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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 26:1066-1071, May 2005
© 2005 American Society of Neuroradiology

BRAIN

Proton MR Spectroscopy in Wilson Disease: Analysis of 36 Cases

Leandro T. Lucato^a, Maria Concepción G. Otaduy^a, Egberto R. Barbosa^b, Alexandre A. C. Machado^b, Alexander McKinney^c, Luiz A. Bacheschi^b, Milberto Scaff^b, Giovanni G. Cerri^a and Claudia C. Leite^a

^a Department of Radiology, the Clinics Hospital of the University of Sao Paulo, School of Medicine, Brazil
^b Department of Neurology, the Clinics Hospital of the University of Sao Paulo, School of Medicine, Brazil
^c Department of Radiology, University of Minnesota Medical School and Hennepin County Medical Center, Minneapolis

Address reprint requests to Leandro Tavares Lucato, Rua Professor Pedreira de Freitas, 372 ap. 101-E, Tatuape, Sao Paulo S.P., Brazil CEP 03312–052

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 (mI), 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 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.

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.