Effects of Strength Training on Bulbar Function in Amyotrophic Lateral Sclerosis

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DESCRIPTION (provided by applicant): Bulbar dysfunction in the form of progressive speech, swallow and respiratory impairment are highly prevalent in Amyotrophic Lateral Sclerosis (ALS) and together account for 95% of disease mortality. Although progressive muscle weakness is the primary sign of ALS, the effects of exercise and strength training in this patient population i controversial. While exercise in persons with ALS has historically been discouraged due to fear that muscle overburden may execrate physical decline, recent animal and human data suggest that moderate intensity exercise applied early in the disease may serve a neuroprotective role in ALS. Indeed, exciting preliminary data from our laboratory indicate that a regimen of expiratory muscle strength training (EMST) in persons with mild-moderate ALS has a positive effect on expiratory force generating abilities, airway protection and cough during swallowing, swallow kinematics, self-reported swallow severity and swallow- related quality of life. The central goal o this proposal is to investigate the effects of an eight-week regimen of EMST on bulbar function in persons with mild to moderate ALS using a blinded, sham-controlled, randomized clinical trial. The central hypothesis of this proposal is that eight-weeks of daily moderate intensity resistance EMST exercise will actively engage the expiratory and submental musculature, deter disuse atrophy and prolong function of airway protection, swallowing and functional oral intake in persons with ALS. The proposed study is designed to specifically determine the effect of EMST on: 1) expiratory force generating ability (maximum expiratory pressure); 2) swallow physiology and airway safety during swallow; 3) cough function; 4) functional oral intake, swallow-related quality of life and self-reported swallow severity; 5) and subsequent disease progression in persons with mild to moderate ALS. The results will provide valuable insight into the potential role of muscle strength training for improving bulbar function in persons with ALS and will help guide the future direction of research and treatment for this devastating disorder.