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Cognitive impairment in Wilson's disease
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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Associação de Neurologia Cognitiva e do
Comportamento
2009
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619026/ https://www.ncbi.nlm.nih.gov/pubmed/29213604 http://dx.doi.org/10.1590/S1980-57642009DN30100004 |
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author | Frota, Norberto Anizio Ferreira Caramelli, Paulo Barbosa, Egberto Reis |
author_facet | Frota, Norberto Anizio Ferreira Caramelli, Paulo Barbosa, Egberto Reis |
author_sort | Frota, Norberto Anizio Ferreira |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5619026 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2009 |
publisher | Associação de Neurologia Cognitiva e do
Comportamento |
record_format | MEDLINE/PubMed |
spelling | pubmed-56190262017-12-06 Cognitive impairment in Wilson's disease Frota, Norberto Anizio Ferreira Caramelli, Paulo Barbosa, Egberto Reis Dement Neuropsychol Views & Reviews 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. Associação de Neurologia Cognitiva e do Comportamento 2009 /pmc/articles/PMC5619026/ /pubmed/29213604 http://dx.doi.org/10.1590/S1980-57642009DN30100004 Text en http://creativecommons.org/licenses/by/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Views & Reviews Frota, Norberto Anizio Ferreira Caramelli, Paulo Barbosa, Egberto Reis Cognitive impairment in Wilson's disease |
title | Cognitive impairment in Wilson's disease |
title_full | Cognitive impairment in Wilson's disease |
title_fullStr | Cognitive impairment in Wilson's disease |
title_full_unstemmed | Cognitive impairment in Wilson's disease |
title_short | Cognitive impairment in Wilson's disease |
title_sort | cognitive impairment in wilson's disease |
topic | Views & Reviews |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5619026/ https://www.ncbi.nlm.nih.gov/pubmed/29213604 http://dx.doi.org/10.1590/S1980-57642009DN30100004 |
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