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Introdução: A Esclerose Lateral Amiotrófica é uma doença neurodegenerativa caraterizada pela degeneração simultânea de neurónio motor superior e neurónio motor inferior. Tem uma incidência estimada de 2,16/100.000 pessoas-ano. Os sintomas de apresentação mais comuns incluem fraqueza, fadiga e atrofia muscular, com início focal. Ocorre uma evolução progressiva e generalizada, culminando na morte por insuficiência muscular respiratória. Fatores ambientais, genéticos e do acaso parecem interagir. A nível celular, diferentes anormalidades contribuem para a lesão nervosa, nomeadamente, excitotoxicidade e agregação proteica. Os fármacos disponíveis, atualmente, não modificam a doença. Oferecem um aumento pouco significativo da sobrevida e diminuem a progressão funcional. A terapêutica fundamental da Esclerose Lateral Amiotrófica é, então, direcionada aos sintomas, como parte integrante de uma abordagem multidisciplinar que coloca o doente no centro.
Objetivos: Com este trabalho pretende-se dar a conhecer de forma breve e simplificada, os mecanismos de doença responsáveis pela Esclerose Lateral Amiotrófica e as manifestações clínicas mais comuns, assim como, apresentar uma estratégia terapêutica completa e atualizada que reúna, paralelamente, as abordagens neuroprotetora e sintomática.
Metodologia: Para a realização desta análise foi realizada uma pesquisa bibliográfica de artigos científicos e de revisão, na base de dados eletrónica PubMed, até 31 de novembro de 2018. Limitou-se a pesquisa aos idiomas inglês e português e foram privilegiados para análise artigos mais recentes, provenientes de revistas com maior fator de impacto, revistos por pares e, ainda, guidelines clínicas. Em adição, foram consultados livros da especialidade e referências bibliográficas dos artigos previamente selecionados.
Conclusão: Após anos de investigação, o tratamento da Esclerose Lateral Amiotrófica mantém-se, sobretudo, sintomático. São múltiplas as formas de tratar o mesmo sintoma, pelo que o futuro passa pelo desenvolvimento de guidelines universais suportadas por evidência científica. Paralelamente, a eficácia de novos fármacos está a ser avaliada em ensaios clínicos de fase 3. Enquanto um tratamento curativo não for descoberto, uma estratégia combinada, centrada no paciente, é a melhor abordagem terapêutica disponível.
Introduction: Amyotrophic Lateral Sclerosis is a neurodegenerative disease characterized by simultaneous degeneration of upper motor neurons and lower motor neurons. It has an estimated incidence of 2,16/100.000 people-year. The most common presenting symptoms include weakness, fatigue, and muscular atrophy with a focal onset. Then, a progressive and generalized evolution occurs, culminating in death due to respiratory muscle insufficiency. Environmental, genetic, and random factors seem to interact. At the cellular level, different abnormalities contribute to nerve damage, namely excitotoxicity and protein aggregation. The drugs currently available do not modify the disease. They offer a small increase in survival and decrease functional progression. As result, fundamental Amyotrophic Lateral Sclerosis therapy is symptom-driven, as part of a multidisciplinary patient-centered approach. Objectives: This monograph aims to provide a brief and simplified knowledge of Amyotrophic Lateral Sclerosis’s mechanisms and its most common clinical manifestations, as well as to present a complete and updated therapeutic strategy that brings together the neuroprotective and symptomatic approaches. Methods: A bibliographic research of scientific and review articles was carried out in the electronic database PubMed, until November 31, 2018. The research was limited to English and Portuguese languages and were privileged to analyze more recent articles, those from journals with greater impact factor, peer reviewed, and clinical guidelines. In addition, specialty books and bibliographic references of previously selected articles were consulted. Conclusions: After years of research, the treatment of Amyotrophic Lateral Sclerosis remains mainly symptomatic. There are plenty of ways to treat the same symptom. The future should be about the development of universal clinical guidelines supported by scientific evidence. In addition, the efficacy of new drugs is being evaluated in phase 3 clinical trials. While curative treatment is not discovered, a combined, patient-centered strategy is the best therapeutic approach available.
Introduction: Amyotrophic Lateral Sclerosis is a neurodegenerative disease characterized by simultaneous degeneration of upper motor neurons and lower motor neurons. It has an estimated incidence of 2,16/100.000 people-year. The most common presenting symptoms include weakness, fatigue, and muscular atrophy with a focal onset. Then, a progressive and generalized evolution occurs, culminating in death due to respiratory muscle insufficiency. Environmental, genetic, and random factors seem to interact. At the cellular level, different abnormalities contribute to nerve damage, namely excitotoxicity and protein aggregation. The drugs currently available do not modify the disease. They offer a small increase in survival and decrease functional progression. As result, fundamental Amyotrophic Lateral Sclerosis therapy is symptom-driven, as part of a multidisciplinary patient-centered approach. Objectives: This monograph aims to provide a brief and simplified knowledge of Amyotrophic Lateral Sclerosis’s mechanisms and its most common clinical manifestations, as well as to present a complete and updated therapeutic strategy that brings together the neuroprotective and symptomatic approaches. Methods: A bibliographic research of scientific and review articles was carried out in the electronic database PubMed, until November 31, 2018. The research was limited to English and Portuguese languages and were privileged to analyze more recent articles, those from journals with greater impact factor, peer reviewed, and clinical guidelines. In addition, specialty books and bibliographic references of previously selected articles were consulted. Conclusions: After years of research, the treatment of Amyotrophic Lateral Sclerosis remains mainly symptomatic. There are plenty of ways to treat the same symptom. The future should be about the development of universal clinical guidelines supported by scientific evidence. In addition, the efficacy of new drugs is being evaluated in phase 3 clinical trials. While curative treatment is not discovered, a combined, patient-centered strategy is the best therapeutic approach available.
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