Effect of carnitine and essential fatty acid supplementation on the uptake of 11C-carnitine in muscle of a myopathic carnitine-deficient patient using positron emission scintigraphy

The aim of this study was to demonstrate the pattern of 11C-carnitine uptake after various treatment regimens in a myopathic carnitine-deficient patient and two normal volunteers, using a whole body counter specially adapted for positron emission. One carnitine-deficient patient and two normal volunteers were scanned after an intravenous injection of 11C-carnitine, both while on carnitine therapy and after discontinuation thereof. The third scan was done on the patient following carnitine and fatty acid therapy for 7 days. Both the carnitine-deficient patient and the normal volunteers showed improved 11C-carnitine uptake by thigh muscles after carnitine supplementation, and the carnitine-deficient patient even more so after carnitine and fatty acid supplementation. It is therefore concluded that the scintigraphic findings support the clinical impression that carnitine deficient patients improve after carnitine and essential fatty acid supplementation.