Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide

Detalhes bibliográficos
Autor(a) principal: Paulos, Jorge Pelicano
Data de Publicação: 2013
Outros Autores: Massano, João
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226
Resumo: Introduction: Frontotemporal Lobar Degeneration encompasses a group of heterogeneous disorders with shared behavioural andcognitive symptoms, as well as gross pathological features. The genetic underpinnings and histopathological aspects are quite diverse and form the basis of molecular classification, which is not easy to correlate with clinical findings and syndromes. Scientific research has brought to light an array of knowledge, often not easy to keep up with, especially in the last few years with regard to genetics and histopathology.Material and Methods: The authors have searched the published literature on this topic, chose relevant references, and extracted and systematized the data.Results and Conclusion: this manuscript presents an updated review of clinical, genetic and histopathological findings in Frontotemporal Lobar Degeneration, with special focus on behavioural variant Frontotemporal Dementia, the most common disorder. Current management is also reviewed, and genetic testing.
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spelling Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and GuideAspetos Clínicos, Genéticos e Neuropatológicos da Demência Frontotemporal: Atualização e GuiaIntroduction: Frontotemporal Lobar Degeneration encompasses a group of heterogeneous disorders with shared behavioural andcognitive symptoms, as well as gross pathological features. The genetic underpinnings and histopathological aspects are quite diverse and form the basis of molecular classification, which is not easy to correlate with clinical findings and syndromes. Scientific research has brought to light an array of knowledge, often not easy to keep up with, especially in the last few years with regard to genetics and histopathology.Material and Methods: The authors have searched the published literature on this topic, chose relevant references, and extracted and systematized the data.Results and Conclusion: this manuscript presents an updated review of clinical, genetic and histopathological findings in Frontotemporal Lobar Degeneration, with special focus on behavioural variant Frontotemporal Dementia, the most common disorder. Current management is also reviewed, and genetic testing.Introdução: A Degenerescência Lobar Frontotemporal engloba um conjunto de situações heterogéneas que partilham sintomas cognitivos e comportamentais, bem como características patológicas macroscópicas. As bases genéticas e características histopatológicas são bastante diversas e formam a base da classificação molecular das várias doenças, sendo difícil fazer uma correlação com os achados clínicos e síndromas. A investigação científica trouxe um conjunto vasto de conhecimentos, nem sempre fáceis de acompanhar,especialmente nos últimos anos em relação à genética e histopatologia.Material e Métodos: Os autores fizeram uma pesquisa de literatura neste tema, escolheram referências relevantes, extraíram e sistematizaram os dados.Resultados e Conclusão: o texto apresenta uma revisão atualizada dos aspetos clínicos, genéticos e histopatológicos da Degenerescência Lobar Frontotemporal, com ênfase especial na Demência Frontotemporal, a doença mais comum. O tratamento é também revisto e são propostas pelos autores estratégias relativamente à escolha dos testes genéticos na prática clínica. Deveriam serpromovidos a atenção e conhecimento públicos sobre este grupo de doenças.Ordem dos Médicos2013-08-30info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfimage/tiffimage/tiffhttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226oai:ojs.www.actamedicaportuguesa.com:article/1226Acta Médica Portuguesa; Vol. 26 No. 4 (2013): July-August; 392-401Acta Médica Portuguesa; Vol. 26 N.º 4 (2013): Julho-Agosto; 392-4011646-07580870-399Xreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttps://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226/3713https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226/6838https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226/6839Paulos, Jorge PelicanoMassano, Joãoinfo:eu-repo/semantics/openAccess2022-12-20T10:57:40Zoai:ojs.www.actamedicaportuguesa.com:article/1226Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:17:02.955584Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
Aspetos Clínicos, Genéticos e Neuropatológicos da Demência Frontotemporal: Atualização e Guia
title Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
spellingShingle Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
Paulos, Jorge Pelicano
title_short Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
title_full Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
title_fullStr Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
title_full_unstemmed Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
title_sort Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide
author Paulos, Jorge Pelicano
author_facet Paulos, Jorge Pelicano
Massano, João
author_role author
author2 Massano, João
author2_role author
dc.contributor.author.fl_str_mv Paulos, Jorge Pelicano
Massano, João
description Introduction: Frontotemporal Lobar Degeneration encompasses a group of heterogeneous disorders with shared behavioural andcognitive symptoms, as well as gross pathological features. The genetic underpinnings and histopathological aspects are quite diverse and form the basis of molecular classification, which is not easy to correlate with clinical findings and syndromes. Scientific research has brought to light an array of knowledge, often not easy to keep up with, especially in the last few years with regard to genetics and histopathology.Material and Methods: The authors have searched the published literature on this topic, chose relevant references, and extracted and systematized the data.Results and Conclusion: this manuscript presents an updated review of clinical, genetic and histopathological findings in Frontotemporal Lobar Degeneration, with special focus on behavioural variant Frontotemporal Dementia, the most common disorder. Current management is also reviewed, and genetic testing.
publishDate 2013
dc.date.none.fl_str_mv 2013-08-30
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https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/1226/6838
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dc.publisher.none.fl_str_mv Ordem dos Médicos
publisher.none.fl_str_mv Ordem dos Médicos
dc.source.none.fl_str_mv Acta Médica Portuguesa; Vol. 26 No. 4 (2013): July-August; 392-401
Acta Médica Portuguesa; Vol. 26 N.º 4 (2013): Julho-Agosto; 392-401
1646-0758
0870-399X
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