Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency
Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency
We have performed localized in vivo proton magnetic resonance spectroscopy on two females with ornithine carbamoyl transferase deficiency during episodes of acute hyperammonemic encephalopathy with focal neurologic abnormalities. Spectra obtained from 2 x 2 x 2 cm cubic volumes at relatively long (135-ms) echo times contain additional signals that are characteristic of glutamine and indicate that glutamine is present in very high concentrations in the brain. The findings are consistent with the hypothesis that intracerebral accumulation of glutamine contributes to the encephalopathy associated with hyperammonemia. In one of the children, spectra obtained after treatment showed a marked decrease in the glutamine signals.
77-81
Connelly, A.
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Cross, J.H.
3c10596a-3f6e-418d-9957-8ec0a4e5078c
Gadian, D.G.
2b0e526e-9bd0-4173-b5cd-ee32c8f66c57
Hunter, J.V.
00956d6c-542a-49e0-9e4d-711591d89ff6
Kirkham, F.J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Leonard, J.V.
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1 January 1993
Connelly, A.
0cb19e67-b09d-458b-8c30-1e9f8a279887
Cross, J.H.
3c10596a-3f6e-418d-9957-8ec0a4e5078c
Gadian, D.G.
2b0e526e-9bd0-4173-b5cd-ee32c8f66c57
Hunter, J.V.
00956d6c-542a-49e0-9e4d-711591d89ff6
Kirkham, F.J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Leonard, J.V.
d59675cd-3509-4e9b-9ad8-c7be47c6eea9
Connelly, A., Cross, J.H., Gadian, D.G., Hunter, J.V., Kirkham, F.J. and Leonard, J.V.
(1993)
Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency.
Pediatric Research, 33 (1), .
(doi:10.1203/00006450-199301000-00016).
Abstract
We have performed localized in vivo proton magnetic resonance spectroscopy on two females with ornithine carbamoyl transferase deficiency during episodes of acute hyperammonemic encephalopathy with focal neurologic abnormalities. Spectra obtained from 2 x 2 x 2 cm cubic volumes at relatively long (135-ms) echo times contain additional signals that are characteristic of glutamine and indicate that glutamine is present in very high concentrations in the brain. The findings are consistent with the hypothesis that intracerebral accumulation of glutamine contributes to the encephalopathy associated with hyperammonemia. In one of the children, spectra obtained after treatment showed a marked decrease in the glutamine signals.
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Published date: 1 January 1993
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Local EPrints ID: 429780
URI: http://eprints.soton.ac.uk/id/eprint/429780
ISSN: 0031-3998
PURE UUID: 40420244-572c-462d-9694-19409d39b5e0
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Date deposited: 05 Apr 2019 16:30
Last modified: 16 Mar 2024 03:22
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Author:
A. Connelly
Author:
J.H. Cross
Author:
D.G. Gadian
Author:
J.V. Hunter
Author:
J.V. Leonard
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