Abstract Chorea is a rare manifestation of Wilson's disease (WD), resulting from copper-induced dysfunction of the basal ganglia. We herein report the case of a 22-year-old woman with progressive dysarthria and generalized chorea, later confirmed as WD. After 1 year of penicillamine therapy, she showed marked clinical improvement, with only residual chorea in the right upper limb. A 7-Tesla (7-T) magnetic resonance imaging (MRI) scan revealed predominant involvement of the left basal ganglia and corticospinal tract, including the hyperintense globus pallidus rim sign. This case highlights chorea as a potential initial manifestation of WD and reinforces its reversibility with the appropriate treatment.
Santana et al. (Mon,) studied this question.