Exercise training in mitochondrial myopathy: A randomized controlled trial

Abstract

Patients with mitochondrial myopathies (MM) usually suffer from exercise intolerance due to their impaired oxidative capacity and physical deconditioning. We evaluated the effects of a 12-week supervised randomized rehabilitation program involving endurance training in patients with MM. Twenty MM patients were assigned to a training or control group. For three nonconsecutive days each week, patients combined cycle exercise at 70% of their peak work rate with three upper-body weight-lifting exercises performed at 50% of maximum capacity. Training increased maximal oxygen uptake (28.5%), work output (15.5%), and minute ventilation (40%), endurance performance (62%), walking distance in shuttle walking test (+95 m), and peripheral muscle strength (32%-62%), and improved Nottingham Health Profile scores (21.47%) and clinical symptoms. Control MM patients did not change from baseline. Results show that our exercise program is an adequate training strategy for patients with mitochondrial myopathy.

Endurance Exercise Training in Young Adults with Barth Syndrome: A Pilot Study

“…Endurance exercise training has also been found to be safe and improve peak exercise tolerance and cardiac and skeletal muscle function in conditions that share similar characteristics as BTHS: mitochondrial myopathies (Cejudo et al 2005;Jeppesen et al 2006Jeppesen et al , 2009 and congestive heart failure (Coats et al 1992;Delagardelle et al 2002;Giannuzzi et al 2003;Bartlo 2007). In patients with other mitochondrial myopathies, an endurance exercise program (3-5Â/week, 12 weeks, 70% VO 2peak ) increased peak oxygen consumption 23-29% and peak work rate 16-100% (Cejudo et al 2005;Jeppesen et al 2006Jeppesen et al , 2009. Also, 14 weeks of endurance exercise training increased peak oxygen consumption (~25%), peak skeletal muscle oxygen extraction (20%), and mitochondrial function, enzyme activity and volume (~50%) without an improvement in cardiac function in individuals with mitochondrial myopathies.…”

“…Endurance exercise training has been shown to improve subjective quality of life in non-BTHS heart failure (Tyni- Lenne et al 1996;Belardinelli et al 1999;Taylor et al 2014) and mitochondrial myopathy (Cejudo et al 2005). In the current study, endurance exercise training did not significantly improve overall (i.e., total score of the Minnesota Living with Heart Failure Questionnaire) subjective quality of life but did significantly improve scores on specific questions related to dyspnea and side effects of heart failure treatment.…”

“…Most importantly, endurance exercise training in patients with non-BTHS heart failure was safe, improved survival (Belardinelli et al 1999), decreased hospitalization (Belardinelli et al 1999), and increased quality of life (Coats et al 1992;Tyni-Lenne et al 1996;Belardinelli et al 1999). Similarly, endurance exercise training has been found to be safe, improve exercise capacity, increase skeletal muscle oxygen extraction, and improve quality of life in individuals with other non-BTHS mitochondrial myopathies (Taivassalo et al 2001;Cejudo et al 2005;Jeppesen et al 2006Jeppesen et al , 2009. However, the effect of endurance exercise training on these measures in BTHS is unknown.…”

Functional Diagnostics in Mitochondrial Diseases

“…Generally speaking, recent studies on patients with different types of muscle disease have demonstrated the beneficial effects of aerobic training on exercise performance and in terms of improved quality of life Orngreen et al 2005). Benefits of aerobic exercise training on blood lactate kinetics, muscular strength, oxygen consumption and endurance performance have also been demonstrated in MD patients (Cejudo et al 2005). This benefits are thought to be a consequence of modifications in the utilization rate of fuels for oxidative metabolism during exercise, with a reduction in muscle lactate production and respiratory change ratio (Mac Rae et al 1995).…”

Effects of Gas Exchange on Acid‐Base Balance

“…It is of interest that a mild, low-volume exercise-training program using patients with a variety of mitochondrial enzyme defects resulted in significant improvements inVo 2 max, muscle strength and endurance, maximal heart rate, respiratory rate, and VE, and in reducing the lactacidosis (37,297). Therefore, regular exercise appears to have resulted in the improved activities of many enzymes in the respiratory chain in heart, respiratory and skeletal muscles, consistent with the hypothesis that these individuals also suffer from diminished oxidative capacity due to chronic inactivity.…”