Please use this identifier to cite or link to this item: https://ahro.austin.org.au/austinjspui/handle/1/34740
Title: Applying the ILAE diagnostic criteria for Lennox-Gastaut syndrome in the real-world setting: A multicentre retrospective cohort study.
Austin Authors: Nightscales, Russell ;Chen, Zhibin;Barnard, Sarah;Auvrez, Clarissa;Tao, Gerard;Sivathamboo, Shobi;Bennett, Caitlin;Rychkova, Maria;D'Souza, Wendyl;Berkovic, Samuel F ;Nicolo, John-Paul;O'Brien, Terence J;Perucca, Piero ;Scheffer, Ingrid E ;Kwan, Patrick
Affiliation: Department of Neuroscience, Central Clinical School, Monash University, Melbourne, Victoria, Australia.;Department of Neurology, Alfred Health, Melbourne, Victoria, Australia.
NorthWestern Mental Health, Melbourne Health, Melbourne, Victoria, Australia.
Department of Medicine (The Royal Melbourne Hospital), The University of Melbourne, Victoria, Australia.
Department of Neuroscience, Central Clinical School, Monash University, Melbourne, Victoria, Australia.;Department of Neurology, Alfred Health, Melbourne, Victoria, Australia.;Department of Medicine (The Royal Melbourne Hospital), The University of Melbourne, Victoria, Australia.;Department of Neurology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia.
Epilepsy Research Centre, Department of Medicine (Austin Health), The University of Melbourne, Heidelberg, Victoria, Australia.
Department of Medicine (The Royal Melbourne Hospital), The University of Melbourne, Victoria, Australia.
Department of Medicine, St. Vincent's Hospital, The University of Melbourne, Fitzroy, Victoria, Australia.
Epilepsy Research Centre
Neurology
Issue Date: 22-Dec-2023
Date: 2023
Publication information: Epilepsia Open 2023-12-22
Abstract: Lennox-Gastaut syndrome (LGS) is an archetypal developmental and epileptic encephalopathy, for which novel treatments are emerging. Diagnostic criteria for LGS have recently been defined by the International League Against Epilepsy (ILAE). We aimed to apply these criteria in the real-world setting. We applied ILAE diagnostic criteria to a cohort of patients diagnosed with LGS by epileptologists following inpatient video-EEG monitoring (VEM) at tertiary comprehensive epilepsy centres between 1995 and 2015. We also assessed mortality in this cohort. Sixty patients diagnosed with LGS and had complete records available for review were Identified. Among them, 29 (48%) patients met ILAE diagnostic criteria for LGS (ILAE-DC group). Thirty-one did not meet criteria (non-ILAE-DC) due to absence of documented tonic seizures (n=7), EEG features (n=12), or both tonic seizures and EEG features (n=10), intellectual disability (n=1), or drug resistance (n=1). The ILAE-DC group had a shorter duration of epilepsy at VEM than the non-ILAE-DC group (median = 12.0 years Vs 23.7 years, respectively; p=0.015). The proportions of patients with multiple seizure types (100% vs. 96.7%), ≤2.5Hz slow spike-and-wave EEG activity (100% vs. 90%), seizure-related injuries (27.6% vs 25.8%), and mortality (standardized mortality ratio 4.60 vs. 5.12) were similar between the groups. Up to 52% of patients diagnosed with LGS following VEM may not meet recently accepted ILAE criteria for LGS diagnosis. This may reflect both the limitations of retrospective medical record review and a historical tendency of applying the LGS diagnosis to a broad spectrum of severe, early-onset drug-resistant epilepsies with drop attacks. The ILAE criteria allow delineation of LGS based on distinct electroclinical features, potentiating accurate diagnosis, prognostication, and management formulation. Nonetheless, mortality outcomes between those who did and did not meet ILAE diagnostic criteria for LGS were similarly poor, and both groups suffered high rates of seizure-related injury.
URI: https://ahro.austin.org.au/austinjspui/handle/1/34740
DOI: 10.1002/epi4.12894
ORCID: 0000-0001-9108-207X
0000-0002-1888-6917
0000-0003-0517-2533
0000-0003-4638-9579
0000-0003-4580-841X
0000-0002-0473-6475
0000-0002-7855-7066
0000-0002-2311-2174
0000-0001-7310-276X
Journal: Epilepsia Open
PubMed URL: 38135919
ISSN: 2470-9239
Type: Journal Article
Appears in Collections:Journal articles

Show full item record

Page view(s)

16
checked on Apr 17, 2024

Google ScholarTM

Check


Items in AHRO are protected by copyright, with all rights reserved, unless otherwise indicated.