Abstract

Background Huntington’s disease (HD) is a neurodegenerative disorder characterised by progressive motor, cognitive and psychiatric impairments. Motor abnormalities affect all parts of the body including respiratory and swallowing muscles. Respiratorymuscle training (RMT) has been shown to improve pulmonary function in Parkinson’s disease and multiple sclerosis patients, however no previous studies have reported its effects on HD patients. This study examined the effects of 4-month RMT on pulmonary and swallowing function, exercise capacity and dyspnoea in manifest HD patients. Methods Eighteen manifest HD patients (11 men, 7 women; 53 ± 10 years) were randomly assigned to control group (n = 9) and training group (n = 9). Both groups received home-based inspiratory (5 sets of 5 repetitions) and expiratory (5 sets of 5 repetitions) muscle training 6 times a week for 4 months. The control group used a fixed resistance of 9 cm H2O, and the training group used a progressively increased resistance from 30% to 75% of each patient’s maximum respiratory pressure. Pulmonary and swallowing function, 6 minutes walk test and dyspnoea were assessed before, at 2 and 4 months after training. Results The magnitude of increases in maximum inspiratory and expiratory pressures, forced vital capacity, forced expiratory volume in 1 second and peak expiratory flow was substantially greater for the training than the control group. Changes in swallowing function, dyspnoea and exercise capacity were small for both groups without substantial differences between groups. Conclusions A home-based RMT program appeared to be beneficial to improve pulmonary function in manifest HD patients.

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