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Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants

Background  Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms...

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Autores principales: Sampaio, Letícia Pereira de Brito, Henriques-Souza, Adélia Maria de Miranda, Silveira, Mariana Ribeiro Marcondes da, Seguti, Lisiane, Santos, Mara Lúcia Schmitz Ferreira, Montenegro, Maria Augusta, Antoniuk, Sérgio, Manreza, Maria Luíza Giraldes de
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Thieme Revinter Publicações Ltda. 2023
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10550353/
https://www.ncbi.nlm.nih.gov/pubmed/37793406
http://dx.doi.org/10.1055/s-0043-1772835
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author Sampaio, Letícia Pereira de Brito
Henriques-Souza, Adélia Maria de Miranda
Silveira, Mariana Ribeiro Marcondes da
Seguti, Lisiane
Santos, Mara Lúcia Schmitz Ferreira
Montenegro, Maria Augusta
Antoniuk, Sérgio
Manreza, Maria Luíza Giraldes de
author_facet Sampaio, Letícia Pereira de Brito
Henriques-Souza, Adélia Maria de Miranda
Silveira, Mariana Ribeiro Marcondes da
Seguti, Lisiane
Santos, Mara Lúcia Schmitz Ferreira
Montenegro, Maria Augusta
Antoniuk, Sérgio
Manreza, Maria Luíza Giraldes de
author_sort Sampaio, Letícia Pereira de Brito
collection PubMed
description Background  Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent. Objective  To evaluate the literature regarding treatment of IESS and provide a practical guidance to a healthcare system with limited resources. Methods  An expert committee from the Brazilian Society of Child Neurology reviewed and discussed relevant scientific evidence in the treatment of IESS regarding the drugs available in Brazil. Results  Oral prednisolone and vigabatrin are the most common drugs used as first-line therapy; they are efficient and affordable therapy as both are available in the Brazilian unified health system (SUS, in the Portuguese acronym). Intramuscular adrenocorticotropic hormone (ACTH) presents similar efficacy as oral prednisolone but has a higher cost and is not available in Brazil. Other antiseizure medications such as topiramate, levetiracetam, or benzodiazepines have limited response and are prescribed as adjuvant therapy. If the health service has nutritionists, a ketogenic diet should be implemented for those not responding to hormonal and vigabatrin treatment. Epilepsy surgery is mainly indicated for patients with focal lesions that do not respond to pharmacological therapy. Conclusion  Early treatment of IESS with efficient drugs is feasible in our country. Using standard protocols increases the odds of achieving complete cessation in a shorter time and decreases relapse.
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spelling pubmed-105503532023-10-05 Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants Sampaio, Letícia Pereira de Brito Henriques-Souza, Adélia Maria de Miranda Silveira, Mariana Ribeiro Marcondes da Seguti, Lisiane Santos, Mara Lúcia Schmitz Ferreira Montenegro, Maria Augusta Antoniuk, Sérgio Manreza, Maria Luíza Giraldes de Arq Neuropsiquiatr Background  Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent. Objective  To evaluate the literature regarding treatment of IESS and provide a practical guidance to a healthcare system with limited resources. Methods  An expert committee from the Brazilian Society of Child Neurology reviewed and discussed relevant scientific evidence in the treatment of IESS regarding the drugs available in Brazil. Results  Oral prednisolone and vigabatrin are the most common drugs used as first-line therapy; they are efficient and affordable therapy as both are available in the Brazilian unified health system (SUS, in the Portuguese acronym). Intramuscular adrenocorticotropic hormone (ACTH) presents similar efficacy as oral prednisolone but has a higher cost and is not available in Brazil. Other antiseizure medications such as topiramate, levetiracetam, or benzodiazepines have limited response and are prescribed as adjuvant therapy. If the health service has nutritionists, a ketogenic diet should be implemented for those not responding to hormonal and vigabatrin treatment. Epilepsy surgery is mainly indicated for patients with focal lesions that do not respond to pharmacological therapy. Conclusion  Early treatment of IESS with efficient drugs is feasible in our country. Using standard protocols increases the odds of achieving complete cessation in a shorter time and decreases relapse. Thieme Revinter Publicações Ltda. 2023-10-04 /pmc/articles/PMC10550353/ /pubmed/37793406 http://dx.doi.org/10.1055/s-0043-1772835 Text en Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit ( https://creativecommons.org/licenses/by/4.0/ ) https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Sampaio, Letícia Pereira de Brito
Henriques-Souza, Adélia Maria de Miranda
Silveira, Mariana Ribeiro Marcondes da
Seguti, Lisiane
Santos, Mara Lúcia Schmitz Ferreira
Montenegro, Maria Augusta
Antoniuk, Sérgio
Manreza, Maria Luíza Giraldes de
Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title_full Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title_fullStr Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title_full_unstemmed Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title_short Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
title_sort brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10550353/
https://www.ncbi.nlm.nih.gov/pubmed/37793406
http://dx.doi.org/10.1055/s-0043-1772835
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