Structural and neurochemical evaluation of the brain and pons in patients with Wilson's disease

Abstract

The aim of this study was to examine the structural-neurochemical abnormalities of the frontal white matter (FWM), deep gray matter nuclei, and pons in patients with Wilson's disease (WD) using proton magnetic resonance spectroscopy (MRS) and diffusion-weighted imaging (DWI).

Nine patients with WD and 14 age-matched controls were examined with MRS. N-Acetylaspartate (NAA), choline (Cho), and creatine (Cr) peaks were calculated. DWI scans from six WD patients and six controls were also obtained. The relative metabolite ratios and apparent diffusion coefficient (ADC) values of the WD patients were compared to those of the control subjects by using statistical measures.

Measurements in the thalamus and pons showed significantly lower NAA/Cho and NAA/Cr ratios in the WD group than in the control group (P < 0.05). Thalamic and pontine Cho/Cr ratios in the patient group were significantly higher than those of the control group (P < 0.05). No statistically significant relation was found between the patient and control groups as a result of the MRS examinations of FWM and all ADC measurements (P > 0.05).

MRS is a noninvasive, valuable modality for detecting structural-neurochemical changes of the brain stem and deep gray matter in patients with WD. The contribution of DWI in these patients is limited.