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Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms

Infantile spasms (IS) is an epileptic encephalopathy with unique clinical and electrographic features, which affects children in the middle of the first year of life. The pathophysiology of IS remains incompletely understood, despite the heterogeneity of IS etiologies, more than 200 of which are kno...

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Autores principales: Janicot, Remi, Shao, Li-Rong, Stafstrom, Carl E.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7023485/
https://www.ncbi.nlm.nih.gov/pubmed/31935804
http://dx.doi.org/10.3390/children7010005
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author Janicot, Remi
Shao, Li-Rong
Stafstrom, Carl E.
author_facet Janicot, Remi
Shao, Li-Rong
Stafstrom, Carl E.
author_sort Janicot, Remi
collection PubMed
description Infantile spasms (IS) is an epileptic encephalopathy with unique clinical and electrographic features, which affects children in the middle of the first year of life. The pathophysiology of IS remains incompletely understood, despite the heterogeneity of IS etiologies, more than 200 of which are known. In particular, the neurobiological basis of why multiple etiologies converge to a relatively similar clinical presentation has defied explanation. Treatment options for this form of epilepsy, which has been described as “catastrophic” because of the poor cognitive, developmental, and epileptic prognosis, are limited and not fully effective. Until the pathophysiology of IS is better clarified, novel treatments will not be forthcoming, and preclinical (animal) models are essential for advancing this knowledge. Here, we review preclinical IS models, update information regarding already existing models, describe some novel models, and discuss exciting new data that promises to advance understanding of the cellular mechanisms underlying the specific EEG changes seen in IS—interictal hypsarrhythmia and ictal electrodecrement.
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spelling pubmed-70234852020-03-12 Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms Janicot, Remi Shao, Li-Rong Stafstrom, Carl E. Children (Basel) Review Infantile spasms (IS) is an epileptic encephalopathy with unique clinical and electrographic features, which affects children in the middle of the first year of life. The pathophysiology of IS remains incompletely understood, despite the heterogeneity of IS etiologies, more than 200 of which are known. In particular, the neurobiological basis of why multiple etiologies converge to a relatively similar clinical presentation has defied explanation. Treatment options for this form of epilepsy, which has been described as “catastrophic” because of the poor cognitive, developmental, and epileptic prognosis, are limited and not fully effective. Until the pathophysiology of IS is better clarified, novel treatments will not be forthcoming, and preclinical (animal) models are essential for advancing this knowledge. Here, we review preclinical IS models, update information regarding already existing models, describe some novel models, and discuss exciting new data that promises to advance understanding of the cellular mechanisms underlying the specific EEG changes seen in IS—interictal hypsarrhythmia and ictal electrodecrement. MDPI 2020-01-06 /pmc/articles/PMC7023485/ /pubmed/31935804 http://dx.doi.org/10.3390/children7010005 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Janicot, Remi
Shao, Li-Rong
Stafstrom, Carl E.
Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title_full Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title_fullStr Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title_full_unstemmed Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title_short Infantile Spasms: An Update on Pre-Clinical Models and EEG Mechanisms
title_sort infantile spasms: an update on pre-clinical models and eeg mechanisms
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7023485/
https://www.ncbi.nlm.nih.gov/pubmed/31935804
http://dx.doi.org/10.3390/children7010005
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