Demonstration of glycine peaks at 3.50 ppm in a patient with van der KnaapSyndrome

Elevated levels of glycine in the CSF have recently been documented in van der Knaap syndrome (diffuse leukoencephalopathy associated with cystic dege neration of the white matter). This report describes a patient affected wit h the syndrome in whom proton NIR spectroscopy showed probable glycine peak s at 3.50 ppm in the brain parenchyma. An experimental study with a superco nducting 9.397-T laboratory spectrometer identified the location of pure gl ycine at 3.52 ppm, originating from the methylene (CH2) group of the molecu le. 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 pos sible as a result of spatial electron interactions.