Abstract
Mitochondrial encephalomyopathy, lactic acidosis, and recurrent stroke-like episodes syndrome (MELAS) is a rare degenerative disease. Recent studies have shown that resistant training (RT) can ameliorate muscular force in mitochondrial diseases. However, the effects of RT in MELAS are unknown. The aim of this case report was to investigate the effects of RT on skeletal muscle and mitochondrial function in a 21-years old patient with MELAS. RT included 12 weeks of RT at 85% of 1 repetition maximum. Body composition (DXA), in vivo mitochondrial respiration capacity (mVO2) utilizing Near-infrared spectroscopy on the right plantar-flexor muscles, maximal voluntary torque (MVC), electrically evoked resting twitch (EET) and maximal voluntary activation (VMA) of the right leg extensors (LE) muscles were measured with the interpolated twitch technique. The participant with MELAS exhibited a marked increase in body mass (1.4 kg) and thigh muscle mass (0.3 kg). After the training period MVC (+5.5 Nm), EET (+2.1 N⋅m) and VMA (+13.1%) were ameliorated. Data of mVO2 revealed negligible changes in the end-exercise mVO2 (0.02 mM min-1), Δ mVO2 (0.09 mM min-1), while there was a marked amelioration in the kinetics of mVO2 (τ mVO2; Δ70.2 s). This is the first report of RT-induced ameliorations on skeletal muscle and mitochondrial function in MELAS. This case study suggests a preserved plasticity in the skeletal muscle of a patient with MELAS. RT appears to be an effective method to increase skeletal muscle function, and this effect is mediated by both neuromuscular and mitochondrial adaptations.
Highlights
Mitochondrial diseases caused by mitochondrial DNA mutations are rare pathologies causing devastating physical and neural impairments (Matsumoto et al, 2005; Sproule and Kaufmann, 2008)
The participant with MELAS exhibited an increase in maximal voluntary contraction (MVC) (5.5 N·m), evoked resting twitch (EET) (2.1 N·m) and VMA (13.1%)
In the present study we investigated the effects of resistant training (RT) on skeletal muscle and mitochondrial function in a 21 years old male patient with MELAS
Summary
Mitochondrial diseases caused by mitochondrial DNA mutations are rare pathologies causing devastating physical and neural impairments (Matsumoto et al, 2005; Sproule and Kaufmann, 2008). It is important to note, that besides a remarkable impairment of the aerobic metabolism, patients with MELAS presents severe skeletal muscle losses This imply that, perhaps, a training method that can induce both positive changes of mitochondrial (Porter et al, 2015) and neuromuscular function of skeletal muscle may provide large benefits in terms of exercise capacity and quality of life. The participants completed an in vivo mitochondrial respiration capacity protocol utilizing a Nearinfrared spectroscopy device (NIRS) on the right plantar-flexor muscles. From a clinical point of view, the participant with MELAS showed dysphagia to solids (he had a modified diet and assumed food integrators) He presented with easy fatigability and decreased muscle mass. Control group data and muscle function values measured in the participant with MELAS during six repetitions of isometric LE are presented as mean ± standard deviation. Due to the descriptive nature of this single case study any specific analysis was applied to the collected data
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