Choreiform Wilson's disease and a distinctive 7-Tesla magnetic resonance imaging finding

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.

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Journal
Arquivos de Neuro-Psiquiatria
Published
2026-03-23
DOI
https://doi.org/10.1055/s-0046-1817044
Primary Topic
Neurological and metabolic disorders
Type
article
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article

Choreiform Wilson's disease and a distinctive 7-Tesla magnetic resonance imaging finding

Egberto Reis Barbosa, Júlio Melão Cordeiro, Jacy Bezerra Parmera, Izaely Ramos Prates et al.
Arquivos de Neuro-Psiquiatria
Neurological and metabolic disorders
article

Choreiform Wilson's disease and a distinctive 7-Tesla magnetic resonance imaging finding

Egberto Reis Barbosa, Júlio Melão Cordeiro, Jacy Bezerra Parmera, Izaely Ramos Prates, Igor Vilela Brum, Leandro Tavares Lucato, Ana Rosa Santana, Rubens Gisbert Cury
article en

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.

Arquivos de Neuro-PsiquiatriaVol. 84(04)
Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (BR)
Good health and well-being
Openalex Percentile: Top 14%
Neurological and metabolic disorders
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Choreiform Wilson's disease and a distinctive 7-Tesla magnetic resonance imaging finding — Egberto Reis Barbosa, Júlio Melão Cordeiro, et al. · Arquivos de Neuro-Psiquiatria (2026) | TGRS Research Map | TGRS