Magnetic resonance imaging and proton MR spectroscopy in Wilson's disease

MRI of the brain and liver using T-2 relaxation time measurements and proto n spectroscopy (H-1-MRS) of the brain was performed in four siblings with W ilson's disease tone with clinical disease and three asymptomatic) as well as age- and sex-matched control subjects. The T-2 values of the liver were correlated with liver biopsy results. H-1-MRS of the left and right globus pallidus was obtained. The patient with clinical disease was examined three times, and two of three asymptomatic siblings twice. MR images of the brai n were abnormal in all four patients. High signal intensity areas in the po sterior thalamus, general atrophy and pontine myelinolysis were present in the patient with clinical manifestations. The T-2 measurements of these are as confirmed the results of image analysis. Apart from general brain atroph y, the changes in the patient with clinical disease were largely reversible . The T-2 values were significantly different from those of the control sub jects only in the globus pallidus. The NAA/Cho, NAA/Cr and Cho/Cr ratios fr om the H-1-MR spectra of globus pallidus showed no significant difference b etween patients and control subjects. The mean values of NAA/Cho and NAA/Cr were lower in patients with Wilson's disease than in the control subjects. One of the patients had hepatic steatosis, but the liver T-2 values were n o different to those of the control subjects. In conclusion, the MRI findin gs reflect the success of the specific therapy in patients. MRI thus seems to be useful in the follow-up of Wilson's disease.