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J Cent Nerv Syst Dis. 2013 Jul 4;5:25-30. doi: 10.4137/JCNSD.S11861. Print 2013.

Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy.

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1
Departments of Neurology and Pediatrics, Robert Wood Johnson Medical School.

Abstract

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.

KEYWORDS:

MLD; lactate; mI; multi-voxel 1H-MRS

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