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

Details

Serval ID
serval:BIB_75F79F257C12
Type
Article: article from journal or magazin.
Collection
Publications
Title
Assessment of adrenoleukodystrophy lesions by high field MRS in non-sedated pediatric patients.
Journal
Neurology
Author(s)
Oz G., Tkác I., Charnas L.R., Choi I.Y., Bjoraker K.J., Shapiro E.G., Gruetter R.
ISSN
1526-632X (Electronic)
ISSN-L
0028-3878
Publication state
Published
Issued date
2005
Volume
64
Number
3
Pages
434-441
Language
english
Notes
Publication types: Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, P.H.S.Publication Status: ppublish
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.
Keywords
Adolescent, Adrenoleukodystrophy/classification, Adrenoleukodystrophy/diagnosis, Aspartic Acid/analogs & derivatives, Aspartic Acid/analysis, Brain Chemistry, Child, Child, Preschool, Creatine/analysis, Disease Progression, Follow-Up Studies, Glutamine/analysis, Hematopoietic Stem Cell Transplantation/mortality, Humans, Inositol/analysis, Lactates/analysis, Lipids/analysis, Magnetic Resonance Spectroscopy/methods, Male, Neuropsychological Tests, Prognosis
Pubmed
Web of science
Create date
04/08/2010 15:28
Last modification date
20/08/2019 14:33
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