Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy.

Metachromatic leukodystrophy (MLD) is characterized by the accumulation of sulfatide sphingolipids in the brain and peripheral nerves. We report metabolite alterations recorded using multi-voxel proton spectroscopy of the brain in four children with MLD. The data revealed elevated myoinositol/creatine and lactate/creatine ratios as well as decreased N-acetyl aspartate/creatine ratios. We propose that elevation in myoinositol and lactate are caused by astrocytic gliosis and may be used as biomarkers for disease progression in MLD.