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Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe

Dravet syndrome (DS) is a severe, rare, and complex developmental and epileptic encephalopathy affecting 1 in 16 000 live births and characterized by a drug‐resistant epilepsy, cognitive, psychomotor, and language impairment, and behavioral disorders. Evidence suggests that optimal treatment of seiz...

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Autores principales: Cardenal‐Muñoz, Elena, Auvin, Stéphane, Villanueva, Vicente, Cross, J. Helen, Zuberi, Sameer M., Lagae, Lieven, Aibar, José Ángel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8886070/
https://www.ncbi.nlm.nih.gov/pubmed/34882995
http://dx.doi.org/10.1002/epi4.12569
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author Cardenal‐Muñoz, Elena
Auvin, Stéphane
Villanueva, Vicente
Cross, J. Helen
Zuberi, Sameer M.
Lagae, Lieven
Aibar, José Ángel
author_facet Cardenal‐Muñoz, Elena
Auvin, Stéphane
Villanueva, Vicente
Cross, J. Helen
Zuberi, Sameer M.
Lagae, Lieven
Aibar, José Ángel
author_sort Cardenal‐Muñoz, Elena
collection PubMed
description Dravet syndrome (DS) is a severe, rare, and complex developmental and epileptic encephalopathy affecting 1 in 16 000 live births and characterized by a drug‐resistant epilepsy, cognitive, psychomotor, and language impairment, and behavioral disorders. Evidence suggests that optimal treatment of seizures in DS may improve outcomes, even though neurodevelopmental impairments are the likely result of both the underlying genetic variant and the epilepsy. We present an updated guideline for DS diagnosis and treatment, taking into consideration care of the adult patient and nonpharmaceutical therapeutic options for this disease. This up‐to‐date guideline, which is based on an extensive review of the literature and culminates with a new treatment algorithm for DS, is a European consensus developed through a survey involving 29 European clinical experts in DS. This guideline will serve professionals in their clinical practice and, as a consequence, will benefit DS patients and their families.
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spelling pubmed-88860702022-03-04 Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe Cardenal‐Muñoz, Elena Auvin, Stéphane Villanueva, Vicente Cross, J. Helen Zuberi, Sameer M. Lagae, Lieven Aibar, José Ángel Epilepsia Open Critical Review Dravet syndrome (DS) is a severe, rare, and complex developmental and epileptic encephalopathy affecting 1 in 16 000 live births and characterized by a drug‐resistant epilepsy, cognitive, psychomotor, and language impairment, and behavioral disorders. Evidence suggests that optimal treatment of seizures in DS may improve outcomes, even though neurodevelopmental impairments are the likely result of both the underlying genetic variant and the epilepsy. We present an updated guideline for DS diagnosis and treatment, taking into consideration care of the adult patient and nonpharmaceutical therapeutic options for this disease. This up‐to‐date guideline, which is based on an extensive review of the literature and culminates with a new treatment algorithm for DS, is a European consensus developed through a survey involving 29 European clinical experts in DS. This guideline will serve professionals in their clinical practice and, as a consequence, will benefit DS patients and their families. John Wiley and Sons Inc. 2021-12-19 /pmc/articles/PMC8886070/ /pubmed/34882995 http://dx.doi.org/10.1002/epi4.12569 Text en © 2021 The Authors. Epilepsia Open published by Wiley Periodicals LLC on behalf of International League Against Epilepsy. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Critical Review
Cardenal‐Muñoz, Elena
Auvin, Stéphane
Villanueva, Vicente
Cross, J. Helen
Zuberi, Sameer M.
Lagae, Lieven
Aibar, José Ángel
Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title_full Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title_fullStr Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title_full_unstemmed Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title_short Guidance on Dravet syndrome from infant to adult care: Road map for treatment planning in Europe
title_sort guidance on dravet syndrome from infant to adult care: road map for treatment planning in europe
topic Critical Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8886070/
https://www.ncbi.nlm.nih.gov/pubmed/34882995
http://dx.doi.org/10.1002/epi4.12569
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