Clinical, CT and evoked potential manifestations inWilson's disease with cerebral white matter involvement

Four patients with Wilson's disease had CT manifestations of cerebral white matter lesions involving frontal lobes in two patients but extending to other cerebral areas in the remaining two. Two patients with extensive white matter involvement were bed-ridden with generalized flexion rigidity and spasticity, but the other 2 patients led an independent life with minimal extrapyramidal symptoms. Three patients developed tonic focal seizures with or without secondary generalization. Scalp-recorded somatosensory evoked potentials (SEPs) over the affected hemispheres were absent but the spinal SEPs and brain stem auditory evoked potentials were preserved. The evoked potential data support the CT findings that the white matter involvement in Wilson's disease was primarily confined to the cerebral hemispheres.