title: Wilson's disease with myoclonus and white matter lesions.
abstract: White matter lesions (WML) and epilepsy have been occasionally seen in Wilson's disease. No cases of generalized myoclonus have been reported so far. We present a patient with psychiatric symptoms starting at age 16, followed by tremor, generalized dystonia and severe generalized myoclonus. In addition to classical findings, the MRI showed also extensive WML in temporal, parietal and frontal regions, preserving interhemispheric fibers. Necropsy revealed marked alterations of white matter, cortex and basal ganglia. We subsequently review the literature concerning WML and myoclonus in Wilson's disease.
authors.affiliation: Department of Neurology, São Paulo University Medical School, R. Sergipe, 401-conj.311 R. 01234-001 Sao Paulo, SP, Brazil.
authors.forename: Egberto Reis
authors.initials: ER
authors.lastname: Barbosa
authors.forename: Laura
authors.initials: L
authors.lastname: Silveira-Moriyama
authors.forename: Alexandre Costa
authors.initials: AC
authors.lastname: Machado
authors.forename: Luiz Alberto
authors.initials: LA
authors.lastname: Bacheschi
authors.forename: Sérgio
authors.initials: S
authors.lastname: Rosemberg
authors.forename: Milberto
authors.initials: M
authors.lastname: Scaff
mesh_terms: D000293:Adolescent; D002540:Cerebral Cortex / Q000473:pathology*; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D009207:Myoclonus / Q000150:complications* / Q000473:pathology*; D012196:Review Literature as Topic