Premium
Neurological manifestations in Wilson's disease: Report of 119 cases
Author(s) -
Machado Alexandre,
Fen Chien Hsin,
Mitiko Deguti Marta,
Cançado Eduardo,
Soares Azevedo Raymundo,
Scaff Milberto,
Reis Barbosa Egberto
Publication year - 2006
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.21170
Subject(s) - athetosis , dysarthria , chorea , dystonia , dysphagia , pediatrics , gait disturbance , medicine , movement disorders , neurological disorder , neurological examination , disease , central nervous system disease , surgery , physical medicine and rehabilitation , psychiatry
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