title: CT hypodensity on cerebral white matter in Wilson's disease.
abstract: Wilson's disease in an autosomal recessive disorder of copper metabolism where systemic manifestations are secondary to the accumulation of copper in hepatic, nervous and other tissues. In CNS, the structural lesions most commonly found by CT scan are ventricular dilatation, cortical atrophy, basal ganglia hyperdensities, and brainstem and cerebellar atrophy. Degenerative changes of cerebral white matter seen on early anatomo-pathologic studies, but were almost never found on CT scan from recently described patients. We report a case of Wilson's disease with an unusually rapid deterioration where asymmetric low-densities in the subcortical white matter were disclosed by CT scan.
authors.affiliation: Medical Genetics Unit, Clinical Hospital of Porto Alegre, Brasil.
authors.forename: L B
authors.initials: LB
authors.lastname: Jardim
authors.forename: A
authors.initials: A
authors.lastname: Carneiro
authors.forename: S
authors.initials: S
authors.lastname: Hansel
authors.forename: C R
authors.initials: CR
authors.lastname: Rieder
authors.forename: R
authors.initials: R
authors.lastname: Giugliani
mesh_terms: D000293:Adolescent; D002540:Cerebral Cortex / Q000000981:diagnostic imaging; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D014057:Tomography, X-Ray Computed