Proton MR Spectroscopy in Wilson Disease: Analysis of 36 Cases

Wilson disease (WD) is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. The literature regarding proton MR spectroscopy (MRS) in WD is scarce and controversial. The purpose of this study was to determine the brain metabolic changes due to WD...

Full description

Saved in:
Bibliographic Details
Published in:American journal of neuroradiology : AJNR 2005-05, Vol.26 (5), p.1066-1071
Main Authors: Lucato, Leandro T, Otaduy, Maria Concepcion G, Barbosa, Egberto R, Machado, Alexandre A. C, McKinney, Alexander, Bacheschi, Luiz A, Scaff, Milberto, Cerri, Giovanni G, Leite, Claudia C
Format: Article
Language:English
Subjects:
Adolescent
Adult
Biological and medical sciences
Brain
Child
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Fundamental and applied biological sciences. Psychology
Hepatolenticular Degeneration - metabolism
Humans
Investigative techniques, diagnostic techniques (general aspects)
Magnetic Resonance Spectroscopy
Male
Medical sciences
Miscellaneous
Nervous system
Neurology
Perception
Prospective Studies
Psychology. Psychoanalysis. Psychiatry
Psychology. Psychophysiology
Radiodiagnosis. Nmr imagery. Nmr spectrometry
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Wilson disease (WD) is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. The literature regarding proton MR spectroscopy (MRS) in WD is scarce and controversial. The purpose of this study was to determine the brain metabolic changes due to WD by using MRS. To our knowledge, this is the first time that MRS was performed in such a large sample of patients with WD. Thirty-six patients with WD and 37 healthy volunteers were examined with MRS in the parieto-occipital cortex, frontal white matter, and basal ganglia (BG). Ratios of the following metabolites were calculated in relation to creatine (Cr): N-acetylaspartate (NAA), choline (Cho), myo-inositol (mI), and glutamine/glutamate (Glx). The mean peak line width was measured on each spectrum. Compared with control subjects, patients with WD had significantly decreased NAA/Cr ratios in the three studied areas (P < .005) and an increased mI/Cr ratio in the BG (P < .001). Cho/Cr and Glx/Cr did not differ between the groups. The mean peak line in the BG was wider in patients than in control subjects. WD is unequivocally associated with MRS changes that could possibly be assigned to neuronal loss (in the three studied areas), to gliosis, and to iron and/or copper deposition in the BG.
ISSN:0195-6108
1936-959X