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Abnormal carnitine distribution in the muscles of patients with idiopathic inflammatory myopathy
J Arenas1, M R Gonzalez-Crespo, Y Campos
1Hospital Doce de Octubre, Madrid, Spain.
Objective:
To analyze the levels of free carnitine and carnitine esters in the muscles of patients with inflammatory myopathies.
Methods:
Six men and 7 women with inflammatory myopathy and 25 age-matched healthy controls were studied. Free carnitine and carnitine esters in muscle homogenates were measured by a radiochemical procedure. Muscle histochemical staining and measurement of respiratory chain enzyme activity were also performed.
Results:
Eleven patients had muscle carnitine insufficiency. Five of them had subsarcolemmal oxidative accumulations, 5 had lipid droplets, and 4 had defects of the respiratory chain enzyme complexes.
Conclusion:
Abnormal distribution of muscle carnitine is present in patients with inflammatory myopathies and could impair muscle function. Coexistent mitochondrial dysfunction may contribute to carnitine insufficiency.