Effect of L-carnitine on exercise performance in patients with mitochondrial myopathy

Effect of L-carnitine on exercise performance in patients with mitochondrial myopathy

Author Gimenes, Ana Cristina Oliveira Autor UNIFESP Google Scholar
Bravo, Daniela Manzoli Autor UNIFESP Google Scholar
Nápolis, Lara Maris Autor UNIFESP Google Scholar
Mello, Marco Tulio de Autor UNIFESP Google Scholar
Oliveira, Acary Souza Bulle Autor UNIFESP Google Scholar
Neder, Jose Alberto Autor UNIFESP Google Scholar
Nery, Luiz Eduardo Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Exercise intolerance due to impaired oxidative metabolism is a prominent symptom in patients with mitochondrial myopathy (MM), but it is still uncertain whether L-carnitine supplementation is beneficial for patients with MM. The aim of our study was to investigate the effects of L-carnitine on exercise performance in MM. Twelve MM subjects (mean age±SD=35.4±10.8 years) with chronic progressive external ophthalmoplegia (CPEO) were first compared to 10 healthy controls (mean age±SD=29±7.8 years) before they were randomly assigned to receive L-carnitine supplementation (3 g/daily) or placebo in a double-blind crossover design. Clinical status, body composition, respiratory function tests, peripheral muscle strength (isokinetic and isometric torque) and cardiopulmonary exercise tests (incremental to peak exercise and at 70% of maximal), constant work rate (CWR) exercise test, to the limit of tolerance [Tlim]) were assessed after 2 months of L-carnitine/placebo administration. Patients with MM presented with lower mean height, total body weight, fat-free mass, and peripheral muscle strength compared to controls in the pre-test evaluation. After L-carnitine supplementation, the patients with MM significantly improved their Tlim (14±1.9 vs 11±1.4 min) and oxygen consumption ( V ˙ O 2 ) at CWR exercise, both at isotime (1151±115 vs 1049±104 mL/min) and at Tlim (1223±114 vs 1060±108 mL/min). These results indicate that L-carnitine supplementation may improve aerobic capacity and exercise tolerance during high-intensity CWRs in MM patients with CPEO.
Keywords Exercise tolerance
Muscle strength
Mitochondrial myopathy
L-carnitine supplementation
Language English
Date 2015-01-01
Published in Brazilian Journal of Medical and Biological Research. Associação Brasileira de Divulgação Científica, v. 00, n. ahead, p. 000-000, 2015.
ISSN 0100-879X (Sherpa/Romeo)
Publisher Associação Brasileira de Divulgação Científica
Origin http://dx.doi.org/10.1590/1414-431X20143467
Access rights Open access Open Access
Type Article
SciELO ID S0100-879X2015005043467 (statistics in SciELO)
URI http://repositorio.unifesp.br/handle/11600/8779

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