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

delete2026-04-01
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PRE
AI
A
Ana Rosa Santana
I
Igor Vilela Brum *
P
Prates, Izaely Ramos
J
Júlio Melão Cordeiro
R
Rubens Gisbert Cury
L
Leandro Tavares Lucato
B
Barbosa, Egberto Reis
P
Parmera, Jacy Bezerra
DOI:10.1055/s-0046-1817044delete
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Abstract

Abstract

En 中文
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.
Keywords:
Hepatolenticular Degeneration
Chorea
Magnetic Resonance Imaging

Journal

A
Arquivos de Neuro-Psiquiatria
IF:
1.6
Papers:
222
Citations:
3.7K

Organization

U
universidade de sao paulo
Scholars:
10.4W
Papers: 6.6W
Citations: 93
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