title: Brain glutamine by MRS in a patient with urea cycle disorder and coma.
abstract: In patients who undergo metabolic decompensation from urea cycle disorders, cerebrospinal fluid glutamine level may be a better marker of cerebral dysfunction than blood ammonia or glutamine levels. However, obtaining cerebrospinal fluid by lumbar puncture carries risk in these acutely ill patients with cerebral edema. Using magnetic resonance single voxel spectroscopy as an alternative to cerebrospinal fluid analysis, elevated brain glutamine/glutamate complex levels were detected in a patient with carbamyl phosphate synthetase deficiency, who had been comatose for many days after normalization of blood ammonia and improvement in blood glutamine levels. Brain glutamine by single voxel spectroscopy decreased toward normal with neurologic recovery. We conclude that brain glutamine may be a better marker than serum ammonia for the management of urea cycle disorders, particularly in patients with prolonged mental status changes.
authors.affiliation: Department of Pediatrics, the University of Michigan School of Medicine, Ann Arbor, MI 48109-0203, USA.
authors.forename: Jasna
authors.initials: J
authors.lastname: Kojic
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Robertson
authors.forename: Douglas J
authors.initials: DJ
authors.lastname: Quint
authors.forename: Donna M
authors.initials: DM
authors.lastname: Martin
authors.forename: Yuxi
authors.initials: Y
authors.lastname: Pang
authors.forename: Pia C
authors.initials: PC
authors.lastname: Sundgren
mesh_terms: D000293:Adolescent; D001921:Brain / Q000378:metabolism*; D020165:Carbamoyl-Phosphate Synthase I Deficiency Disease / Q000378:metabolism* / Q000628:therapy; D005260:Female; D018698:Glutamic Acid / Q000378:metabolism*; D005973:Glutamine / Q000378:metabolism*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D020127:Recovery of Function; D016896:Treatment Outcome
chemical_list: D005973:Glutamine; D018698:Glutamic Acid