Title | Brain MRS glutamine as a biomarker to guide therapy of hyperammonemic coma. |
Publication Type | Journal Article |
Year of Publication | 2017 |
Authors | O'Donnell-Luria AH, Lin AP, Merugumala SK, Rohr F, Waisbren SE, Lynch R, Tchekmedyian V, Goldberg AD, Bellinger A, J McFaline-Figueroa R, Simon T, Gershanik EF, Levy BD, Cohen DE, Samuels MA, Berry GT, Frank NY |
Journal | Mol Genet Metab |
Volume | 121 |
Issue | 1 |
Pagination | 9-15 |
Date Published | 2017 05 |
ISSN | 1096-7206 |
Keywords | Brain, Coma, Female, Gastric Bypass, Glutamates, Glutamine, Humans, Hyperammonemia, Magnetic Resonance Spectroscopy, Middle Aged, Phenylacetates, Sodium Benzoate, Treatment Outcome |
Abstract | Acute idiopathic hyperammonemia in an adult patient is a life-threatening condition often resulting in a rapid progression to irreversible cerebral edema and death. While ammonia-scavenging therapies lower blood ammonia levels, in comparison, clearance of waste nitrogen from the brain may be delayed. Therefore, we used magnetic resonance spectroscopy (MRS) to monitor cerebral glutamine levels, the major reservoir of ammonia, in a gastric bypass patient with hyperammonemic coma undergoing therapy with N-carbamoyl glutamate and the ammonia-scavenging agents, sodium phenylacetate and sodium benzoate. Improvement in mental status mirrored brain glutamine levels, as coma persisted for 48h after plasma ammonia normalized. We hypothesize that the slower clearance for brain glutamine levels accounts for the delay in improvement following initiation of treatment in cases of chronic hyperammonemia. We propose MRS to monitor brain glutamine as a noninvasive approach to be utilized for diagnostic and therapeutic monitoring purposes in adult patients presenting with idiopathic hyperammonemia. |
DOI | 10.1016/j.ymgme.2017.03.003 |
Alternate Journal | Mol. Genet. Metab. |
PubMed ID | 28408159 |
Submitted by kej2006 on June 6, 2018 - 4:12pm