Cerebral 1H MR spectroscopy revealing white matter NAA decreases in glutaric aciduria type I.
MR spectroscopy in two patients with glutaric aciduria type I revealed reductions in the white matter N-acetylaspartate signal, in the more severe case accompanied by a loss of glutamate and the appearance of lactate signals.
Department of Radiology, University Medical Center Groningen and University of Groningen, Groningen, The Netherlands. p.e.sijens@rad.umcg.nl
P E
PE
Sijens
G P A
GP
Smit
L C
LC
Meiners
M
M
Oudkerk
F J
FJ
van Spronsen
D000592:Amino Acid Metabolism, Inborn Errors / Q000235:genetics* / Q000378:metabolism; D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000378:metabolism; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005971:Glutamates / Q000378:metabolism; D050770:Glutaryl-CoA Dehydrogenase / Q000172:deficiency / Q000235:genetics*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007294:Inositol / Q000378:metabolism; D007773:Lactates / Q000378:metabolism; D008279:Magnetic Resonance Imaging; D009682:Magnetic Resonance Spectroscopy; D008297:Male
D005971:Glutamates; D007773:Lactates; D001224:Aspartic Acid; D007294:Inositol; C000179:N-acetylaspartate; D050770:Glutaryl-CoA Dehydrogenase