. https://doi.org/10.1002/mus.26292
Polverino F, Sampaolo S, Capuozzo A, Fasolino M, Aliberti M, Satta E et al. Diagnosis of amyotrophic lateral sclerosis by respiratory function test. Multidiscip Respir Med 2023;18(1):941. https://doi.org/10.4081/mrm.2023.941
Spiliopoulos KC, Lykouras D, Veltsista D, Skaramagkas V, Karkoulias K, Tzouvelekis A et al. The utility of diaphragm ultrasound thickening indices for assessing respiratory decompensation in amyotrophic lateral sclerosis. Muscle Nerve 2023;68(6):850-856. https://doi.org/10.1002/mus.27980
Kelly CR, Parra-Cantu C, Thapa P, Boynton B, Selim BJ, Sorenson EJ et al. Comparative performance of different respiratory test parameters for detection of early respiratory insufficiency in patients with ALS. Neurology 2022;99(7):e743-e750. https://doi.org/10.1212/WNL.0000000000200758
Michels S, Widmann P, Rapp D, Willkomm F, Ludolph AC, Dorst J. Predictive parameters of early respiratory decline in amyotrophic lateral sclerosis. Eur J Neurol 2022;29(11):3170-3176. https://doi.org/10.1111/ene.15486
Zhang QJ, Huang JC, Chen J, Hu W, Xu LQ, Guo QF. Peak expiratory flow is a reliable household pulmonary function parameter correlated with disease severity and survival in patients with amyotrophic lateral sclerosis. BMC Neurol 2022;22(1):105. https://doi.org/10.1186/s12883-022-02635-z
Pihtili A, Bingol Z, Durmus H, Parman Y, Kiyan E. Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis. Muscle Nerve 2021;63:683-689. https://doi.org/10.1002/mus.27200
Wen Q, Ma J, Pang X, Huang S, Zhang J, Wang J et al. Diaphragm ultrasound in the diagnosis of respiratory dysfunction in patients with amyotrophic lateral sclerosis. Rev Neurol (Paris) 2021;177(6):639-646. https://doi.org/10.1016/j.neurol.2020.07.020
Digala LP, Govindarajan R. Thickening fraction as a measure of ultrasonographic diaphragm dysfunction in amyotrophic lateral sclerosis. Clin Neurophysiol Pract 2020;5:35-37. https://doi.org/10.1016/j.cnp.2020.01.001
Manera U, Torrieri MC, Moglia C, Viglione M, Daviddi MAR, Matteoni E et al. The role of arterial blood gas analysis (ABG) in amyotrophic lateral sclerosis respiratory monitoring. J Neurol Neurosurg Psychiatry 2020;91(9):999-1000. https://doi.org/10.1136/jnnp-2020-323810
Fantini R, Tonelli R, Castaniere I, Tabbì L, Pellegrino MR, Cerri S et al. Serial ultrasound assessment of diaphragmatic function and clinical outcome in patients with amyotrophic lateral sclerosis. BMC Pulm Med 2019;19(1):160. https://doi.org/10.1186/s12890-019-0924-5
Sartucci F, Pelagatti A, Santin M, Bocci T, Dolciotti C, Bongioanni P. Diaphragm ultrasonography in amyotrophic lateral sclerosis: a diagnostic tool to assess ventilatory dysfunction and disease severity. Neurol Sci 2019;40(10):2065-2071. https://doi.org/10.1007/s10072-019-03938-9