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Discussion The results of the present study could be summarized as follows: 1) levocarnitine supplementation led to an increase in FC, AC and TC levels, irrespective of an improvement of hyperammonemia, 2) levocarnitine add-on therapy was effective in reducing high plasma NH3 concentrations in about half of the subjects with VPA-induced hyperammonemia, 3) baseline levels of AC and longitudinal changes of AC/FC ratios were correlated with fluctuation of NH3 levels from baseline, 4) levocarnitine treatment did not generally exacerbated mental status, but ameliorated a part of psychiatric symptoms