Demonstration of glycine peaks at 3.50 ppm in a patient with van der Knaap syndrome.

Elevated levels of glycine in the CSF have recently been documented in van der Knaap syndrome (diffuse leukoencephalopathy associated with cystic degeneration of the white matter). This report describes a patient affected with the syndrome in whom proton MR spectroscopy showed probable glycine peaks at 3.50 ppm in the brain parenchyma. An experimental study with a superconducting 9.397-T laboratory spectrometer identified the location of pure glycine at 3.52 ppm, originating from the methylene (CH(2)) group of the molecule. This suggests that the peak at 3.50 ppm in patients with van der Knaap syndrome may belong to glycine, as a slight shift in peak resonances is possible as a result of spatial electron interactions.