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Wilson’s disease with extensive cortico-subcortical lesions represents a rare pathological condition. We experienced an unusual case of prominent frontal cortico-subcortical lesions in association with epilepsy. One year after decoppering therapy, improvement of extrapyramidal signs was correlated with decrement of the abnormal signals in basal ganglia and thalamus, while atrophic change was apparent. However, the lesions in subcortical white matter indicated the presence of encephalomalacic change. These findings suggest that the prognosis of subcortical white-matter changes can differ from that of basal ganglia changes.