Computed tomography in Wilson disease

Computed tomography (CT) was performed on five patients with the "cerebral form" and one with the "hepatic form" of Wilson disease. The diagnosis was confirmed biochemically and by the presence of Kayser-Fleischer rings in all cases. In four patients, CT was done at the time of diagnosis; in two of these patients, the scan was repeated at intervals after initiation of treatment. CT was abnormal in all patients with the "cerebral form." All CT abnormalities were of low density and were not changed by contrast infusion. The abnormalities involved the basal ganglia in all five patients; in two patients, areas of low density also involved the cerebellar nuclei and surrounding white matter. CT abnormalities, however, did not always correlate well with the clinical state and in two patients, CT findings worsened despite successful cupruresis. The one patient with the "hepatic form" had no CT abnormalities.