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

Eur Radiol. 2002 Apr;12(4):858-61. doi: 10.1007/s003300101073. Epub 2001 Aug 28.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brain / metabolism*
  • Brain Chemistry
  • Glycine / analysis
  • Humans
  • Hyperglycinemia, Nonketotic / diagnosis*
  • Infant, Newborn
  • Magnetic Resonance Spectroscopy
  • Male


  • Glycine