title: Neurological manifestations in Wilson's disease: Report of 119 cases.
abstract: We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%).
authors.affiliation: Department of Neurology, University of São Paulo School of Medicine, São Paulo, Brazil. alexandremachado@hotmail.com
authors.forename: Alexandre
authors.initials: A
authors.lastname: Machado
authors.forename: Hsin Fen
authors.initials: HF
authors.lastname: Chien
authors.forename: Marta Mitiko
authors.initials: MM
authors.lastname: Deguti
authors.forename: Eduardo
authors.initials: E
authors.lastname: Cançado
authors.forename: Raymundo Soares
authors.initials: RS
authors.lastname: Azevedo
authors.forename: Milberto
authors.initials: M
authors.lastname: Scaff
authors.forename: Egberto Reis
authors.initials: ER
authors.lastname: Barbosa
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008499:Medical Records / Q000706:statistics & numerical data; D009422:Nervous System Diseases / Q000503:physiopathology*; D009460:Neurologic Examination; D012189:Retrospective Studies