Diffusion MR Imaging Changes Associated with Wilson Disease

Summary: We herein report the case of a patient with Wilson disease. The patient underwent echo-planar diffu-sion MR imaging twice, 1.5 years apart. The lesions were in the putamina and caudate nuclei. At the first examination, undertaken after onset of extrapyramidal symptoms, a re-stricted diffusion pattern was evident. It is likely that this corresponded to cell swelling caused by the accumulation of copper. On the images obtained 1.5 years later, an opposite pattern (an elevated diffusion pattern) was noted. It is likely that this reflected necrosis, spongiform degeneration, and demyelination, which are among the known his-topathologic changes associated with Wilson disease. Neurologic symptoms of Wilson disease are usually caused by cerebral copper accumulation sufficient to destroy nerve cells. MR imaging findings in cases o