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BIBTEX RIS

Neurological manifestations in Wilson's disease: Report of 119 cases

2006; Wiley; Volume: 21; Issue: 12 Linguagem: Inglês

10.1002/mds.21170

1531-8257

Alexandre Alves Machado, Hsin Fen Chien, Marta Mitiko Deguti, Eduardo Luiz Rachid Cançado, Raymundo Soares Azevedo, Milberto Scaff, Egberto Reis Barbosa,

Neurological diseases and metabolism

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%). © 2006 Movement Disorder Society