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Diagnosis of Lennox-Gastaut syndrome and strategies for early recognition

Pujar, Suresh; Cross, J Helen; (2024) Diagnosis of Lennox-Gastaut syndrome and strategies for early recognition. Expert Review of Neurotherapeutics , 24 (4) pp. 383-389. 10.1080/14737175.2024.2323568. Green open access

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Abstract

INTRODUCTION: Lennox Gastaut syndrome (LGS) as an electroclinical diagnosis has been utilized as a clinical entity for more than 70 years. However, with the recognition of other distinct electroclinical epilepsy syndromes, no consistent single etiology, and the variability of criteria used in clinical trials, the clinical utility of such a diagnosis has been questioned. Recently, the International League Against Epilepsy for the first time defined diagnostic criteria for epilepsy syndromes, thereby allowing consistent language and inclusion criteria to be utilized. AREAS COVERED: Recent diagnostic criteria for syndrome diagnosis are explored as defined by the International League Against Epilepsy, with further literature reviewed to highlight relevant features, and differential diagnosis explored. EXPERT OPINION: Developmental and Epileptic Encephalopathy (DEE) is an overall term that may be descriptive of many different epilepsies, most of early onset, whether electroclinically or etiologically defined, of which LGS is one. Although we have moved forward in defining an increasing number of etiologically specific syndromes, this to date remains a minority of the DEEs. Although there is progress with precision medicine targeted at specific causes, the term LGS still remains useful as a diagnosis in defining treatment options, as well as overall prognosis.

Type: Article
Title: Diagnosis of Lennox-Gastaut syndrome and strategies for early recognition
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1080/14737175.2024.2323568
Publisher version: http://dx.doi.org/10.1080/14737175.2024.2323568
Language: English
Additional information: © 2024 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way. The terms on which this article has been published allow the posting of the Accepted Manuscript in a repository by the author(s) or with their consent.
Keywords: DEE, Lennox Gastaut syndrome, diagnostic challenge, early diagnosis, encephalopathy, syndrome-in-evolution, targeted therapy
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > ICH - Directors Office
URI: https://discovery-pp.ucl.ac.uk/id/eprint/10188919
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