Pub Date : 2025-02-20DOI: 10.1016/j.yebeh.2025.110269
M.Scott Perry , Dax Bourcier , Paula Brna
The goal of epilepsy treatment is to achieve the most significant seizure reduction, aiming for seizure freedom, in the absence of consequential adverse effects. For children with drug resistant epilepsy (DRE), surgical therapy may offer the best chance of seizure freedom, but is vastly underutilized. In cases where seizure freedom is not possible, surgery may still provide meaningful seizure reduction over that expected from continued medical management. In addition, seizure freedom and reduction can have meaningful impact on non-seizure outcomes including mortality, cognition, behavior, and cost of care. It is essential to compare the relative risks and benefits of continued medical therapy versus surgical therapy in order to best inform choice of treatment in pediatric DRE and avoid unnecessary delays. In this review, we explore the seizure and non-seizure benefits of epilepsy surgery, including curative procedures and those expected to result in meaningful seizure reduction, compared to continued medical management in children.
{"title":"Surgical versus medical treatment of drug resistant epilepsy in children: Seizure and non-seizure outcomes","authors":"M.Scott Perry , Dax Bourcier , Paula Brna","doi":"10.1016/j.yebeh.2025.110269","DOIUrl":"10.1016/j.yebeh.2025.110269","url":null,"abstract":"<div><div>The goal of epilepsy treatment is to achieve the most significant seizure reduction, aiming for seizure freedom, in the absence of consequential adverse effects. For children with drug resistant epilepsy (DRE), surgical therapy may offer the best chance of seizure freedom, but is vastly underutilized. In cases where seizure freedom is not possible, surgery may still provide meaningful seizure reduction over that expected from continued medical management. In addition, seizure freedom and reduction can have meaningful impact on non-seizure outcomes including mortality, cognition, behavior, and cost of care. It is essential to compare the relative risks and benefits of continued medical therapy versus surgical therapy in order to best inform choice of treatment in pediatric DRE and avoid unnecessary delays. In this review, we explore the seizure and non-seizure benefits of epilepsy surgery, including curative procedures and those expected to result in meaningful seizure reduction, compared to continued medical management in children.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110269"},"PeriodicalIF":2.3,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143444649","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-20DOI: 10.1016/j.yebeh.2025.110267
Karina Morales , Tracy De Los Santos , Danielle Harvey , David Dunn , Jana Jones , Anna Byars , Joan Austin , Bruce Hermann , Temitayo Oyegbile-Chidi
Rationale
Children with new-onset epilepsy often experience co-morbid cognitive and behavioral challenges, which can be influenced by Social Determinants Of Health (SDOH) such as household income and parental education level. Although unsupervised machine learning has identified distinct cognitive and behavioral phenotypes at or near diagnosis, the relationship between these clusters remains underexplored. This study aims to examine the relationship between cognitive and behavioral clusters and the impact of SDOH among children with new-onset seizures.
Methods
We recruited 312 children (ages 6–16) within six weeks of their first recognized seizure. Each participant underwent a comprehensive neuropsychological assessment, from which factor analysis identified four primary domains: language, processing speed, executive function, and verbal memory. Parents also completed the Child’s Behavior Checklist (CBCL). K-means cluster analysis was applied to the mean factor scores and CBCL T-scores to identify unique clusters. We assessed SDOH factors, including maternal education level, child’s race, household income, and parental marital status, along with clinical epilepsy characteristics such as age at seizure onset, seizure frequency/intensity, seizure syndrome, MRI/EEG abnormalities, and neurologic examination findings to distinguish these clusters.
Results
We identified two primary clusters within both cognitive and behavioral scores: Resilient and At-Risk. Children in the Resilient Cognitive Cluster exhibited fewer behavioral problems, while those in the Resilient Behavior Cluster demonstrated higher cognitive performance. Conversely, the At-Risk Cognitive Cluster was associated with greater behavioral problems, and the At-Risk Behavior Cluster correlated with lower cognitive performance. Notably, almost two-thirds of participants showed congruence in clustering, either displaying resilience in both cognition and behavior or vulnerability in both domains. Resilient children exhibited lower levels of sociodemographic disadvantage, whereas those in the At-Risk Clusters faced significant disadvantages. Sociodemographic factors were more pronounced in differentiating clusters compared to traditional clinical epilepsy characteristics.
Conclusions
Among children with new-onset seizures, some display significant resilience to multimorbidities, while others are particularly vulnerable to neurobehavioral challenges, often linked to sociodemographic disadvantages. Future research should explore whether early interventions targeting SDOH can mitigate these risks and improve outcomes for children with new-onset epilepsy.
{"title":"Stratifying cognitive and behavioral comorbidities in children with new-onset seizures – The influence of sociodemographic disadvantage","authors":"Karina Morales , Tracy De Los Santos , Danielle Harvey , David Dunn , Jana Jones , Anna Byars , Joan Austin , Bruce Hermann , Temitayo Oyegbile-Chidi","doi":"10.1016/j.yebeh.2025.110267","DOIUrl":"10.1016/j.yebeh.2025.110267","url":null,"abstract":"<div><h3>Rationale</h3><div>Children with new-onset epilepsy often experience co-morbid cognitive and behavioral challenges, which can be influenced by Social Determinants Of Health (SDOH) such as household income and parental education level. Although unsupervised machine learning has identified distinct cognitive and behavioral phenotypes at or near diagnosis, the relationship between these clusters remains underexplored. This study aims to examine the relationship between cognitive and behavioral clusters and the impact of SDOH among children with new-onset seizures.</div></div><div><h3>Methods</h3><div>We recruited 312 children (ages 6–16) within six weeks of their first recognized seizure. Each participant underwent a comprehensive neuropsychological assessment, from which factor analysis identified four primary domains: language, processing speed, executive function, and verbal memory. Parents also completed the Child’s Behavior Checklist (CBCL). K-means cluster analysis was applied to the mean factor scores and CBCL T-scores to identify unique clusters. We assessed SDOH factors, including maternal education level, child’s race, household income, and parental marital status, along with clinical epilepsy characteristics such as age at seizure onset, seizure frequency/intensity, seizure syndrome, MRI/EEG abnormalities, and neurologic examination findings to distinguish these clusters.</div></div><div><h3>Results</h3><div>We identified two primary clusters within both cognitive and behavioral scores: Resilient and At-Risk. Children in the Resilient Cognitive Cluster exhibited fewer behavioral problems, while those in the Resilient Behavior Cluster demonstrated higher cognitive performance. Conversely, the At-Risk Cognitive Cluster was associated with greater behavioral problems, and the At-Risk Behavior Cluster correlated with lower cognitive performance. Notably, almost two-thirds of participants showed congruence in clustering, either displaying resilience in both cognition and behavior or vulnerability in both domains. Resilient children exhibited lower levels of sociodemographic disadvantage, whereas those in the At-Risk Clusters faced significant disadvantages. Sociodemographic factors were more pronounced in differentiating clusters compared to traditional clinical epilepsy characteristics.</div></div><div><h3>Conclusions</h3><div>Among children with new-onset seizures, some display significant resilience to multimorbidities, while others are particularly vulnerable to neurobehavioral challenges, often linked to sociodemographic disadvantages. Future research should explore whether early interventions targeting SDOH can mitigate these risks and improve outcomes for children with new-onset epilepsy.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110267"},"PeriodicalIF":2.3,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143444647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-20DOI: 10.1016/j.yebeh.2025.110323
Sofía Lallana , Samuel López-Maza , Gemma Ortega , Elena Fonseca , Manuel Quintana , Laura Abraira , Enric Bellido , Daniel Campos-Fernández , Estevo Santamarina , Agustín Ruiz , Lluís Tárraga , Mercè Boada , Manuel Toledo
Background
This study aimed to explore quantitative electroencephalography (qEEG) biomarkers of cognitive performance in drug-resistant temporal lobe epilepsy (TLE) and analyze their relationship with clinical characteristics.
Methods
Cross-sectional study including adult patients with drug-resistant TLE and a control group. Resting-state, eyes-closed qEEG samples were analyzed using the fast Fourier transform approach. Power spectral density was calculated for four frequency bands: delta (1–3.9 Hz), theta (4–7.9 Hz), alpha (8–12.9 Hz), and beta (13–18 Hz). Neuropsychological tests were administered to TLE patients.
Results
Twenty-nine TLE patients (mean age 42 ± 8.2 years; 44.8 % women) and 23 age- and sex-matched controls were enrolled. Clinically significant cognitive impairment was found in 86.2 % of patients (58.6 % amnestic). Compared to controls, TLE patients showed increased ipsilateral power spectral density for the theta (p = 0.045), alpha (p = 0.023) and beta bands in the anterior region (p = 0.029) and for the delta band in the posterior region (p = 0.03). Alpha/theta ratio (ATR) was lower in the posterior quadrant of the epileptogenic hemisphere (p = 0.013), and higher seizure frequency correlated with a lower ATR in the ipsilateral temporal region (r: −0.425; p = 0.021). Patients with amnestic cognitive impairment exhibited higher power spectral density across most frequency bands (p < 0.005). Impaired verbal memory and executive function were associated with increased power density.
Conclusion
Increased power spectral density was evident in all frequency bands in the epileptogenic hemisphere, particularly in those patients with amnestic cognitive impairment. Moreover, higher seizure frequency correlated with a lower ATR in the temporal region. Power spectral analysis can provide useful information in drug-resistant TLE patients.
{"title":"Quantitative EEG biomarkers of cognitive performance in drug-resistant temporal lobe epilepsy","authors":"Sofía Lallana , Samuel López-Maza , Gemma Ortega , Elena Fonseca , Manuel Quintana , Laura Abraira , Enric Bellido , Daniel Campos-Fernández , Estevo Santamarina , Agustín Ruiz , Lluís Tárraga , Mercè Boada , Manuel Toledo","doi":"10.1016/j.yebeh.2025.110323","DOIUrl":"10.1016/j.yebeh.2025.110323","url":null,"abstract":"<div><h3>Background</h3><div>This study aimed to explore quantitative electroencephalography (qEEG) biomarkers of cognitive performance in drug-resistant temporal lobe epilepsy (TLE) and analyze their relationship with clinical characteristics.</div></div><div><h3>Methods</h3><div>Cross-sectional study including adult patients with drug-resistant TLE and a control group. Resting-state, eyes-closed qEEG samples were analyzed using the fast Fourier transform approach. Power spectral density was calculated for four frequency bands: delta (1–3.9 Hz), theta (4–7.9 Hz), alpha (8–12.9 Hz), and beta (13–18 Hz). Neuropsychological tests were administered to TLE patients.</div></div><div><h3>Results</h3><div>Twenty-nine TLE patients (mean age 42 ± 8.2 years; 44.8 % women) and 23 age- and sex-matched controls were enrolled. Clinically significant cognitive impairment was found in 86.2 % of patients (58.6 % amnestic). Compared to controls, TLE patients showed increased ipsilateral power spectral density for the theta (p = 0.045), alpha (p = 0.023) and beta bands in the anterior region (p = 0.029) and for the delta band in the posterior region (p = 0.03). Alpha/theta ratio (ATR) was lower in the posterior quadrant of the epileptogenic hemisphere (p = 0.013), and higher seizure frequency correlated with a lower ATR in the ipsilateral temporal region (r: −0.425; p = 0.021). Patients with amnestic cognitive impairment exhibited higher power spectral density across most frequency bands (p < 0.005). Impaired verbal memory and executive function were associated with increased power density.</div></div><div><h3>Conclusion</h3><div>Increased power spectral density was evident in all frequency bands in the epileptogenic hemisphere, particularly in those patients with amnestic cognitive impairment. Moreover, higher seizure frequency correlated with a lower ATR in the temporal region. Power spectral analysis can provide useful information in drug-resistant TLE patients.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110323"},"PeriodicalIF":2.3,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143452891","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2025.110279
Ana Arenivas , Lisa Ferguson , Brittany Lapin , Yadi Li , Ingmar Blumcke , Imad Najm , Robyn M. Busch
Purpose
Almost half of pharmacoresistant epilepsies in childhood and adolescence are caused by malformations of cortical development (MCDs), but little is known about the associated neuropsychological morbidities. This study comprehensively characterized presurgical neuropsychological functions in children and adolescents with pharmacoresistant epilepsy due to MCDs and examined their relationships to neuropathological substrate and other clinical variables.
Methods
Retrospective data were obtained from 137 children and adolescents (mean age = 13 years; 58 % male) who underwent resective surgery for treatment of epilepsy and had pathologically-confirmed MCDs. Neuropsychological domain composite scores and overall cognitive phenotype were examined. Logistic regressions identified demographic and disease variables associated with neuropsychological functioning.
Results
Pathological diagnoses included focal cortical dysplasia (FCD, n = 69; 30 % FCD Type IIB, 20 % FCD Type IIA, 1 % FCD Type IA) and other MCDs (n = 68; 23 % mild MCD, 7 % polymicrogyria, 7 % tuberous sclerosis complex, 6 % complex MCD, 5 % mild MCD with oligodendroglial hyperplasia in epilepsy, 2 % periventricular nodular heterotopia). Performance was highly variable, ranging from superior to extremely low across cognitive domains. Impairment rates ranged from 40.1 % (visuospatial skills) to 70.8 % (fine motor skills). Of patients and parents/caregivers able to complete standardized inventories of mood and anxiety, approximately 20 % endorsed concerns for depression and anxiety. A large subset (29 %) demonstrated cognitive deficits limited to a single domain, with processing speed (24 %) and language (20 %) being the most commonly affected domains. Younger age at epilepsy onset and multilobar seizure locus were associated with lower cognitive performance across multiple domains. No significant differences in cognition existed between children and adolescents with focal cortical dysplasias and those with other MCDs.
Conclusions
Findings suggest the range of cognitive abilities in children and adolescents with MCDs is much broader than previously described, with over 20% demonstrating an intact cognitive phenotype. Despite high prevalence of cognitive impairment in this cohort, significant variability existed at the individual level that was not fully accounted for by demographic and clinical variables. Results highlight the importance of neuropsychological evaluation and routine emotional/behavioral screening in pediatric epilepsy caused by MCDs.
{"title":"Neuropsychological functioning in children and adolescents with pharmacoresistant epilepsy due to malformations of cortical development","authors":"Ana Arenivas , Lisa Ferguson , Brittany Lapin , Yadi Li , Ingmar Blumcke , Imad Najm , Robyn M. Busch","doi":"10.1016/j.yebeh.2025.110279","DOIUrl":"10.1016/j.yebeh.2025.110279","url":null,"abstract":"<div><h3>Purpose</h3><div>Almost half of pharmacoresistant epilepsies in childhood and adolescence are caused by malformations of cortical development (MCDs), but little is known about the associated neuropsychological morbidities. This study comprehensively characterized presurgical neuropsychological functions in children and adolescents with pharmacoresistant epilepsy due to MCDs and examined their relationships to neuropathological substrate and other clinical variables.</div></div><div><h3>Methods</h3><div>Retrospective data were obtained from 137 children and adolescents (mean age = 13 years; 58 % male) who underwent resective surgery for treatment of epilepsy and had pathologically-confirmed MCDs. Neuropsychological domain composite scores and overall cognitive phenotype were examined. Logistic regressions identified demographic and disease variables associated with neuropsychological functioning.</div></div><div><h3>Results</h3><div>Pathological diagnoses included focal cortical dysplasia (FCD, n = 69; 30 % FCD Type IIB, 20 % FCD Type IIA, 1 % FCD Type IA) and other MCDs (n = 68; 23 % mild MCD, 7 % polymicrogyria, 7 % tuberous sclerosis complex, 6 % complex MCD, 5 % mild MCD with oligodendroglial hyperplasia in epilepsy, 2 % periventricular nodular heterotopia). Performance was highly variable, ranging from superior to extremely low across cognitive domains. Impairment rates ranged from 40.1 % (visuospatial skills) to 70.8 % (fine motor skills). Of patients and parents/caregivers able to complete standardized inventories of mood and anxiety, approximately 20 % endorsed concerns for depression and anxiety. A large subset (29 %) demonstrated cognitive deficits limited to a single domain, with processing speed (24 %) and language (20 %) being the most commonly affected domains. Younger age at epilepsy onset and multilobar seizure locus were associated with lower cognitive performance across multiple domains. No significant differences in cognition existed between children and adolescents with focal cortical dysplasias and those with other MCDs.</div></div><div><h3>Conclusions</h3><div>Findings suggest the range of cognitive abilities in children and adolescents with MCDs is much broader than previously described, with over 20% demonstrating an intact cognitive phenotype. Despite high prevalence of cognitive impairment in this cohort, significant variability existed at the individual level that was not fully accounted for by demographic and clinical variables. Results highlight the importance of neuropsychological evaluation and routine emotional/behavioral screening in pediatric epilepsy caused by MCDs.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"164 ","pages":"Article 110279"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143444256","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2025.110312
Maria Augusta Montenegro , Kette D. Valente , Matheus Dorigatti Soldatelli , Leticia Brito Sampaio , Anna Leticia Pinto
Sturge-Weber Syndrome (SWS) is a neurovascular condition caused by a mutation in the GNAQ gene. The most common neurological manifestations of SWS are epilepsy, developmental delay, and stroke-like episodes. Seizures are often the first neurological symptom, and most patients have drug-resistant epilepsy. Predictors for unfavorable epilepsy outcomes and the need for ASM polytherapy included age of onset, bilateral brain involvement, extensive unilateral hemispheric disease, and a positive family history of epilepsy. The surgical approach to SWS is challenging due to the associated abnormal vasculature and potential complications. Hemispherotomy is associated with high seizure freedom rates and ASM discontinuation, but resective surgery may be an efficient alternative in well-selected patients. Complete seizure control is the ultimate goal of epilepsy surgery; however, reducing seizure severity and frequency may help improve cognitive outcome and quality of life.
{"title":"Epilepsy surgery in patients with Sturge-Weber Syndrome","authors":"Maria Augusta Montenegro , Kette D. Valente , Matheus Dorigatti Soldatelli , Leticia Brito Sampaio , Anna Leticia Pinto","doi":"10.1016/j.yebeh.2025.110312","DOIUrl":"10.1016/j.yebeh.2025.110312","url":null,"abstract":"<div><div>Sturge-Weber Syndrome (SWS) is a neurovascular condition caused by a mutation in the GNAQ gene. The most common neurological manifestations of SWS are epilepsy, developmental delay, and stroke-like episodes. Seizures are often the first neurological symptom, and most patients have drug-resistant epilepsy. Predictors for unfavorable epilepsy outcomes and the need for ASM polytherapy included age of onset, bilateral brain involvement, extensive unilateral hemispheric disease, and a positive family history of epilepsy. The surgical approach to SWS is challenging due to the associated abnormal vasculature and potential complications. Hemispherotomy is associated with high seizure freedom rates and ASM discontinuation, but resective surgery may be an efficient alternative in well-selected patients. Complete seizure control is the ultimate goal of epilepsy surgery; however, reducing seizure severity and frequency may help improve cognitive outcome and quality of life.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110312"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2025.110308
Attila Altunel , Aynur Muduroglu-Kirmizibekmez , Alparslan Onder , Ozlem Altunel , Ali Sever , Ihsan Kara
Background
Landau-Kleffner Syndrome (LKS) and Autism Spectrum Disorder (ASD), both neurodevelopmental disorders, are frequently associated with epileptic seizures and characteristic epileptiform activity. Electrical Status Epilepticus during Sleep (ESES) is commonly observed in LKS, while Interictal Epileptiform Discharges (IEDs) are typical in ASD. Adrenocorticotropic hormone (ACTH) treatment has demonstrated the potential to reduce the indexes of these related discharges and the number of seizures.
Objective
This retrospective study aimed to assess the response to ACTH treatment in children diagnosed with LKS or ASD, both with and without epileptic seizures.
Methods
The study included 236 children, with separate analyses performed for those diagnosed with LKS or ASD. EEG recordings and treatment outcomes were retrospectively evaluated. Key assessments included changes in the indexes of ESES and IED, seizure control rates, and reported side effects.
Results
ACTH treatment led to significant improvements in indexes and seizure control in both LKS and ASD populations. In children with LKS and epileptic seizures, the mean ESES index reduction was 49.9 % (±17.7), with 50 % achieving complete seizure control. For children with ASD and epileptic seizures, the mean IED index reduction was 47.2 % (±16.7), with 41 % achieving complete seizure control. Rare side effects were transient and reversible, with no reports of serious adverse events.
Conclusion
ACTH treatment demonstrates efficacy in reducing ESES and IED indexes and controlling seizures in children with LKS and ASD. These findings underscore the importance of early intervention and careful management of side effects in optimizing outcomes for these patient populations.
{"title":"Efficacy of ACTH therapy in children with Landau-Kleffner Syndrome and Autism Spectrum Disorder: A retrospective analysis","authors":"Attila Altunel , Aynur Muduroglu-Kirmizibekmez , Alparslan Onder , Ozlem Altunel , Ali Sever , Ihsan Kara","doi":"10.1016/j.yebeh.2025.110308","DOIUrl":"10.1016/j.yebeh.2025.110308","url":null,"abstract":"<div><h3>Background</h3><div>Landau-Kleffner Syndrome (LKS) and Autism Spectrum Disorder (ASD), both neurodevelopmental disorders, are frequently associated with epileptic seizures and characteristic epileptiform activity. Electrical Status Epilepticus during Sleep (ESES) is commonly observed in LKS, while Interictal Epileptiform Discharges (IEDs) are typical in ASD. Adrenocorticotropic hormone (ACTH) treatment has demonstrated the potential to reduce the indexes of these related discharges and the number of seizures.</div></div><div><h3>Objective</h3><div>This retrospective study aimed to assess the response to ACTH treatment in children diagnosed with LKS or ASD, both with and without epileptic seizures.</div></div><div><h3>Methods</h3><div>The study included 236 children, with separate analyses performed for those diagnosed with LKS or ASD. EEG recordings and treatment outcomes were retrospectively evaluated. Key assessments included changes in the indexes of ESES and IED, seizure control rates, and reported side effects.</div></div><div><h3>Results</h3><div>ACTH treatment led to significant improvements in indexes and seizure control in both LKS and ASD populations. In children with LKS and epileptic seizures, the mean ESES index reduction was 49.9 % (±17.7), with 50 % achieving complete seizure control. For children with ASD and epileptic seizures, the mean IED index reduction was 47.2 % (±16.7), with 41 % achieving complete seizure control. Rare side effects were transient and reversible, with no reports of serious adverse events.</div></div><div><h3>Conclusion</h3><div>ACTH treatment demonstrates efficacy in reducing ESES and IED indexes and controlling seizures in children with LKS and ASD. These findings underscore the importance of early intervention and careful management of side effects in optimizing outcomes for these patient populations.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110308"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437131","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2025.110309
Nurul Aisyah Rapai Kasini , Alvin Oliver Payus , Ching Soong Khoo , Hui Jan Tan , Shahizon Azura Mohamed Mukari , Aida-Widure Mustapha Mohd Mustapha , Rozita Hod
Introduction
It can be challenging at times to clinically differentiate between acute seizure and acute stroke. This study aimed to compare the computed tomography perfusion (CTP) changes in patients presenting with acute seizures of different etiologies to those with acute cortical stroke.
Methods
We conducted a single-center cross-sectional case-control study on the CTP changes of 31 patients presenting with acute seizures of different etiologies, and compared them with 31 patients of acute cortical stroke patients (n = 31) as the control.
Results
58.1% had increased time to peak (TTP) in the seizure group. None of the patients with acute stroke had reduced TTP compared to 25.8% in the seizure group. The majority of patients in the seizure group had normal to reduced relative cerebral blood flow (rCBF) (38.7% and 35.5%, respectively). 35.5% and 38.7% of the patients presenting with acute seizures had normal and reduced relative cerebral blood volume (rCBV), respectively. The association between the perfusion parameters and etiology of seizures, presence of underlying epilepsy, or presence of postictal neurological deficits was not statistically significant. The seizure group had cortical ribbon (51.6%), holo-hemispheric (32.3%), multi-lobar (9.7%), and normal (6.5%) CT perfusion patterns.
In conclusion, our study shows that the majority of the patients of the acute seizure group had normal to hypoperfusion patterns. Seizure-related perfusion changes did not conform to expected vascular territories, and cortical ribbon pattern was found to be predominant in our study.
Significance
There is cerebral hypoperfusion or normal perfusion in most patients presenting with acute seizures. Seizure-related perfusion changes are not limited to arterial vascular territories.
{"title":"Determining the computed tomography perfusion changes in patients presenting with acute seizures of different etiologies – A case-control study","authors":"Nurul Aisyah Rapai Kasini , Alvin Oliver Payus , Ching Soong Khoo , Hui Jan Tan , Shahizon Azura Mohamed Mukari , Aida-Widure Mustapha Mohd Mustapha , Rozita Hod","doi":"10.1016/j.yebeh.2025.110309","DOIUrl":"10.1016/j.yebeh.2025.110309","url":null,"abstract":"<div><h3>Introduction</h3><div>It can be challenging at times to clinically differentiate between acute seizure and acute stroke. This study aimed to compare the computed tomography perfusion (CTP) changes in patients presenting with acute seizures of different etiologies to those with acute cortical stroke.</div></div><div><h3>Methods</h3><div>We conducted a single-center cross-sectional case-control study on the CTP changes of 31 patients presenting with acute seizures of different etiologies, and compared them with 31 patients of acute cortical stroke patients (n = 31) as the control.</div></div><div><h3>Results</h3><div>58.1% had increased time to peak (TTP) in the seizure group. None of the patients with acute stroke had reduced TTP compared to 25.8% in the seizure group. The majority of patients in the seizure group had normal to reduced relative cerebral blood flow (rCBF) (38.7% and 35.5%, respectively). 35.5% and 38.7% of the patients presenting with acute seizures had normal and reduced relative cerebral blood volume (rCBV), respectively. The association between the perfusion parameters and etiology of seizures, presence of underlying epilepsy, or presence of postictal neurological deficits was not statistically significant. The seizure group had cortical ribbon (51.6%), holo-hemispheric (32.3%), multi-lobar (9.7%), and normal (6.5%) CT perfusion patterns.</div><div>In conclusion, our study shows that the majority of the patients of the acute seizure group had normal to hypoperfusion patterns. Seizure-related perfusion changes did not conform to expected vascular territories, and cortical ribbon pattern was found to be predominant in our study.</div></div><div><h3>Significance</h3><div>There is cerebral hypoperfusion or normal perfusion in most patients presenting with acute seizures. Seizure-related perfusion changes are not limited to arterial vascular territories.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110309"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143444721","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2025.110313
Victor Rodrigues Santos , Lilian G. Jerow , Candi L. LaSarge
Tuberous sclerosis complex (TSC) is typically associated with epilepsy, but patients also present with a myriad of comorbid neuropsychiatric disorders. TSC is caused by mutations in the tuberous sclerosis complex genes 1 or 2 (TSC1, TSC2). This TSC1/2 complex serves as a negative regulator of the mammalian target of rapamycin (mTOR) signaling pathway, which plays a crucial role in regulating neuronal function, including cell proliferation, survival, growth, and protein synthesis. Mutations result in hyperactivation of the pathway. Animal models with mutations in Tsc1 or Tsc2 consistently exhibit epilepsy and behavioral phenotypes. Additionally, abnormal neuronal populations can impact the broader network, leading to deficits in learning and memory, anxiety-like behaviors, deficits in social behaviors, and perseverative and repetitive behaviors. This review aims to synthesize the existing animal literature linking TSC models to epileptogenesis and behavioral impairments, with insights on how modifications in TSC signaling influence both the structure and function of neurons and behavior. Understanding these relationships may provide valuable insights into potential therapeutic targets for managing epilepsy and neuropsychiatric disorders associated with TSC dysregulation.
{"title":"Behavioral analyses in rodent models of tuberous sclerosis complex","authors":"Victor Rodrigues Santos , Lilian G. Jerow , Candi L. LaSarge","doi":"10.1016/j.yebeh.2025.110313","DOIUrl":"10.1016/j.yebeh.2025.110313","url":null,"abstract":"<div><div>Tuberous sclerosis complex (TSC) is typically associated with epilepsy, but patients also present with a myriad of comorbid neuropsychiatric disorders. TSC is caused by mutations in the tuberous sclerosis complex genes 1 or 2 (<em>TSC1</em>, <em>TSC2</em>). This TSC1/2 complex serves as a negative regulator of the mammalian target of rapamycin (mTOR) signaling pathway, which plays a crucial role in regulating neuronal function, including cell proliferation, survival, growth, and protein synthesis. Mutations result in hyperactivation of the pathway. Animal models with mutations in <em>Tsc1</em> or <em>Tsc2</em> consistently exhibit epilepsy and behavioral phenotypes. Additionally, abnormal neuronal populations can impact the broader network, leading to deficits in learning and memory, anxiety-like behaviors, deficits in social behaviors, and perseverative and repetitive behaviors. This review aims to synthesize the existing animal literature linking TSC models to epileptogenesis and behavioral impairments, with insights on how modifications in TSC signaling influence both the structure and function of neurons and behavior. Understanding these relationships may provide valuable insights into potential therapeutic targets for managing epilepsy and neuropsychiatric disorders associated with TSC dysregulation.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110313"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437138","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-19DOI: 10.1016/j.yebeh.2024.110248
Su-Hyun Han , Hye Jeong Lee , Jung-Kyeom Kim , Hyesung Lee , Seo-Young Lee
Purpose
This study aims to investigate trends in birth and cesarean section (CS) rates among women with epilepsy (WWE) in Korea.
Methods
We conducted a nationwide, population-based, repeated cross-sectional study using data from the Korean National Health Insurance Service database. We evaluated the annual childbirth rate and proportion of CS among all deliveries for WWE and the entire female population aged 15–49 years from 2004 to 2019.
Results
The annual childbirth rates declined more sharply for WWE than those for the general population, with an average annual percent change (AAPC) of −3.5 % for WWE compared to −1.3 % for general women. The CS rate was higher in WWE (51.2 %) than in general population (38.9 %), with increasing trends observed in both groups (AAPC = 2.2 % for WWE vs. AAPC = 1.8 % for general women). Among WWE under monotherapy, without emergency room visits related to epilepsy, and without both central nervous system and psychiatric diseases, the CS rates were 47.8%, 50.6%, and 48.3%, respectively. After adjusting for age and obstetric comorbidities, factors associated with increased CS risk included the use of four or more antiseizure medications (adjusted odds ratio (aOR) 1.74 [1.06–2.87]), emergency room visits (aOR 5.64 [2.83–11.24]), and an Epilepsy-Specific Comorbidity Index of ≥2 (aOR 1.45 [1.05–2.01]).
Conclusions
The annual decline in childbirth and increase in CS rates were more prominent in WWE. While epilepsy severity and comorbidities were associated with CS deliveries, the persistently high CS rates in WWE even under favorable maternal conditions suggest the potential for unnecessary CS procedures.
{"title":"Trends of childbirth and cesarean section among women with epilepsy in Korea","authors":"Su-Hyun Han , Hye Jeong Lee , Jung-Kyeom Kim , Hyesung Lee , Seo-Young Lee","doi":"10.1016/j.yebeh.2024.110248","DOIUrl":"10.1016/j.yebeh.2024.110248","url":null,"abstract":"<div><h3>Purpose</h3><div>This study aims to investigate trends in birth and cesarean section (CS) rates among women with epilepsy (WWE) in Korea.</div></div><div><h3>Methods</h3><div>We conducted a nationwide, population-based, repeated cross-sectional study using data from the Korean National Health Insurance Service database. We evaluated the annual childbirth rate and proportion of CS among all deliveries for WWE and the entire female population aged 15–49 years from 2004 to 2019.</div></div><div><h3>Results</h3><div>The annual childbirth rates declined more sharply for WWE than those for the general population, with an average annual percent change (AAPC) of −3.5 % for WWE compared to −1.3 % for general women. The CS rate was higher in WWE (51.2 %) than in general population (38.9 %), with increasing trends observed in both groups (AAPC = 2.2 % for WWE vs. AAPC = 1.8 % for general women). Among WWE under monotherapy, without emergency room visits related to epilepsy, and without both central nervous system and psychiatric diseases, the CS rates were 47.8%, 50.6%, and 48.3%, respectively. After adjusting for age and obstetric comorbidities, factors associated with increased CS risk included the use of four or more antiseizure medications (adjusted odds ratio (aOR) 1.74 [1.06–2.87]), emergency room visits (aOR 5.64 [2.83–11.24]), and an Epilepsy-Specific Comorbidity Index of ≥2 (aOR 1.45 [1.05–2.01]).</div></div><div><h3>Conclusions</h3><div>The annual decline in childbirth and increase in CS rates were more prominent in WWE. While epilepsy severity and comorbidities were associated with CS deliveries, the persistently high CS rates in WWE even under favorable maternal conditions suggest the potential for unnecessary CS procedures.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"164 ","pages":"Article 110248"},"PeriodicalIF":2.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437698","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-02-18DOI: 10.1016/j.yebeh.2025.110322
Kristien Verhaert , Karolien Persyn , An De Cock , Lieve Troch , Lieven Lagae
Objectives
Cognitive and behavioral comorbidity is frequent in childhood epilepsy and impacts on prognosis and QOL. Comorbidity often precedes seizure onset. Early screening is recommended but no consensus exists on the screening method. The current pilot study investigated the feasibility and validity of a newly developed screening method in children with recently diagnosed epilepsy.
Methods
An online screening method was developed using a combination of existing and validated screening instruments (i.e. 2 standardised questionnaires and 2 psychometric tests), selected to detect the most common comorbid problems in childhood epilepsies. Feasibility was studied using patient and parent questionnaires and drop-out rates. Validity was studied by comparing the screening results to an in-depth diagnostic assessment. Descriptive statistics were used to analyse results.
Results
Out of twenty referred children, 13 entered the study, of whom 1 dropped out (retention rate 93 %). Of those, ten were girls. Most patients were aged 9–12 year (38 %) or 12–15-year (38 %). Eighty-three percent of tested children proved to have cognitive or behavioral comorbidity. Screening results corresponded with diagnostic assessment results in most cases (9 true positives, one true negative), there was 1 false positive and 1 false negative screening result. Sensitivity of the screening amounts to 90 % (CI 73–107).
Conclusions
The current pilot study shows promising results with regards to feasibility and validity of the tested screening method for cognitive and behavioral comorbidity in childhood epilepsy. This warrants further investigation of the method.
{"title":"Screening of cognitive and behavioral comorbidity in children with recently diagnosed epilepsy: A pilot study exploring the feasibility and validity of a newly composed online screening tool","authors":"Kristien Verhaert , Karolien Persyn , An De Cock , Lieve Troch , Lieven Lagae","doi":"10.1016/j.yebeh.2025.110322","DOIUrl":"10.1016/j.yebeh.2025.110322","url":null,"abstract":"<div><h3>Objectives</h3><div>Cognitive and behavioral comorbidity is frequent in childhood epilepsy and impacts on prognosis and QOL. Comorbidity often precedes seizure onset. Early screening is recommended but no consensus exists on the screening method. The current pilot study investigated the feasibility and validity of a newly developed screening method in children with recently diagnosed epilepsy.</div></div><div><h3>Methods</h3><div>An online screening method was developed using a combination of existing and validated screening instruments (i.e. 2 standardised questionnaires and 2 psychometric tests), selected to detect the most common comorbid problems in childhood epilepsies. Feasibility was studied using patient and parent questionnaires and drop-out rates. Validity was studied by comparing the screening results to an in-depth diagnostic assessment. Descriptive statistics were used to analyse results.</div></div><div><h3>Results</h3><div>Out of twenty referred children, 13 entered the study, of whom 1 dropped out (retention rate 93 %). Of those, ten were girls. Most patients were aged 9–12 year (38 %) or 12–15-year (38 %). Eighty-three percent of tested children proved to have cognitive or behavioral comorbidity. Screening results corresponded with diagnostic assessment results in most cases (9 true positives, one true negative), there was 1 false positive and 1 false negative screening result. Sensitivity of the screening amounts to 90 % (CI 73–107).</div></div><div><h3>Conclusions</h3><div>The current pilot study shows promising results with regards to feasibility and validity of the tested screening method for cognitive and behavioral comorbidity in childhood epilepsy. This warrants further investigation of the method.</div></div>","PeriodicalId":11847,"journal":{"name":"Epilepsy & Behavior","volume":"165 ","pages":"Article 110322"},"PeriodicalIF":2.3,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143437137","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
扫码关注我们
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号