TY - JOUR
T1 - Proton MR spectroscopy in Wilson disease
T2 - Analysis of 36 cases
AU - Lucato, Leandro Tavares
AU - Otaduy, Maria Concepción G.
AU - Barbosa, Egberto R.
AU - Machado, Alexandre A.C.
AU - McKinney, Alexander M
AU - Bacheschi, Luiz A.
AU - Scaff, Milberto
AU - Cerri, Giovanni G.
AU - Leite, Claudia C.
PY - 2005/12/1
Y1 - 2005/12/1
N2 - BACKGROUND AND PURPOSE: 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. METHODS: 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 (ml), and glutamine/glutamate (Glx). The mean peak line width was measured on each spectrum. RESULTS: Compared with control subjects, patients with WD had significantly decreased NAA/Cr ratios in the three studied areas (P < .005) and an increased ml/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. CONCLUSION: 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.
AB - BACKGROUND AND PURPOSE: 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. METHODS: 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 (ml), and glutamine/glutamate (Glx). The mean peak line width was measured on each spectrum. RESULTS: Compared with control subjects, patients with WD had significantly decreased NAA/Cr ratios in the three studied areas (P < .005) and an increased ml/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. CONCLUSION: 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.
UR - http://www.scopus.com/inward/record.url?scp=33644826600&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33644826600&partnerID=8YFLogxK
M3 - Article
C2 - 15891161
AN - SCOPUS:33644826600
SN - 0195-6108
VL - 26
SP - 1066
EP - 1071
JO - American Journal of Neuroradiology
JF - American Journal of Neuroradiology
IS - 5
ER -