Pilot Study of Progressive Resistance Training for Sedentary Adults with Charcot-Marie-Tooth

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Charcot-Marie-Tooth (CMT) is the most frequently inherited peripheral neuropathy affecting around 1 in 2500 persons. CMT causes slow degeneration of the nerves in the extremities including feet, legs, arms and hands. Typically muscles weaken and atrophy due to the loss of activation by the affected nerves. In the past, persons with CMT have been discouraged from performing exercise and physical activity because clinicians believed that such activity might hasten the disease; these beliefs have never been validated by studies. However, it is now known that regular exercise or activity helps maintain good health and function in most populations. Inactivity and a poor muscle mass can also lead to metabolic syndrome, increasing morbidity and the risk of developing diabetes and cardiovascular disease. These metabolic diseases may even worsen the symptoms of Charcot-Marie-Tooth disease, for example diabetes can worsen CMT symptoms. More evidence is required on exercise for persons with CMT, especially at moderate to high intensities. Recent research has shown that light and moderate intensity exercise is not detrimental to persons with CMT and offers many of the same benefits to persons with CMT as it does to the general population . The few previous studies have used home-based low intensity training (i.e. 20-30% maximum lift) for the upper limbs and moderate intensity for the legs (i.e. 40-50% maximum lift). In the neurologically normal population, muscle and strength gains are correlated with the intensity of exercise, i.e. high intensity training develops greater strength and muscle mass. A considerable amount of weakness and muscle atrophy in persons with CMT may be due to secondary disuse due inactivity. Higher intensity training could be beneficial for those with CMT to improve muscle mass, power and strength. The benefits of intense training might also provide persons with CMT from developing secondary metabolic disease or lead to improve functional ability in daily activities. We propose to conduct a study investigating the benefits of a high intensity training gym-based program (i.e. power training) for individuals with CMT. The proposed project is an eight-week resistance training randomized controlled pilot trial. This pilot study will provide information on the effectiveness of progressive resistance training (PRT) for people with CMT. We expect power training may benefit individuals with CMT by increasing muscle mass (strength and metabolic benefits) and muscle power (increased function). This research will provide data for a larger National Health and Medical Research Council funding application that will have the statistical power to answer more specific questions about CMT and exercise. Recently, many people with CMT have become aware that exercise might be beneficial for them, but do not understand how to accommodate exercise in their lives. Therefore, the results of this pilot study will be promoted to educate Exercise Sports Science Australia (ESSA) and Australian Accredited Exercise Physiologists (AEP) on the benefits of exercise for CMT. In time, we hope that people with CMT will be able to easily contact AEPs in the community for advice on exercise.

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