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.

• Keywords
• Huntington disease, cough, mHealth, resistance training, respiratory muscles,
• MeSH
• Breathing Exercises methods   MeSH
• Adult MeSH
• Respiratory Muscles * physiopathology   MeSH
• Huntington Disease * physiopathology   complications   rehabilitation   MeSH
• Cough * physiopathology   etiology   MeSH
• Middle Aged MeSH
• Humans MeSH
• Resistance Training methods   MeSH
• Prospective Studies MeSH
• Case-Control Studies MeSH
• Muscle Strength * physiology   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH

BACKGROUND: The effects of expiratory muscle strength training (EMST) has not yet been investigated in MSA patients. OBJECTIVE: The primary objective was to test the effects of EMST on expiratory muscle strength and voluntary peak cough flow (vPCF) in patients with multiple system atrophy (MSA). The secondary objective was to assess the suitability of the pulmonary dysfunction index as a tool for identifying MSA patients with expiratory muscle weakness and reduced voluntary peak cough flow. METHODS: This was an open label, non-controlled study, with an 8-week intensive home-based EMST protocol. The outcome measures included: maximal expiratory pressure (MEP) and vPCF. The sensitivity and specificity of the index of pulmonary dysfunction in the respiratory diagnostic process were assessed using receiver operating characteristic (ROC) analysis. RESULTS: Fifteen MSA patients were enrolled in the study. Twelve MSA patients completed the training period. After the training period, MEP significantly increased (P = 0.006). Differences in vPCF were not significant (P = 0.845). ROC analysis indicated that the overall respiratory diagnostic accuracy of the index of pulmonary dysfunction had an outstanding capability to detect patients at risk of less effective coughing and an acceptable capability of detecting patients with decreased expiratory muscle strength. CONCLUSIONS: These findings indicate non-significant differences in vPCF after 8 weeks of EMST. The index of pulmonary dysfunction appears to be a promising prognostic screening tool for identifying altered cough efficacy in MSA patients. Test cut-offs may be used to select an appropriate respiratory physiotherapy technique.

• Keywords
• expiratory muscle strength training, maximum expiratory pressure, multiple system atrophy, voluntary peak cough flow,
• Publication type
• Journal Article MeSH