TY - JOUR T1 - Localized Proton MR Spectroscopy in Infants with Urea Cycle Defect JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 834 LP - 837 VL - 22 IS - 5 AU - Choong-Gon Choi AU - Han Wook Yoo Y1 - 2001/05/01 UR - http://www.ajnr.org/content/22/5/834.abstract N2 - Summary: Urea cycle defect is an inborn error of ammonium metabolism caused by a deficient activity of the enzymes involved in urea synthesis. Localized short-TE proton MR spectroscopy, performed in two infants who had citrullinemia and ornithine transcarbamylase deficiency, respectively, showed a prominent increase of glutamine/glutamate and lipid/lactate complex in both cases. N-acetylaspartate, total creatine, and myo-inositol were decreased in the infant with citrullinemia. Proton MR spectroscopy provided useful information for the diagnosis and understanding of the pathophysiology of urea cycle enzyme defect. ER -