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Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome

BACKGROUND: Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are two typical forms of...

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Autores principales: He, Na, Li, Bing-Mei, Li, Zhao-Xia, Wang, Jie, Liu, Xiao-Rong, Meng, Heng, Tang, Bin, Bian, Wen-Jun, Shi, Yi-Wu, Liao, Wei-Ping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5859706/
https://www.ncbi.nlm.nih.gov/pubmed/29558884
http://dx.doi.org/10.1186/s11689-018-9229-x
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author He, Na
Li, Bing-Mei
Li, Zhao-Xia
Wang, Jie
Liu, Xiao-Rong
Meng, Heng
Tang, Bin
Bian, Wen-Jun
Shi, Yi-Wu
Liao, Wei-Ping
author_facet He, Na
Li, Bing-Mei
Li, Zhao-Xia
Wang, Jie
Liu, Xiao-Rong
Meng, Heng
Tang, Bin
Bian, Wen-Jun
Shi, Yi-Wu
Liao, Wei-Ping
author_sort He, Na
collection PubMed
description BACKGROUND: Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are two typical forms of intractable epileptic encephalopathy associated with ID. We previously reported that ASD was diagnosed in 24.3% of patients with DS, higher in those with profound ID. Given the severe epilepsy and high frequency of ID in LGS, it is necessary to know whether ASD is a common psychomotor co-morbidity of LGS. This study evaluated the autistic behaviors and intelligence in patients with LGS and further compared that between LGS and DS, aiming to understand the complex pathogenesis of epilepsy-ASD-ID triad. METHODS: A total of 50 patients with LGS and 45 patients with DS were enrolled and followed up for at least 3 years. The clinical characteristics were analyzed, and evaluations of ASD and ID were performed. RESULTS: No patients with LGS fully met the diagnostic criteria for ASD, but three of them exhibited more or less autistic behaviors. Majority (86%) of LGS patients presented ID, among which moderate to severe ID was the most common. Early onset age and symptomatic etiology were risk predictors for ID. The prevalence of ASD in LGS was significantly lower than that in DS (0/50 vs. 10/45, p < 0.001), while the prevalence and severity of ID showed no significant difference between the two forms of epileptic encephalopathy. CONCLUSIONS: This study demonstrated a significant difference in the co-morbidity of ASD between LGS and DS, although they had a similar prevalence and severity of ID, refuting the proposal that the prevalence of ASD in epilepsy is accounted for by ID. These findings suggest that the co-morbidity of ASD, ID, and epilepsy may result from multifaceted pathogenic mechanisms.
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spelling pubmed-58597062018-03-22 Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome He, Na Li, Bing-Mei Li, Zhao-Xia Wang, Jie Liu, Xiao-Rong Meng, Heng Tang, Bin Bian, Wen-Jun Shi, Yi-Wu Liao, Wei-Ping J Neurodev Disord Research BACKGROUND: Autism spectrum disorder (ASD) in epilepsy has been a topic of increasing interest, which in general occurs in 15–35% of the patients with epilepsy, more frequently in those with intellectual disability (ID). Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS) are two typical forms of intractable epileptic encephalopathy associated with ID. We previously reported that ASD was diagnosed in 24.3% of patients with DS, higher in those with profound ID. Given the severe epilepsy and high frequency of ID in LGS, it is necessary to know whether ASD is a common psychomotor co-morbidity of LGS. This study evaluated the autistic behaviors and intelligence in patients with LGS and further compared that between LGS and DS, aiming to understand the complex pathogenesis of epilepsy-ASD-ID triad. METHODS: A total of 50 patients with LGS and 45 patients with DS were enrolled and followed up for at least 3 years. The clinical characteristics were analyzed, and evaluations of ASD and ID were performed. RESULTS: No patients with LGS fully met the diagnostic criteria for ASD, but three of them exhibited more or less autistic behaviors. Majority (86%) of LGS patients presented ID, among which moderate to severe ID was the most common. Early onset age and symptomatic etiology were risk predictors for ID. The prevalence of ASD in LGS was significantly lower than that in DS (0/50 vs. 10/45, p < 0.001), while the prevalence and severity of ID showed no significant difference between the two forms of epileptic encephalopathy. CONCLUSIONS: This study demonstrated a significant difference in the co-morbidity of ASD between LGS and DS, although they had a similar prevalence and severity of ID, refuting the proposal that the prevalence of ASD in epilepsy is accounted for by ID. These findings suggest that the co-morbidity of ASD, ID, and epilepsy may result from multifaceted pathogenic mechanisms. BioMed Central 2018-03-20 /pmc/articles/PMC5859706/ /pubmed/29558884 http://dx.doi.org/10.1186/s11689-018-9229-x Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research
He, Na
Li, Bing-Mei
Li, Zhao-Xia
Wang, Jie
Liu, Xiao-Rong
Meng, Heng
Tang, Bin
Bian, Wen-Jun
Shi, Yi-Wu
Liao, Wei-Ping
Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title_full Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title_fullStr Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title_full_unstemmed Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title_short Few individuals with Lennox-Gastaut syndrome have autism spectrum disorder: a comparison with Dravet syndrome
title_sort few individuals with lennox-gastaut syndrome have autism spectrum disorder: a comparison with dravet syndrome
topic Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5859706/
https://www.ncbi.nlm.nih.gov/pubmed/29558884
http://dx.doi.org/10.1186/s11689-018-9229-x
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