BACKGROUND AND PURPOSE: Dysfunction of the airway defence system in Huntington's disease (HD) is a significant but often overlooked problem. Although expiratory muscle strength training (EMST) is frequently utilized in cough effectiveness treatment, its specific impact in HD patients has not yet been explored. This study investigated the effects of EMST on voluntary peak cough flow (vPCF) in HD patients and evaluated the retention of potential gains post-intervention. METHODS: In this prospective case-controlled trial, 29 HD patients completed an 8-week wait-to-start period, which served to identify the natural development of expiratory muscle strength and vPCF. This was followed by 8 weeks of EMST training and an additional 8 weeks of follow-up. The study's outcome parameters, vPCF and maximum expiratory pressure (MEP), were measured against those of age- and sex-matched healthy controls. RESULTS: Huntington's disease patients had significantly lower MEP (p < 0.001) and vPCF (p = 0.012) compared to healthy controls at baseline. Following the EMST, significant improvements in MEP (d = 1.39, p < 0.001) and vPCF (d = 0.77, p = 0.001) were observed, with HD patients reaching the cough performance levels of healthy subjects. However, these gains diminished during the follow-up, with a significant decline in vPCF (d = -0.451, p = 0.03) and in MEP (d = -0.71; p = 0.002). CONCLUSIONS: Expiratory muscle strength training improves expiratory muscle strength and voluntary cough effectiveness in HD patients, but an ongoing maintenance programme is necessary to sustain the improvements.

Department of Neurology and Centre of Clinical Neuroscience General University Hospital and 1st Faculty of Medicine Charles University Prague Czech Republic

Faculty of Biomedical Engineering Czech Technical University Prague Czech Republic

Zobrazit více v PubMed

Dubinsky RM. No going home for hospitalized Huntington's disease patients. Mov Disord. 2005;20(10):1316‐1322. PubMed

Rodrigues FB, Abreu D, Damásio J, et al. Survival, mortality, causes and places of death in a European Huntington's disease prospective cohort. Mov Disord Clin Pract. 2017;4(5):737‐742. PubMed PMC

Lüthi‐Müller E, Kool J, Mylius V, Diesener P. A new therapeutic approach for dystussia and atussia in neurogenic dysphagia: effect of aerosolized capsaicin on peak cough flow. Dysphagia. 2022;37(6):1814‐1821. PubMed PMC

Jones U, Busse M, Enright S, Rosser AE. Respiratory decline is integral to disease progression in Huntington's disease. Eur Respir J. 2016;48(2):585‐588. PubMed PMC

Schumann‐Werner B, Dogan I, Mirzazade S, et al. Clinical predictors and neural correlates for compromised swallowing safety in Huntington disease. Eur J Neurol. 2021;28(9):2855‐2862. PubMed

van de Wetering‐van DV, Kalf JG, van der Wees PJ, Bloem BR, Nijkrake MJ. The effects of respiratory training in Parkinson's disease: a systematic review. J Parkinsons Dis. 2020;10(4):1315‐1333. PubMed PMC

Srp M, Capek V, Gal O, et al. Severely disabled multiple sclerosis patients can achieve the performance of healthy subjects after expiratory muscle strength training. Mult Scler Relat Disord. 2021;55:103187. PubMed

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. PubMed

Brooks M, McLaughlin E, Shields N. Expiratory muscle strength training improves swallowing and respiratory outcomes in people with dysphagia: a systematic review. Int J Speech Lang Pathol. 2019;21(1):89‐100. PubMed

Troche MS, Curtis JA, Sevitz JS, et al. Rehabilitating cough dysfunction in Parkinson's disease: a randomized controlled trial. Mov Disord. 2023;38(2):201‐211. PubMed

Reyes A, Cruickshank T, Thompson J, Ziman M, Nosaka K. Surface electromyograph activity of submental muscles during swallowing and expiratory muscle training tasks in Huntington's disease patients. J Electromyogr Kinesiol. 2014;24(1):153‐158. PubMed

Reyes A, Cruickshank T, Ziman M, Nosaka K. Pulmonary function in patients with Huntington's disease. BMC Pulm Med. 2014;14(1):1‐8. PubMed PMC

Reyes A, Cruickshank T, Nosaka K, Ziman M. Respiratory muscle training on pulmonary and swallowing function in patients with Huntington's disease: a pilot randomised controlled trial. Clin Rehabil. 2015;29(10):961‐973. PubMed

Troche MS, Rosenbek JC, Okun MS, Sapienza CM. Detraining outcomes with expiratory muscle strength training in Parkinson disease. J Rehabil Res Dev. 2014;51(2):305‐310. PubMed PMC

Borders JC, Troche MS. Voluntary cough effectiveness and airway clearance in neurodegenerative disease. J Speech Lang Hear Res. 2022;65(2):431‐449. PubMed

Miller MR, Hankinson J, Brusasco V, et al. Standardisation of spirometry. Eur Respir J. 2005;26(2):319‐338. PubMed

Laveneziana P, Albuquerque A, Aliverti A, et al. ERS statement on respiratory muscle testing at rest and during exercise. Eur Respir J. 2019;53(6):1801214. PubMed

Reyes A, Castillo A, Castillo J, Cornejo I. The effects of respiratory muscle training on peak cough flow in patients with Parkinson's disease: a randomized controlled study. Clin Rehabil. 2018;32(10):1317‐1327. PubMed

Pitts T, Bolser D, Rosenbek J, Troche M, Okun MS, Sapienza C. Impact of expiratory muscle strength training on voluntary cough and swallow function in Parkinson disease. Chest. 2009;135(5):1301‐1308. PubMed PMC

Playle R, Dimitropoulou P, Kelson M, Quinn L, Busse M. Exercise interventions in Huntington's disease: an individual patient data meta‐analysis. Mov Disord Clin Pract. 2019;6(7):567‐575. PubMed PMC

Srp M, Korteová R, Kliment R, Jech R, Růžička E, Hoskovcová M. Expiratory muscle strength training in patients with Parkinson's disease: a pilot study of mobile monitoring application. Mov Disord Clin Pract. 2021;8(7):1148‐1149. PubMed PMC

Kieburtz K, Penney JB, Como P, et al. Unified Huntington's disease rating scale: reliability and consistency. Mov Disord. 1996;11(2):136‐142. PubMed

Siesling S, Van Vugt JP, Zwinderman KA, Kieburtz K, Roos RA. Unified Huntington's disease rating scale: a follow up. Mov Disord. 1998;13(6):915‐919. PubMed

Reyes A, Castillo A, Castillo J. Effects of expiratory muscle training and air stacking on peak cough flow in individuals with Parkinson's disease. Lung. 2020;198:207‐211. PubMed

Sapienza C, Hoffman B. Respiratory Muscle Strength Training. Plural Publishing; 2021.

Quinn L, Kegelmeyer D, Kloos A, Rao AK, Busse M, Fritz NE. Clinical recommendations to guide physical therapy practice for Huntington disease. Neurology. 2020;94(5):217‐228. PubMed PMC

Park H‐K, Kim Y‐J, Kim T‐H. The role of visual feedback in respiratory muscle activation and pulmonary function. J Phys Ther Sci. 2015;27(9):2883‐2886. PubMed PMC

Al‐Wardat M, Schirinzi T, Hadoush H, et al. Home‐based exercise to improve motor functions, cognitive functions, and quality of life in people with Huntington's disease: a systematic review and meta‐analysis. Int J Environ Res Public Health. 2022;19(22):14915. PubMed PMC