Magnetic resonance imaging in hepatolenticular degeneration. Analysis of 9 cases

Nine patients with biochemically proved Wilson's disease underwent magnetic resonance imaging (MRI) of the brain. Areas of abnormal signal, long T1 and long T2, caused by gliosis and edema were seen in the lenticula, thalami, caudatum, brain stem as well as in the dentate nuclei. The abnormalities were bilateral and symmetric. Asymmetric focal white matter lesions were noted in a few patients. Focal atrophies were seen in the head of caudatum. Symmetric cavitations were only seen in the lenticular nuclei. Acute progressive type (2 patients) was characterized by cavity formation, and chronic progressive type (7 patients) by gliosis, edema and focal atrophy.