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˙O2) 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.