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, PhD
From the Center for MR Research (Drs. Öz, Tká, 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.
Address correspondence and reprint requests to Dr. Gülin Öz, Center for MR Research, 2021 6th St. SE, Minneapolis, MN 55455; e-mail: gulin{at}cmrr.umn.edu
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.
See also page 406
Supported by a grant from the March of Dimes Birth Defects Foundation (E.G.S.). The Center for MR Research is supported by MIND Institute and a National Center for Research Resources (NCRR) biotechnology research resource grant P41RR08079 and the General Clinical Research Center at the University of Minnesota by NCRR grant M01RR00400.
Received June 21, 2004. Accepted in final form September 15, 2004.
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