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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...
Autores principales: | , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Thieme Revinter Publicações Ltda.
2023
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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. |
format | Online Article Text |
id | pubmed-10550353 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Thieme Revinter Publicações Ltda. |
record_format | MEDLINE/PubMed |
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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