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Cognitive impairment in Wilson's disease

Comprometimento cognitivos na doença de Wilson

Abstract

Wilson's disease (WD) or hepatolenticular degeneration is a rare, genetic and systemic disease, caused by a deficit in the metabolism of copper, leading to its accumulation in different organs, mainly the liver, followed by the central nervous system, especially the basal ganglia. When symptoms begin between the second and third decades of life, approximately 50% of the patients show neurological symptoms. Although dystonia and dysarthria are the most common neurological signs, cognitive changes have been reported since the first cases were described in 1912. Memory change is one of the most common impairments, but other cognitive changes have been reported, including dementia in untreated cases. In this article we review the cognitive changes in WD patients and the occurrence of dementia.

Key words:
Wilson's disease; cognition; dementia.

Resumo

Doença de Wilson (DW) ou degeneração hepatolenticular é uma doença sistêmica genética rara, causada por um déficit no metabolismo do cobre, levando a sua acumulação in diferentes órgãos, principalmente o fígado, seguido pelo sistema nervoso central, especialmente os gânglios da base. Quando os sintomas iniciam entre a segunda e terceira décadas de vida, aproximadamente 50% dos pacientes apresentam sintomas neurológicos. Apesar de distonia e disartria serem as alterações neurológicas mais comuns, mudanças cognitivas tem sido relatadas desde os primeiros casos em 1912. Alteração de memória é o comprometimento mais comum, mas outras alterações foram descritas, incluindo demência em casos não tratados. Nesse artigo nós revisaremos as alterações cognitivas em pacientes com DW e a ocorrência de demência.

Palavras-chave:
Doença de Wilson; cognição e demência.

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Publication Dates

  • Publication in this collection
    Jan-Mar 2009

History

  • Received
    19 Nov 2008
  • Accepted
    20 Feb 2009
Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
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