Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

Adam P Ostendorf,1 Yu-Tze Ng2 1Department of Pediatrics, Neurology Section, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 2Department of Pediatrics, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX, USA Abstract: Len...

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Autores principales:	Ostendorf AP, Ng YT
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2017
Materias:	Lennox-Gastaut syndrome epilepsy epilepsy surgery cannabidiol epileptic encephalopathy Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/602d57df195d46dc9b7dc3c75d0b12ec
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spelling	oai:doaj.org-article:602d57df195d46dc9b7dc3c75d0b12ec2021-12-02T07:44:20ZTreatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions1178-2021https://doaj.org/article/602d57df195d46dc9b7dc3c75d0b12ec2017-04-01T00:00:00Zhttps://www.dovepress.com/treatment-resistant-lennox-gastaut-syndrome-therapeutic-trends-challen-peer-reviewed-article-NDThttps://doaj.org/toc/1178-2021Adam P Ostendorf,1 Yu-Tze Ng2 1Department of Pediatrics, Neurology Section, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 2Department of Pediatrics, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX, USA Abstract: Lennox-Gastaut syndrome is a severe, childhood-onset electroclinical syndrome comprised of multiple seizure types, intellectual and behavioral disturbances and characteristic findings on electroencephalogram of slow spike and wave complexes and paroxysmal fast frequency activity. Profound morbidity often accompanies a common and severe seizure type, the drop attack. Seizures often remain refractory, or initial treatment efficacy fades. Few individuals are seizure free despite the development of multiple generations of antiseizure medications over decades and high-level evidence on several choices. Approved medications such as lamotrigine, topiramate, rufinamide, felbamate and clobazam have demonstrated efficacy in reducing seizure burden. Cannabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation. Palliative surgical procedures, such as vagal nerve stimulation and corpus callosotomy may provide reduction in total seizures and drop attacks. Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects. Etiologies such as dysplasia or hypothalamic hamartoma may be amenable for focal resection and thus offer potential to reverse this devastating epileptic encephalopathy. Keywords: Lennox-Gastaut syndrome, epilepsy, epilepsy surgery, cannabidiol, epileptic encephalopathyOstendorf APNg YTDove Medical PressarticleLennox-Gastaut syndromeepilepsyepilepsy surgerycannabidiolepileptic encephalopathyNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeuropsychiatric Disease and Treatment, Vol Volume 13, Pp 1131-1140 (2017)
institution	DOAJ
collection	DOAJ
language	EN
topic	Lennox-Gastaut syndrome epilepsy epilepsy surgery cannabidiol epileptic encephalopathy Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
spellingShingle	Lennox-Gastaut syndrome epilepsy epilepsy surgery cannabidiol epileptic encephalopathy Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429 Ostendorf AP Ng YT Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
description	Adam P Ostendorf,1 Yu-Tze Ng2 1Department of Pediatrics, Neurology Section, Nationwide Children’s Hospital, The Ohio State University, Columbus, OH, 2Department of Pediatrics, Baylor College of Medicine, The Children’s Hospital of San Antonio, San Antonio, TX, USA Abstract: Lennox-Gastaut syndrome is a severe, childhood-onset electroclinical syndrome comprised of multiple seizure types, intellectual and behavioral disturbances and characteristic findings on electroencephalogram of slow spike and wave complexes and paroxysmal fast frequency activity. Profound morbidity often accompanies a common and severe seizure type, the drop attack. Seizures often remain refractory, or initial treatment efficacy fades. Few individuals are seizure free despite the development of multiple generations of antiseizure medications over decades and high-level evidence on several choices. Approved medications such as lamotrigine, topiramate, rufinamide, felbamate and clobazam have demonstrated efficacy in reducing seizure burden. Cannabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation. Palliative surgical procedures, such as vagal nerve stimulation and corpus callosotomy may provide reduction in total seizures and drop attacks. Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects. Etiologies such as dysplasia or hypothalamic hamartoma may be amenable for focal resection and thus offer potential to reverse this devastating epileptic encephalopathy. Keywords: Lennox-Gastaut syndrome, epilepsy, epilepsy surgery, cannabidiol, epileptic encephalopathy
format	article
author	Ostendorf AP Ng YT
author_facet	Ostendorf AP Ng YT
author_sort	Ostendorf AP
title	Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
title_short	Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
title_full	Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
title_fullStr	Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
title_full_unstemmed	Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions
title_sort	treatment-resistant lennox-gastaut syndrome: therapeutic trends, challenges and future directions
publisher	Dove Medical Press
publishDate	2017
url	https://doaj.org/article/602d57df195d46dc9b7dc3c75d0b12ec
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Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

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