WILSON DISEASE - FINDINGS AT MR-IMAGING AND CT OF THE BRAIN WITH CLINICAL CORRELATION

PURPOSE: To describe the spectrum of brain abnormalities in Wilson dis ease (hepatolenticular degeneration) as depicted at magnetic resonance (MR) imaging and computed tomography (CT) and to relate these finding s to neurologic and hepatologic abnormalities. MATERIALS AND METHODS: Fifty patients with Wilson disease participated in the cross-sectional study: Patients underwent cerebral MR imaging (n = 49), CT (n = 44), abdominal duplex ultrasound (US) (n = 46), and neurologic examination (n = 50) within a week. Relative risk and the Fisher exact test were u sed for statistical analysis. RESULTS: Supratentorial and infratentori al abnormalities in the gray and white matter were found in the pyrami dal and extrapyramidal system. In Wilson disease, an abnormal striatum depicted on MR images correlated with pseudoparkinsonian signs, an ab normal dentatothalamic tract correlated with cerebellar signs, and an abnormal pontocerebellar tract correlated with pseudoparkinsonian sign s. The presence of portosystemic shunt was strongly associated with ab normality of the globus pallidus. CONCLUSION: MR imaging findings were of some use in the clinical treatment of patients with Wilson disease .