Which factors are associated with global cognitive impairment in Wilson's disease?

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author FROTA, Norberto Anízio Ferreira
BARBOSA, Egberto Reis FMUSP-HC
PORTO, Claudia Sellitto
LUCATO, Leandro Tavares FMUSP-HC
ONO, Carla Rachel FMUSP-HC
BUCHPIGUEL, Carlos Alberto FMUSP-HC
MACHADO, Alexandre Aluizio Costa FMUSP-HC
CARAMELLI, Paulo
dc.date.issued 2016
dc.identifier.citation DEMENTIA & NEUROPSYCHOLOGIA, v.10, n.4, p.320-326, 2016
dc.identifier.issn 1980-5764
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/18166
dc.description.abstract ABSTRACT Background: Patients with Wilson's disease (WD) present cognitive impairment, especially in executive functions. Which other factors might be associated with global cognitive decline in these patients remains unclear. Objective: To assess which factors are associated with worse performance on a global cognitive test in patients with WD. Methods: Twenty patients with WD underwent cognitive assessment with the following tests: the Mini-Mental State Examination (MMSE), Dementia Rating Scale (DRS), verbal fluency test, brief cognitive battery, clock drawing test, Frontal Assessment Battery, Stroop test, Wisconsin card sorting test, Hopper test, cubes (WAIS) and the Pfeffer questionnaire. MRI changes were quantified. Patients with poor performance on the DRS were compared to patients with normal performance. Results: Nine patients had a poor performance on the DRS. This group had lower educational level (9.11±3.58 × 12.82±3.06) and a greater number of changes on MRI (9.44±2.74 × 6.27±2.45). The presence of hyperintensity in the globus pallidus on MRI was more frequent in this group (66.6% vs 9.0%), with OR=5.38 (95% CI 0.85-33.86). Conclusion: Global cognitive impairment was prevalent in this sample of patients with WD and was associated with low educational level, number of changes on MRI and MRI hyperintensity in the globus pallidus.
dc.description.abstract RESUMO Embasamento: Pacientes com doença de Wilson (DW) apresentam comprometimento cognitivo, principalmente de funções executivas. Existem dúvidas sobre quais outros fatores poderiam estar associados ao declínio cognitivo global nesses pacientes. Objetivo: Avaliar quais fatores estão associados ao pior desempenho em teste cognitivo global em pacientes com DW. Métodos: Vinte pacientes com DW em tratamento regular foram submetidos à avaliação cognitiva com os seguintes testes: Mini-Exame do Estado Mental, escala de demência de Mattis (DRS), fluência verbal, bateria cognitiva breve, desenho do relógio, bacteria de avaliação frontal, Stroop, teste de seleção de cartões de Wisconsin, Hopper, Cubos e ao questionário de Pfeffer. As alterações em RM foram quantificadas. Pacientes com desempenho alterado na DRS foram comparados aos pacientes com desempenho normal. Resultados: Nove pacientes apresentavam desempenho alterado na DRS. Eles apresentavam menor nivel educacional (9,11±3,58 × 12,82±3,06 anos, respectivamente) e maior quantidade de alterações na RM (9,44±2,74 × 6,27±2,45). A presença de hipersinal no globo pálido na RM foi mais frequente nesse grupo (66,6% × 9,0%), com OR=5,38 (IC 95% 0,85-33,86). Conclusão: Alterações cognitivas globais foram frequentes nesta amostra de pacientes com DW e se associaram à baixa escolaridade, quantidade de alterações em RM e a hipersinal no globo pálido à RM.
dc.language.iso eng
dc.publisher Associação de Neurologia Cognitiva e do Comportamento
dc.relation.ispartof Dementia & Neuropsychologia
dc.rights openAccess
dc.subject Wilson's disease; cognitive impairment; neuropsychological tests; magnetic resonance imaging; globus pallidus; doença de Wilson; deterioração cognitiva; testes neuropsicológicos; imagem de ressonância magnética
dc.title Which factors are associated with global cognitive impairment in Wilson's disease?
dc.title.alternative QUAIS FATORES ESTÃO ASSOCIADOS AO COMPROMETIMENTO COGNITIVO GLOBAL NA DOENÇA DE WILSON?
dc.type article
dc.rights.holder Copyright Associação de Neurologia Cognitiva e do Comportamento
dc.description.group LIM/43
dc.description.group LIM/44
dc.description.group LIM/45
dc.identifier.doi 10.1590/s1980-5764-2016dn1004011
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author BARBOSA, Egberto Reis:HC:ICHC
hcfmusp.author LUCATO, Leandro Tavares:HC:INRAD
hcfmusp.author ONO, Carla Rachel:HC:ICESP
hcfmusp.author BUCHPIGUEL, Carlos Alberto:FM:MDR
hcfmusp.author MACHADO, Alexandre Aluizio Costa:HC:ICHC
hcfmusp.author.external · FROTA, Norberto Anízio Ferreira:Universidade de Fortaleza, Brazil
· PORTO, Claudia Sellitto:Universidade de São Paulo, Brazil
· CARAMELLI, Paulo:Universidade Federal de Minas Gerais, Brazil
hcfmusp.origem.id 2-s2.0-85007553299
hcfmusp.origem.id SCIELO:S1980-57642016000400320
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dc.description.index PubMed
hcfmusp.citation.scopus 1
hcfmusp.affiliation.country Brasil


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