Cerebral white matter involvement in Wilson's disease

Although Wilson's disease (WD) involves primarily gray matter with the main target on the basal ganglia, white matter degeneration has been reported. From a review of the literature and our studies, cerebral white matter involvement occurred in 6-20% of patients with WD and mainly affected the frontal lobes. The majority of the patients were adolescents or young adults who usually had had the disease for several years. Patients were often not treated, poorly treated, or induced by d-penicillamine treatment. Clinical features were characterized by contralateral weakness, tremor and dystonia. Psychiatric symptoms, particularly schizophrenia-like psychosis, were common and might be the initial presentation. Epileptic seizures were also common and patients responded to antiepileptic and decoppering treatments. The long-term prognosis appeared to be favorable when the patients had regular and proper treatment.