Seven-Tesla Magnetic Resonance Imaging in Wilson Disease Using Quantitative Susceptibility Mapping for Measurement of Copper Accumulation

Abstract

In Wilson disease (WD), the copper content of cerebral tissue is increased, particularly in the basal ganglia. This study investigated whether a change in magnetic susceptibility can be detected using quantitative susceptibility mapping of the brain in patients with WD compared with healthy controls. Eleven patients with WD (6 with the neurological form, 5 with the hepatic form) and 10 age-matched healthy controls who gave informed consent were examined at 7 T in a whole-body scanner (MAGNETOM; Siemens Medical Solutions, Erlangen, Germany) using a 24-channel phased array coil (Nova Medical). For imaging, a 3-dimensional spoiled gradient multiecho sequence (repetition time, 40 milliseconds; echo time, 9.76/19.19/28.62 milliseconds; bandwidth, 150 hertz per pixel; voxel size, 0.6 × 0.6 × 0.8 mm) was used. The susceptibility of selected regions (substantia nigra, red nucleus, pallidum, putamen, caudate nucleus) was analyzed in susceptibility maps. The patients with WD showed significantly increased susceptibility (P value, 0.001-0.05) in all analyzed regions compared with healthy controls. This was evident not only in patients with a neurological syndrome but also, with lower values, in patients with isolated hepatic manifestations. The distribution patterns of copper accumulation were different between the patients with neurological and non-neurological manifestations of the disease. In neurologically symptomatic and asymptomatic patients with WD, we found increased magnetic susceptibility in the brain tissue using quantitative susceptibility mapping.