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.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Kojic J,Robertson PL,Quint DJ,Martin DM,Pang Y,Sundgren PC

doi

10.1016/j.pediatrneurol.2004.07.013

subject

Has Abstract

pub_date

2005-02-01 00:00:00

pages

143-6

issue

2

eissn

0887-8994

issn

1873-5150

pii

S0887-8994(04)00407-2

journal_volume

32

pub_type

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