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Abstract(s)
As demências rapidamente progressivas definem-se como um grupo de patologias que têm em comum o quadro clínico caracterizado pelo desenvolvimento de declínio cognitivo, comportamental e/ou motor, que evolui de forma muito rápida, habitualmente num período de semanas a meses.
O diagnóstico diferencial de um quadro clínico com estas características deve incluir causas degenerativas, inflamatórias, neoplásicas e metabólicas. Geralmente, o tratamento destes quadros depende unicamente do correcto diagnóstico efectuado. Assim, o desafio na abordagem das demências rapidamente progressivas é a distinção entre as causas reversíveis e irreversíveis da demência, no sentido de poder actuar terapeuticamente de forma assertiva e rápida.
A presente dissertação pretende efectuar uma revisão, através de pesquisa bibliográfica, das principais patologias que compõem o diagnóstico diferencial das demências rapidamente progressivas, focando os principais aspectos distintivos entre elas, e os principais passos que compõem a investigação clínica destes casos.
Métodos: pesquisa bibliográfica com recurso a bases de dados on-line (PubMed; B-on; Sciencedirect) assim como livros de referência na área, com preferência por artigos publicados entre os anos de 2000-2014 e nas línguas Inglesa e Portuguesa.
Rapidly progressive dementias can be defined as a wide group of conditions who share the same clinical hallmarks, including the development of rapid cognitive decline, behavioral and/or motor disturbances, usually over a period of weeks to months. The differential diagnosis of these conditions should include degenerative, inflammatory, neoplastic as well as metabolic causes. Typically, the correct treatment for this condition relies exclusively in the correct diagnosis of the underlying disease. Hence, the greatest challenge regarding the approach to these patients is to discern between reversible and irreversible causes of rapidly progressive dementia, in such a way that the correct treatment is administered as soon and as accurately as possible. The current dissertation will try to approach the main causes of rapidly progressive dementia, mainly through the review of literature, focusing on the distinctive features of these diseases and the main diagnostic steps required to manage them. Methods: extensive bibliographical research using electronic databases such as PubMed, B-on, Sciencedirect, as well as some reference books from the scientific area. Preference was given to articles published between the years of 2000-2014, written in English or Portuguese
Rapidly progressive dementias can be defined as a wide group of conditions who share the same clinical hallmarks, including the development of rapid cognitive decline, behavioral and/or motor disturbances, usually over a period of weeks to months. The differential diagnosis of these conditions should include degenerative, inflammatory, neoplastic as well as metabolic causes. Typically, the correct treatment for this condition relies exclusively in the correct diagnosis of the underlying disease. Hence, the greatest challenge regarding the approach to these patients is to discern between reversible and irreversible causes of rapidly progressive dementia, in such a way that the correct treatment is administered as soon and as accurately as possible. The current dissertation will try to approach the main causes of rapidly progressive dementia, mainly through the review of literature, focusing on the distinctive features of these diseases and the main diagnostic steps required to manage them. Methods: extensive bibliographical research using electronic databases such as PubMed, B-on, Sciencedirect, as well as some reference books from the scientific area. Preference was given to articles published between the years of 2000-2014, written in English or Portuguese
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Keywords
Demência Rapidamente Progressiva Demências Imunologicamente Mediadas Diagnóstico Diferencial Doença de Creutzfeldt-Jakob Encefalopatia de Hashimoto