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February 7, 2005

Assessment of adrenoleukodystrophy lesions by high field MRS in non-sedated pediatric patients

February 8, 2005 issue
64 (3) 434-441

Abstract

Background: Early detection of white matter lesions in childhood-onset cerebral adrenoleukodystrophy (ALD) is important as hematopoietic cell transplantation (HCT), currently the only effective treatment, is beneficial only if performed early in the disease course.
Objective: To establish reliable biochemical markers of cerebral disease progression in patients with ALD to aid in treatment planning.
Methods: The authors used proton magnetic resonance spectroscopy (MRS) in combination with LCModel analysis to quantify brain metabolites in small volumes (3 to 16 mL) in the occipital and frontal white matter and the splenium of the corpus callosum of 17 unsedated patients and 26 healthy volunteers (adult n = 21, age-matched n = 5) at 4 tesla.
Results: Absolute concentrations of 12 metabolites were reliably determined, seven of which were established as markers of lesion development. Among these, creatine and choline containing compounds were the weakest markers while N-acetylaspartate, glutamine, and lipids + lactate were the strongest. The large extent of changes in the markers enabled detection of early neurochemical changes in lesion formation prior to detection of abnormalities by conventional MRI. Concentrations of a number of metabolites were also significantly different between normal appearing white matter of patients and controls indicating biochemical alterations in the absence of cerebral disease. Neurochemical improvements following HCT were measured in six patients.
Conclusions: The progression of adrenoleukodystrophy, as well as effectiveness of its treatment, can be assessed with high precision using high field 1H magnetic resonance spectroscopy in individual patients without the need for sedation.

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Information & Authors

Information

Published In

Neurology®
Volume 64Number 3February 8, 2005
Pages: 434-441
PubMed: 15699371

Publication History

Published online: February 7, 2005
Published in print: February 8, 2005

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Authors

Affiliations & Disclosures

G. Öz, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
I. Tkáč, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
L. R. Charnas, MD, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
I. Y. Choi, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
K. J. Bjoraker, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
E. G. Shapiro, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.
R. Gruetter, PhD
From the Center for MR Research (Drs. Öz, Tkáč, Choi, and Gruetter), Department of Radiology, and Departments of Pediatrics (Drs. Charnas, Bjoraker, and Shapiro) and Neuroscience (Dr. Gruetter), University of Minnesota, Minneapolis. I.Y.C. is currently affiliated with The Nathan Kline Institute, Medical Physics, Orangeburg, NY.

Notes

Address correspondence and reprint requests to Dr. Gülin Öz, Center for MR Research, 2021 6th St. SE, Minneapolis, MN 55455; e-mail: [email protected]

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