Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options

Shelly Wang,1 Aria Fallah2,3 1Department of Neurosurgery, University of Toronto, Toronto, ON, Canada; 2Department of Neurosurgery, Miami Children’s Hospital, Miami, FL, USA; 3Department of Clinical Epidemiology and Biostatistics, McMaster University, Hamilton, ON, Canada Abstract: Seizu...

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Autores principales:	Wang S, Fallah A
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2014
Materias:	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
Acceso en línea:	https://doaj.org/article/3d5d0ad08b364b47a0e8b657d546d7c9
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spelling	oai:doaj.org-article:3d5d0ad08b364b47a0e8b657d546d7c92021-12-02T03:20:20ZOptimal management of seizures associated with tuberous sclerosis complex: current and emerging options1178-2021https://doaj.org/article/3d5d0ad08b364b47a0e8b657d546d7c92014-10-01T00:00:00Zhttp://www.dovepress.com/optimal-management-of-seizures-associated-with-tuberous-sclerosis-comp-peer-reviewed-article-NDThttps://doaj.org/toc/1178-2021 Shelly Wang,1 Aria Fallah2,3 1Department of Neurosurgery, University of Toronto, Toronto, ON, Canada; 2Department of Neurosurgery, Miami Children’s Hospital, Miami, FL, USA; 3Department of Clinical Epidemiology and Biostatistics, McMaster University, Hamilton, ON, Canada Abstract: Seizures are clinically significant manifestations associated with 79%–90% of patients with tuberous sclerosis complex. Often occurring within the first year of life in the form of infantile spasms, seizures interfere with neuropsychiatric, social, and cognitive development and carry significant individual and societal consequences. Prompt identification and treatment of seizures is an important focus in the overall management of tuberous sclerosis complex patients. Medical management, either after seizure onset or prophylactically in infants with electroencephalographic abnormalities, is considered first-line therapy. Vigabatrin and adrenocorticotropic hormone have emerged over the past few decades as mainstay pharmacologic modalities. Furthermore, emerging research on mammalian target of rapamycin inhibitors demonstrated promise for the management of seizures and subependymal giant cell astrocytoma. For appropriate surgical candidates with an epileptogenic zone associated with one or more glioneuronal hamartomas, ideally in noneloquent cortex, resective surgery can be considered, which provides a cure in 56% of patients. For medically refractory patients who do not meet criteria for curative surgery, palliative surgical approaches focused on reducing seizure burden, in the form of corpus callosotomy and vagus nerve stimulation, are alternative management options. Lastly, the ketogenic diet, a reemerging therapy based on the anticonvulsant effects of ketone bodies, can be utilized independently or in conjunction with other treatment modalities for the management of difficult-to-treat seizures. Keywords: epilepsy, adrenocorticotropic hormone, vigabatrin, mammalian target of rapamycin, ketogenic diet, vagus nerve stimulationWang SFallah ADove Medical PressarticleNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571Neurology. Diseases of the nervous systemRC346-429ENNeuropsychiatric Disease and Treatment, Vol 2014, Iss default, Pp 2021-2030 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429
spellingShingle	Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Neurology. Diseases of the nervous system RC346-429 Wang S Fallah A Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
description	Shelly Wang,1 Aria Fallah2,3 1Department of Neurosurgery, University of Toronto, Toronto, ON, Canada; 2Department of Neurosurgery, Miami Children’s Hospital, Miami, FL, USA; 3Department of Clinical Epidemiology and Biostatistics, McMaster University, Hamilton, ON, Canada Abstract: Seizures are clinically significant manifestations associated with 79%–90% of patients with tuberous sclerosis complex. Often occurring within the first year of life in the form of infantile spasms, seizures interfere with neuropsychiatric, social, and cognitive development and carry significant individual and societal consequences. Prompt identification and treatment of seizures is an important focus in the overall management of tuberous sclerosis complex patients. Medical management, either after seizure onset or prophylactically in infants with electroencephalographic abnormalities, is considered first-line therapy. Vigabatrin and adrenocorticotropic hormone have emerged over the past few decades as mainstay pharmacologic modalities. Furthermore, emerging research on mammalian target of rapamycin inhibitors demonstrated promise for the management of seizures and subependymal giant cell astrocytoma. For appropriate surgical candidates with an epileptogenic zone associated with one or more glioneuronal hamartomas, ideally in noneloquent cortex, resective surgery can be considered, which provides a cure in 56% of patients. For medically refractory patients who do not meet criteria for curative surgery, palliative surgical approaches focused on reducing seizure burden, in the form of corpus callosotomy and vagus nerve stimulation, are alternative management options. Lastly, the ketogenic diet, a reemerging therapy based on the anticonvulsant effects of ketone bodies, can be utilized independently or in conjunction with other treatment modalities for the management of difficult-to-treat seizures. Keywords: epilepsy, adrenocorticotropic hormone, vigabatrin, mammalian target of rapamycin, ketogenic diet, vagus nerve stimulation
format	article
author	Wang S Fallah A
author_facet	Wang S Fallah A
author_sort	Wang S
title	Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
title_short	Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
title_full	Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
title_fullStr	Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
title_full_unstemmed	Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
title_sort	optimal management of seizures associated with tuberous sclerosis complex: current and emerging options
publisher	Dove Medical Press
publishDate	2014
url	https://doaj.org/article/3d5d0ad08b364b47a0e8b657d546d7c9
work_keys_str_mv	AT wangs optimalmanagementofseizuresassociatedwithtuberoussclerosiscomplexcurrentandemergingoptions AT fallaha optimalmanagementofseizuresassociatedwithtuberoussclerosiscomplexcurrentandemergingoptions
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Optimal management of seizures associated with tuberous sclerosis complex: current and emerging options

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