Computed tomography in hepatolenticular degeneration (Wilson's disease)

Fifteen cases of hepatolenticular degeneration have been studied by means of computerized cranial tomography (CT). The common abnormalities were hypodense areas in the regions of the basal ganglia, in 9 cases; ventricular dilatation, in 7 cases; cortical atrophy, in 5 cases and brain-stem atrophy, in 4 cases. CT abnormalities were most common and most marked in patients with neurological presentations, but 1 of 3 asymptomatic patients discovered by the genetic screening of the affected family had hypodense areas in bilateral lenticular nuclei.