• European radiology · Apr 2002

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

    • T A G M Huisman, T Thiel, B Steinmann, G Zeilinger, and E Martin.
    • Department of Neuroradiology and Magnetic Resonance, University Children's Hospital Zurich, Switzerland. thierry@nmr.mgh.harvard.edu
    • Eur Radiol. 2002 Apr 1; 12 (4): 858-61.

    AbstractNonketotic 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.

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