It is well known that in patients having NaV therapy, hyperammonemia with carnitine deficiency is frequently observed. In this study, 25 cases (IO male, 15 female; mean age:23) receiving NaV monotherapy for an average of three years; has been given 15 mg/kg carnitine for one month period. At the beginning and at the end of the first month of the therapy the levels of plasma carnitine. ammonium and serum transaminases has been measured. Also patients echocardiogram (ECHO), and electrocardiograms (ECG) were carried out. The results;were compared with the results of 23 normal cases within the same age group.