Proton magnetic resonance spectroscopy of the brain of a neonate with nonketotic hyperglycinemia: in vivo-in vitro (ex vivo) correlation

Nonketotic hyperglycinemia (NKH) is an inborn error of amino acid metabolism caused by a defect in the glycine cleavage multienzyme complex resulting in high concentrations of glycine within the brain and spinal cord. Quantitative magnetic resonance spectroscopy ((1)H-MRS) allows measurement of abso...

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Bibliographic Details
Published in:European radiology 2002-04, Vol.12 (4), p.858-861
Main Authors: Huisman, T A G M, Thiel, T, Steinmann, B, Zeilinger, G, Martin, E
Format: Article
Language:English
Subjects:
Brain - metabolism
Brain Chemistry
Glycine - analysis
Humans
Hyperglycinemia, Nonketotic - diagnosis
Infant, Newborn
Magnetic Resonance Spectroscopy
Male
Spectrum analysis
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Summary:Nonketotic hyperglycinemia (NKH) is an inborn error of amino acid metabolism caused by a defect in the glycine cleavage multienzyme complex resulting in high concentrations of glycine within the brain and spinal cord. Quantitative magnetic resonance spectroscopy ((1)H-MRS) allows measurement of absolute glycine concentrations within different parts of the brain in vivo. In addition, (1)H-MRS may be useful in monitoring treatment of NKH and to differentiate this disease from other disorders of glycine metabolism.
ISSN:0938-7994
1432-1084
DOI:10.1007/s003300101073