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Current role of surgery for tuberous sclerosis complex‐associated epilepsy

Tuberous sclerosis complex (TSC) is a rare multisystem, autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations. Around two thirds of patients develop drug‐resistant epilepsy for whom surgical resection of epileptogenic f...

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Autores principales: Specchio, Nicola, Pavia, Giusy Carfi, de Palma, Luca, De Benedictis, Alessandro, Pepi, Chiara, Conti, Marta, Marras, Carlo Efisio, Vigevano, Federico, Curatolo, Paolo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8960933/
https://www.ncbi.nlm.nih.gov/pubmed/35382422
http://dx.doi.org/10.1002/ped4.12312
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author Specchio, Nicola
Pavia, Giusy Carfi
de Palma, Luca
De Benedictis, Alessandro
Pepi, Chiara
Conti, Marta
Marras, Carlo Efisio
Vigevano, Federico
Curatolo, Paolo
author_facet Specchio, Nicola
Pavia, Giusy Carfi
de Palma, Luca
De Benedictis, Alessandro
Pepi, Chiara
Conti, Marta
Marras, Carlo Efisio
Vigevano, Federico
Curatolo, Paolo
author_sort Specchio, Nicola
collection PubMed
description Tuberous sclerosis complex (TSC) is a rare multisystem, autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations. Around two thirds of patients develop drug‐resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure medications. The challenge with presurgical and surgical approaches with patients with TSC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic zone. Data suggest that seizure freedom is achieved by 55%–60% of patients, but predictive factors for success have remained elusive, which makes for unconfident selection of surgical candidates. This article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TSC patients for epilepsy surgery.
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spelling pubmed-89609332022-04-04 Current role of surgery for tuberous sclerosis complex‐associated epilepsy Specchio, Nicola Pavia, Giusy Carfi de Palma, Luca De Benedictis, Alessandro Pepi, Chiara Conti, Marta Marras, Carlo Efisio Vigevano, Federico Curatolo, Paolo Pediatr Investig Clinical Experience Tuberous sclerosis complex (TSC) is a rare multisystem, autosomal dominant neurocutaneous syndrome in which epilepsy is the most common of several neurological and psychiatric manifestations. Around two thirds of patients develop drug‐resistant epilepsy for whom surgical resection of epileptogenic foci is indicated when seizures remain inadequately controlled following trial of two antiseizure medications. The challenge with presurgical and surgical approaches with patients with TSC is overcoming the complexity from the number of tubers and the multiplex epileptogenic network forming the epileptogenic zone. Data suggest that seizure freedom is achieved by 55%–60% of patients, but predictive factors for success have remained elusive, which makes for unconfident selection of surgical candidates. This article presents three different cases as illustrations of the potential challenges faced when assessing the suitability of TSC patients for epilepsy surgery. John Wiley and Sons Inc. 2022-02-21 /pmc/articles/PMC8960933/ /pubmed/35382422 http://dx.doi.org/10.1002/ped4.12312 Text en © 2022 Chinese Medical Association. Pediatric Investigation published by John Wiley & Sons Australia, Ltd on behalf of Futang Research Center of Pediatric Development. 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 Clinical Experience
Specchio, Nicola
Pavia, Giusy Carfi
de Palma, Luca
De Benedictis, Alessandro
Pepi, Chiara
Conti, Marta
Marras, Carlo Efisio
Vigevano, Federico
Curatolo, Paolo
Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title_full Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title_fullStr Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title_full_unstemmed Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title_short Current role of surgery for tuberous sclerosis complex‐associated epilepsy
title_sort current role of surgery for tuberous sclerosis complex‐associated epilepsy
topic Clinical Experience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8960933/
https://www.ncbi.nlm.nih.gov/pubmed/35382422
http://dx.doi.org/10.1002/ped4.12312
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