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

      Movement Disorders

      Adolescent, Adult, Age of Onset, Child, Female, Hepatolenticular Degeneration, diagnosis, physiopathology, Humans, Medical Records, statistics & numerical data, Nervous System Diseases, Neurologic Examination, Retrospective Studies

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          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%). Copyright 2006 Movement Disorder Society.

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          Journal
          17078070
          10.1002/mds.21170

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