Movement Disorders (revue)

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

Identifieur interne : 002E95 ( Istex/Curation ); précédent : 002E94; suivant : 002E96

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

Auteurs : Alexandre Machado [Brésil] ; Hsin Fen Chien [Brésil] ; Marta Mitiko Deguti [Brésil] ; Eduardo Cançado [Brésil] ; Raymundo Soares Azevedo [Brésil] ; Milberto Scaff [Brésil] ; Egberto Reis Barbosa [Brésil]

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RBID : ISTEX:D5510326E3372F83B9F844233921D6EA6F8F44CE

English descriptors

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

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DOI: 10.1002/mds.21170

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ISTEX:D5510326E3372F83B9F844233921D6EA6F8F44CE

Le document en format XML

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<div type="abstract" xml:lang="en">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</div>
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