Abstract
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 × 2 × 2 cm cubic volumes at relatively long (135-ms) echo times con- tain additional signals that are characteristic of glutamine and indicate that glutamine is present in very high concen- trations in the brain. The findings are consistent with the hypothesis that intracerebral accumulation of glutamine contributes to the encephalopathy associated with hyper-ammonemia. In one of the children, spectra obtained after treatment showed a marked decrease in the glutamine signals.
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Connelly, A., Cross, J., Gadian, D. et al. Magnetic Resonance Spectroscopy Shows Increased Brain Glutamine in Ornithine Carbamoyl Transferase Deficiency. Pediatr Res 33, 77–81 (1993). https://doi.org/10.1203/00006450-199301000-00016
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DOI: https://doi.org/10.1203/00006450-199301000-00016
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