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Localized Proton MR Spectroscopy in Infants with Urea Cycle Defect

Choong-Gon ChoiGo,a and Han Wook Yooa

a From the Departments of Radiology (C.G.C.) and Pediatrics (H.W.Y), Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.



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  		FIG 1. A neonate with OTC deficiency (6 days).

A and B, Axial T2-weighted (3000/102) images obtained with a fast spin-echo sequence and a 16-cm field of view, 256 x 192 matrix, and 5-mm contiguous sections, with the voxel locations and voxel sizes (7.2–8.0 cm3) as indicated for proton MR spectroscopy (STEAM sequence 3000/30).

C, Proton MR spectrum obtained from the parietal gray matter of an age-matched control subject (full-term neonate, 5 days). Glx = glutamine/glutamate complex, tCr = total Cr, mI = myo-inositol, other abbreviations are the same as used in the text.

D, Proton MR spectrum obtained from the parietal gray matter of the patient shows a prominent increase of glutamine/glutamate and lipid/lactate complexes, and relatively well preserved NAA, tCr, Cho, and mI as compared with normal spectrum.

E, Proton MR spectrum obtained from the basal ganglia of the patient also shows similar findings; a prominent increase of glutamine/glutamate and lipid/lactate complexes



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  		FIG 2. An infant with citrullinemia (57 days).

A, Axial T1-weighted (600/16) image obtained with a spin-echo sequence and a 20-cm field of view, 256 x 192 matrix, and 5-mm contiguous sections, with the voxel location and voxel size (7.2 cm3) as indicated for proton MR spectroscopy (STEAM sequence 3000/30).

B, Proton MR spectrum obtained from the parietal white matter of an age-matched control subject (2 months). Glx = glutamine/glutamate complex, tCr = total Cr, mI = myo-inositol, other abbreviations are the same as used in the text.

C, Proton MR spectrum obtained from the parietal white matter of the patient shows a prominent increase of glutamine/glutamate and lipid/lactate complexes, and a decrease of NAA, tCr, and mI as compared with normal spectrum. The amplitude of the Cho peak is relatively well preserved.

D, A follow-up axial T1-weighted (490/14) image obtained at the age of 10 months shows severe atrophy and cystic encephalomalacia in both cerebral hemispheres





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X