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/creati...

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Main Authors: Mitra Assadi, Dah-Jyuu Wang, Yadira Velazquez-Rodriquez, Paola Leone
Format: Article
Language:English
Published: SAGE Publishing 2013-01-01
Series:Journal of Central Nervous System Disease
Online Access:https://doi.org/10.4137/JCNSD.S11861
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author Mitra Assadi
Dah-Jyuu Wang
Yadira Velazquez-Rodriquez
Paola Leone
author_facet Mitra Assadi
Dah-Jyuu Wang
Yadira Velazquez-Rodriquez
Paola Leone
author_sort Mitra Assadi
collection DOAJ
description 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.
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spelling doaj.art-dc54772f8a4e40d481c59189e65db1032022-12-21T20:02:01ZengSAGE PublishingJournal of Central Nervous System Disease1179-57352013-01-01510.4137/JCNSD.S11861Multi-Voxel 1H-MRS in Metachromatic LeukodystrophyMitra Assadi0Dah-Jyuu Wang1Yadira Velazquez-Rodriquez2Paola Leone3Departments of Neurology and Pediatrics, Robert Wood Johnson Medical School.Department of Radiology, University of Pennsylvania.Department of Neurology, Rowan Medical School.School of Osteopathic Medicine, Rowan University.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.https://doi.org/10.4137/JCNSD.S11861
spellingShingle Mitra Assadi
Dah-Jyuu Wang
Yadira Velazquez-Rodriquez
Paola Leone
Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
Journal of Central Nervous System Disease
title Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
title_full Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
title_fullStr Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
title_full_unstemmed Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
title_short Multi-Voxel 1H-MRS in Metachromatic Leukodystrophy
title_sort multi voxel 1h mrs in metachromatic leukodystrophy
url https://doi.org/10.4137/JCNSD.S11861
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