Seizure-European Journal of Epilepsy最新文献_第3页

Prognostic performance of STESS and qSOFA scores in pre-hospital status epilepticus: A prospective cohort study 院前癫痫持续状态的ess和qSOFA评分的预后表现：一项前瞻性队列研究

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-29 DOI: 10.1016/j.seizure.2026.01.017

Hela Manai, Teycir Kharraz, Houyem Zouari, Saida Zelfani

Background

Status epilepticus (SE) is a life-threatening, multisystem disorder. Prognostic factors are variable, and several scores have been developed for SE, some requiring electroencephalogram results that may not be available pre-hospital. We aimed to evaluate the prognostic performance of two rapid, easily applicable pre-hospital scores: the STESS and qSOFA scores.

Methods

We conducted a prospective, observational study of SE patients managed in pre-hospital emergency care in Tunis over 35 months. We assessed the area under the receiver operating characteristic curve (AUC), sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and optimal cutoffs for both STESS and qSOFA in predicting 48-hour mortality, in-hospital mortality, and the need for mechanical ventilation.

Results

One hundred SE patients with motor-predominant seizures were included. For 48-hour mortality, AUC was 0.855 for STESS and 0.787 for qSOFA; for mechanical ventilation, AUC was 0.693 and 0.705, respectively. STESS ≥3 predicted 48-hour mortality with specificity 87.0% and sensitivity 66.7%, while qSOFA ≥3 had specificity 95.8% and sensitivity 64.3%. Exploratory analysis for in-hospital mortality (37%) showed STESS ≥3 with specificity 86% and sensitivity 68%, and qSOFA ≥3 with specificity 95% and sensitivity 64%.

Conclusions

STESS and qSOFA are simple, rapid scores suitable for pre-hospital SE management. Both exhibit good specificity but moderate sensitivity. STESS may be more informative for in-hospital outcomes, while qSOFA reflects early systemic severity. Scores should be interpreted alongside a comprehensive clinical assessment to guide triage and early management.

背景：癫痫持续状态（SE）是一种危及生命的多系统疾病。预后因素是可变的，已经为SE制定了几个评分，其中一些需要院前可能无法获得的脑电图结果。我们的目的是评估两种快速、易于应用的院前评分：ess和qSOFA评分的预后表现。方法：我们对突尼斯35个月以上院前急救管理的SE患者进行了前瞻性观察研究。我们评估了受试者工作特征曲线下面积（AUC）、敏感性、特异性、阳性预测值（PPV）、阴性预测值（NPV）以及ess和qSOFA在预测48小时死亡率、住院死亡率和机械通气需求方面的最佳截止点。结果：纳入100例以运动为主的SE患者。对于48小时死亡率，ess的AUC为0.855，qSOFA的AUC为0.787；机械通气的AUC分别为0.693和0.705。ess≥3预测48小时死亡率的特异性为87.0%，敏感性为66.7%；qSOFA≥3预测48小时死亡率的特异性为95.8%，敏感性为64.3%。对住院死亡率（37%）的探索性分析显示，ess≥3，特异性为86%，敏感性为68%；qSOFA≥3，特异性为95%，敏感性为64%。结论：ess和qSOFA是一种简便、快速的评分方法，适用于院前SE管理。两者均表现出良好的特异性，但敏感性中等。ess可能对住院结果提供更多信息，而qSOFA反映早期系统性严重程度。评分应与综合临床评估一起解释，以指导分诊和早期管理。

{"title":"Prognostic performance of STESS and qSOFA scores in pre-hospital status epilepticus: A prospective cohort study","authors":"Hela Manai, Teycir Kharraz, Houyem Zouari, Saida Zelfani","doi":"10.1016/j.seizure.2026.01.017","DOIUrl":"10.1016/j.seizure.2026.01.017","url":null,"abstract":"<div><h3>Background</h3><div>Status epilepticus (SE) is a life-threatening, multisystem disorder. Prognostic factors are variable, and several scores have been developed for SE, some requiring electroencephalogram results that may not be available pre-hospital. We aimed to evaluate the prognostic performance of two rapid, easily applicable pre-hospital scores: the STESS and qSOFA scores.</div></div><div><h3>Methods</h3><div>We conducted a prospective, observational study of SE patients managed in pre-hospital emergency care in Tunis over 35 months. We assessed the area under the receiver operating characteristic curve (AUC), sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and optimal cutoffs for both STESS and qSOFA in predicting 48-hour mortality, in-hospital mortality, and the need for mechanical ventilation.</div></div><div><h3>Results</h3><div>One hundred SE patients with motor-predominant seizures were included. For 48-hour mortality, AUC was 0.855 for STESS and 0.787 for qSOFA; for mechanical ventilation, AUC was 0.693 and 0.705, respectively. STESS ≥3 predicted 48-hour mortality with specificity 87.0% and sensitivity 66.7%, while qSOFA ≥3 had specificity 95.8% and sensitivity 64.3%. Exploratory analysis for in-hospital mortality (37%) showed STESS ≥3 with specificity 86% and sensitivity 68%, and qSOFA ≥3 with specificity 95% and sensitivity 64%.</div></div><div><h3>Conclusions</h3><div>STESS and qSOFA are simple, rapid scores suitable for pre-hospital SE management. Both exhibit good specificity but moderate sensitivity. STESS may be more informative for in-hospital outcomes, while qSOFA reflects early systemic severity. Scores should be interpreted alongside a comprehensive clinical assessment to guide triage and early management.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"136 ","pages":"Pages 46-50"},"PeriodicalIF":2.8,"publicationDate":"2026-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146183156","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}

引用次数: 0

Clinical reliability and concordance of drug-drug interaction tools as clinical decision support systems in patients with epilepsy 药物-药物相互作用工具作为癫痫患者临床决策支持系统的临床可靠性和一致性。

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-23 DOI: 10.1016/j.seizure.2026.01.011

Maria Krayem , Milena van der Goten , Adam Strzelczyk , Felix Rosenow , Felix von Podewils , Susanne Knake , Stjepana Kovac , Lisa Langenbruch , Catrin Mann , Johann Philipp Zöllner , Laurent M. Willems

Purpose

To evaluate the concordance and clinical correlation of drug-drug interaction tools (DDITs) as clinical decision support systems (CDSS) in patients with epilepsy.

Methods

All patients from the Epi2020 study cohort receiving ≥1 anti-seizure medication (ASM) and ≥1 concomitant drug (CD) were included. Individual treatment regimens were analyzed for drug-drug interactions (DDIs) using five DDITs (Drugbank, Drugs.com, mediQ, Medscape and WebMD). To address the clinical significance of possible DDIs, Spearman correlation between the number of detected DDIs and two established adverse event (AE) metrics (LAEP, QOLIE-31) was performed using post-hoc correction by Fisher’s z-transformation (FZT) for the total number of drugs taken. Multivariate ordinal regression analysis (MORA) was performed to identify ASM or CD classes associated with severe DDIs.

Results

Overall, 140 patients (57.9% female, median age 48 years) taking a median number of 4.0 drugs (2.0 ASMs and 2.0 CDs) were included. DDI were found in 51.4%–84.3% and severe interactions in 2.1%–17.8% of patients, depending on the DDIT. The concordance rate between DDITs was only 6.4% for all and only 63.6% for severe detected DDI, respectively. All concordant cases involved no detected interactions. The number of DDIs significantly correlated with AE metrics in 3/5 DDITs. Following the FZT, none of the DDI/AE correlations were superior to that between the number of DDIs and the number of drugs taken. MORA identified topiramate, valproate, zonisamide, hormones, and antipsychotics as independent predictors for the detection of severe DDIs.

Conclusion

When using DDITs as CDSS, it is important to consider that the results of different tools may vary greatly from one another and do not necessarily correlate with clinical AEs.

Study registration

The Epi2020 study was registered under the trial registration number: DRKS00022024, U1111-1252-5331.

目的：评价药物-药物相互作用工具（dddits）作为癫痫患者临床决策支持系统（CDSS）的一致性和临床相关性。方法：纳入Epi2020研究队列中所有接受≥1种抗癫痫药物（ASM）和≥1种伴随药物（CD）的患者。使用5个ddi （Drugbank、Drugs.com、mediQ、Medscape和WebMD）分析个别治疗方案的药物-药物相互作用（ddi）。为了解决可能的ddi的临床意义，检测到的ddi数量与两个既定的不良事件（AE）指标（LAEP, QOLIE-31）之间的Spearman相关性使用Fisher z变换（FZT）对所服用药物总数进行事后校正。采用多变量有序回归分析（MORA）确定与严重ddi相关的ASM或CD类别。结果：共纳入140例患者（女性57.9%，中位年龄48岁），中位用药数4.0种（asm 2.0, cd 2.0）。DDI发生率为51.4% ~ 84.3%，严重相互作用发生率为2.1% ~ 17.8%，取决于DDI。所有DDI的符合率仅为6.4%，严重DDI的符合率仅为63.6%。所有病例均未检测到相互作用。在3/5的ddi中，ddi的数量与AE指标显著相关。在FZT之后，DDI/AE的相关性并不优于DDI次数与服药次数之间的相关性。MORA发现托吡酯、丙戊酸、唑尼沙胺、激素和抗精神病药物是检测严重ddi的独立预测因子。结论：当使用ddi作为CDSS时，重要的是要考虑到不同工具的结果可能彼此差异很大，并且不一定与临床ae相关。研究注册：Epi2020研究注册，试验注册号：DRKS00022024， U1111-1252-5331。

{"title":"Clinical reliability and concordance of drug-drug interaction tools as clinical decision support systems in patients with epilepsy","authors":"Maria Krayem , Milena van der Goten , Adam Strzelczyk , Felix Rosenow , Felix von Podewils , Susanne Knake , Stjepana Kovac , Lisa Langenbruch , Catrin Mann , Johann Philipp Zöllner , Laurent M. Willems","doi":"10.1016/j.seizure.2026.01.011","DOIUrl":"10.1016/j.seizure.2026.01.011","url":null,"abstract":"<div><h3>Purpose</h3><div>To evaluate the concordance and clinical correlation of drug-drug interaction tools (DDITs) as clinical decision support systems (CDSS) in patients with epilepsy.</div></div><div><h3>Methods</h3><div>All patients from the Epi2020 study cohort receiving ≥1 anti-seizure medication (ASM) and ≥1 concomitant drug (CD) were included. Individual treatment regimens were analyzed for drug-drug interactions (DDIs) using five DDITs (Drugbank, Drugs.com, mediQ, Medscape and WebMD). To address the clinical significance of possible DDIs, Spearman correlation between the number of detected DDIs and two established adverse event (AE) metrics (LAEP, QOLIE-31) was performed using post-hoc correction by Fisher’s z-transformation (FZT) for the total number of drugs taken. Multivariate ordinal regression analysis (MORA) was performed to identify ASM or CD classes associated with severe DDIs.</div></div><div><h3>Results</h3><div>Overall, 140 patients (57.9% female, median age 48 years) taking a median number of 4.0 drugs (2.0 ASMs and 2.0 CDs) were included. DDI were found in 51.4%–84.3% and severe interactions in 2.1%–17.8% of patients, depending on the DDIT. The concordance rate between DDITs was only 6.4% for all and only 63.6% for severe detected DDI, respectively. All concordant cases involved no detected interactions. The number of DDIs significantly correlated with AE metrics in 3/5 DDITs. Following the FZT, none of the DDI/AE correlations were superior to that between the number of DDIs and the number of drugs taken. MORA identified topiramate, valproate, zonisamide, hormones, and antipsychotics as independent predictors for the detection of severe DDIs.</div></div><div><h3>Conclusion</h3><div>When using DDITs as CDSS, it is important to consider that the results of different tools may vary greatly from one another and do not necessarily correlate with clinical AEs.</div></div><div><h3>Study registration</h3><div>The Epi2020 study was registered under the trial registration number: DRKS00022024, U1111-1252-5331.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"136 ","pages":"Pages 31-39"},"PeriodicalIF":2.8,"publicationDate":"2026-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146167752","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}

引用次数: 0

Neonatal-onset epileptic encephalopathy with lissencephaly associated with a SCN3A variant: The first case in Korea and literature review 新生儿癫痫性脑病伴无脑畸形与SCN3A变异相关：韩国首例病例和文献综述

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-18 DOI: 10.1016/j.seizure.2026.01.008

Hyun A Lee , Seong Wan Kim , Seoheui Choi , Jang Hoon Lee , Moon Sung Park , Rita Yu , Yoong-A Suh

Purpose

Pathogenic variants inSCN3A, encoding the voltage-gated sodium channel Naᵥ1.3, have been implicated in early infantile epileptic encephalopathy (EIEE) and cortical malformations. We report the first Korean case of SCN3A-related EIEE and discuss its contribution to the expanding phenotypic spectrum.

Methods

Clinical features, electroencephalography (EEG), brain magnetic resonance imaging (MRI), treatment response, and outcomes were analyzed. A literature review of previously reported SCN3A cases was performed for phenotypic comparison.

Results

A female neonate developed tonic seizures 7 h after birth. EEG showed multifocal epileptiform discharges with burst-suppression patterns. Brain MRI demonstrated diffuse cortical thickening consistent with the lissencephaly–pachygyria spectrum and corpus callosum dysgenesis. Despite multiple antiseizure medications, seizures remained intractable. Profound bulbar dysfunction required gastrostomy, tracheostomy, and home ventilation. Next-generation sequencing identified a heterozygous SCN3A c.2624T>C (p.Ile875Thr) variant, previously reported as pathogenic and associated with gain-of-function effects. The patient died at 6 months of age.

Conclusion

This first Korean case of SCN3A-related EIEE caused by the recurrent p.Ile875Thr variant highlights a severe neonatal phenotype characterized by early-onset refractory seizures, profound cortical malformations, and early mortality. The case broadens both the phenotypic and geographic spectrum of SCN3A-associated neurodevelopmental disorders and underscores the importance of early genetic testing in neonates with refractory seizures and cortical malformations.

目的：编码电压门控钠通道Naᵥ1.3的scn3a致病变异与早期婴儿癫痫性脑病（EIEE）和皮质畸形有关。我们报告了韩国首例scn3a相关eee病例，并讨论了其对扩大表型谱的贡献。方法：分析患者的临床特点、脑电图（EEG）、脑磁共振成像（MRI）、治疗效果及转诊结果。对先前报道的SCN3A病例进行文献回顾，进行表型比较。结果：1例女性新生儿在出生后7 h出现强直性癫痫发作。脑电图显示多灶性癫痫样放电，伴有发作抑制模式。脑MRI显示弥漫性皮质增厚，与无脑-厚回症频谱和胼胝体发育不良一致。尽管有多种抗癫痫药物，但癫痫仍然难以治疗。严重的球功能障碍需要胃造口术、气管造口术和家庭通气。新一代测序鉴定出一种杂合子SCN3A C . 2624t >C （p.i ile875thr）变异，该变异先前被报道为致病性并与功能获得效应相关。患者在6个月大时死亡。结论：这是韩国首例由p.i ile875thr复发性变异体引起的scn3a相关eee病例，突出了一种严重的新生儿表型，其特征是早发性难固性癫痫发作、深度皮质畸形和早期死亡。该病例拓宽了scn3a相关神经发育障碍的表型和地理谱，并强调了对难治性癫痫和皮质畸形的新生儿进行早期基因检测的重要性。

{"title":"Neonatal-onset epileptic encephalopathy with lissencephaly associated with a SCN3A variant: The first case in Korea and literature review","authors":"Hyun A Lee , Seong Wan Kim , Seoheui Choi , Jang Hoon Lee , Moon Sung Park , Rita Yu , Yoong-A Suh","doi":"10.1016/j.seizure.2026.01.008","DOIUrl":"10.1016/j.seizure.2026.01.008","url":null,"abstract":"<div><h3>Purpose</h3><div>Pathogenic variants in<em>SCN3A,</em> encoding the voltage-gated sodium channel Naᵥ1.3, have been implicated in early infantile epileptic encephalopathy (EIEE) and cortical malformations. We report the first Korean case of <em>SCN3A</em>-related EIEE and discuss its contribution to the expanding phenotypic spectrum.</div></div><div><h3>Methods</h3><div>Clinical features, electroencephalography (EEG), brain magnetic resonance imaging (MRI), treatment response, and outcomes were analyzed. A literature review of previously reported <em>SCN3A</em> cases was performed for phenotypic comparison.</div></div><div><h3>Results</h3><div>A female neonate developed tonic seizures 7 h after birth. EEG showed multifocal epileptiform discharges with burst-suppression patterns. Brain MRI demonstrated diffuse cortical thickening consistent with the lissencephaly–pachygyria spectrum and corpus callosum dysgenesis. Despite multiple antiseizure medications, seizures remained intractable. Profound bulbar dysfunction required gastrostomy, tracheostomy, and home ventilation. Next-generation sequencing identified a heterozygous <em>SCN3A</em> c.2624T>C (p.Ile875Thr) variant, previously reported as pathogenic and associated with gain-of-function effects. The patient died at 6 months of age.</div></div><div><h3>Conclusion</h3><div>This first Korean case of <em>SCN3A</em>-related EIEE caused by the recurrent p.Ile875Thr variant highlights a severe neonatal phenotype characterized by early-onset refractory seizures, profound cortical malformations, and early mortality. The case broadens both the phenotypic and geographic spectrum of <em>SCN3A</em>-associated neurodevelopmental disorders and underscores the importance of early genetic testing in neonates with refractory seizures and cortical malformations.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"136 ","pages":"Pages 13-17"},"PeriodicalIF":2.8,"publicationDate":"2026-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146127093","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}

引用次数: 0

Epilepsy phenotypes of Renu syndrome: Novel insights from a European multicentre retrospective cohort study Renu综合征的癫痫表型：来自欧洲多中心回顾性队列研究的新见解

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-17 DOI: 10.1016/j.seizure.2026.01.010

Mario Mastrangelo , Manuela Tolve , Irene Valenzuela , Giuliana Lentini , Elisa Maria Colacino Cinnante , Barbara Masotto , Stefano D’ Arrigo , Paola Francesca Ajmone , Jessica Rosenblum , Donatella Milani , Agusti Rodriguez-Palmero , Claudia Ciaccio , Anna C. Jansen , Francesco Pisani

Background

Epilepsy is a prominent feature in about 60% of patients with ReNU syndrome

Patients and methods

Data including demographics, gene variants, seizure semiology and evolution, EEG patterns, neurodevelopmental features, MRI characteristics and response to antiseizure medications were retrospectively collected in a cohort of patients with ReNU syndrome referred to 6 European tertiary centres.

Results

The cohort included 10 patients (7 males and 3 females). The mean age at epilepsy onset was 2.8 ±2.1 years. No specific epilepsy syndromes were recognized. Focal impaired consciousness with observable manifestations (with onset before 12 months in 3 cases) were the predominant seizure type across all age ranges. The frequency of this seizure type peaked between the ages of 6 and 11 years. Generalized seizures were less common and mainly occurred under the age of 6 with a similar frequency of atypical absences and tonic/tonic clonic seizures. Interictal and ictal epileptiform EEG were more frequently detected after 3 years of age and were mainly focal (with predominant involvement of the fronto-parietal regions). No structural epileptogenic lesions were detected on MRI. Seizure freedom was achieved with antiseizure medications in 5 patients at a mean age of 7.2 ± 25.2. Valproate was judged the most effective medication by referring neurologists followed by levetiracetam, clobazam, lamotrigine and phenytoin. No relevant genotype-phenotype correlations were observed.

Conclusions

The phenotype of the seizure disorders observed in this cohort was dominated by focal impaired consciousness seizures with observable manifestations and a relatively favourable course of epilepsy.

背景：癫痫是约60%的ReNU综合征患者的一个突出特征。患者和方法回顾性收集了6个欧洲三级中心的ReNU综合征患者队列的人口统计学、基因变异、癫痫符号学和进化、脑电图模式、神经发育特征、MRI特征和抗癫痫药物反应等数据。结果共纳入10例患者，其中男7例，女3例。癫痫发作的平均年龄为2.8±2.1岁。未发现特定的癫痫综合征。有明显表现的局灶性意识受损（3例在12个月前发病）是所有年龄段的主要癫痫类型。这种癫痫发作的频率在6岁到11岁之间达到高峰。全身性癫痫发作较少见，主要发生在6岁以下，非典型缺席和强直/强直阵挛发作的频率相似。发作期和发作期癫痫样脑电图多见于3岁以后，且主要为局灶性脑电图（主要累及额顶叶区）。MRI未见结构性癫痫病变。5例患者通过抗癫痫药物治疗获得癫痫发作自由，平均年龄为7.2±25.2岁。以丙戊酸钠最为有效，其次为左乙拉西坦、氯巴赞、拉莫三嗪和苯妥英。未观察到相关的基因型-表型相关性。结论本队列中观察到的癫痫疾病表型以局灶性意识障碍发作为主，具有明显的症状和相对有利的癫痫病程。

{"title":"Epilepsy phenotypes of Renu syndrome: Novel insights from a European multicentre retrospective cohort study","authors":"Mario Mastrangelo , Manuela Tolve , Irene Valenzuela , Giuliana Lentini , Elisa Maria Colacino Cinnante , Barbara Masotto , Stefano D’ Arrigo , Paola Francesca Ajmone , Jessica Rosenblum , Donatella Milani , Agusti Rodriguez-Palmero , Claudia Ciaccio , Anna C. Jansen , Francesco Pisani","doi":"10.1016/j.seizure.2026.01.010","DOIUrl":"10.1016/j.seizure.2026.01.010","url":null,"abstract":"<div><h3>Background</h3><div>Epilepsy is a prominent feature in about 60% of patients with ReNU syndrome</div></div><div><h3>Patients and methods</h3><div>Data including demographics, gene variants, seizure semiology and evolution, EEG patterns, neurodevelopmental features, MRI characteristics and response to antiseizure medications were retrospectively collected in a cohort of patients with ReNU syndrome referred to 6 European tertiary centres.</div></div><div><h3>Results</h3><div>The cohort included 10 patients (7 males and 3 females). The mean age at epilepsy onset was 2.8 ±2.1 years. No specific epilepsy syndromes were recognized. Focal impaired consciousness with observable manifestations (with onset before 12 months in 3 cases) were the predominant seizure type across all age ranges. The frequency of this seizure type peaked between the ages of 6 and 11 years. Generalized seizures were less common and mainly occurred under the age of 6 with a similar frequency of atypical absences and tonic/tonic clonic seizures. Interictal and ictal epileptiform EEG were more frequently detected after 3 years of age and were mainly focal (with predominant involvement of the fronto-parietal regions). No structural epileptogenic lesions were detected on MRI. Seizure freedom was achieved with antiseizure medications in 5 patients at a mean age of 7.2 ± 25.2. Valproate was judged the most effective medication by referring neurologists followed by levetiracetam, clobazam, lamotrigine and phenytoin. No relevant genotype-phenotype correlations were observed.</div></div><div><h3>Conclusions</h3><div>The phenotype of the seizure disorders observed in this cohort was dominated by focal impaired consciousness seizures with observable manifestations and a relatively favourable course of epilepsy.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 46-55"},"PeriodicalIF":2.8,"publicationDate":"2026-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023817","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}

引用次数: 0

Automatic recognition of epileptic spasm via large-scale visual AI model 基于大规模视觉AI模型的癫痫发作自动识别。

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-17 DOI: 10.1016/j.seizure.2026.01.009

Jialu Xu , Chenyu Yan , Xiaoyan Shen , Tiejia Jiang , Wencong Ruan , Haifeng Li , Ning Ma

Purpose

To develop effective visual AI recognition models for epileptic spasm (ES), and to promote the professionalism and convenience of ES detection.

Method

We collected about 330 hours of infant motion videos with epileptic spasm in Children's Hospital, Zhejiang University School of Medicine from November 2022 to October 2024. A video-centered AI model was constructed, with a pre-trained Vision Transformer (ViT) via Contrastive Language-Image Pre-training (CLIP), serving as the core to extract spatial features. Additionally, a temporal convolution module was integrated to extract temporal information and a multi-layer perceptron was used to perform a normal-abnormal binary classification task. Focal loss was applied to mitigate class imbalance, prioritizing the learning of hard-to-classify samples. The model was trained for 100 epochs with 5-times random dataset splitting, in which the dataset was partitioned by individual infants to ensure disjoint training and test sets. Model performance was validated using metrics (including Precision, Recall, F-score, Accuracy, AUROC) based on test set results.

Results

The median age of ES onset was 0.4 (0.3, 0.7) years. All patients exhibited isolated or clustered epileptic spasms. By employing a CLIP-based classifier, the system reached a recall rate of 1.00 ± 0.00, a precision of 0.78 ± 0.01, an F-score of 0.87 ± 0.01, an accuracy of 0.98 ± 0.01, and an AUROC of 0.99 ± 0.01 in detecting epileptic spasm — outperforming previously reported methods. Case studies involving four infants’ motion videos showed a high degree of consistency between the model’s predictions and expert annotations. The model effectively distinguished ES episodes from normal patterns, even in videos with multiple intermittent ES segments.

Conclusion

We developed an automatic motion recognition model that holds significant potential in early automated detection of ES.

目的：建立有效的癫痫性痉挛视觉人工智能识别模型，提高癫痫性痉挛检测的专业性和便捷性。方法：收集浙江大学医学院附属儿童医院于2022年11月至2024年10月收治的癫痫性痉挛患儿运动视频约330小时。构建以视频为中心的人工智能模型，以对比语言图像预训练（CLIP）预训练的视觉变换（ViT）为核心提取空间特征。此外，还集成了时间卷积模块来提取时间信息，并使用多层感知器来执行正常-异常二值分类任务。应用焦点损失来缓解类不平衡，优先学习难以分类的样本。采用5次随机数据分割的方法对模型进行100次epoch的训练，其中数据集由单个婴儿进行分割，以保证训练集和测试集不相交。使用基于测试集结果的指标（包括Precision， Recall, F-score, Accuracy， AUROC）验证模型性能。结果：ES发病的中位年龄为0.4（0.3,0.7）岁。所有患者均表现出孤立或聚集性癫痫痉挛。采用基于clip的分类器，系统检测癫痫痉挛的召回率为1.00±0.00，精密度为0.78±0.01，f值为0.87±0.01，准确率为0.98±0.01，AUROC为0.99±0.01，优于已有报道的方法。涉及四个婴儿运动视频的案例研究表明，该模型的预测与专家注释之间高度一致。该模型有效地将ES片段与正常模式区分开来，即使在具有多个间歇性ES片段的视频中也是如此。结论：我们开发的自动运动识别模型在ES的早期自动检测中具有重要的潜力。

{"title":"Automatic recognition of epileptic spasm via large-scale visual AI model","authors":"Jialu Xu , Chenyu Yan , Xiaoyan Shen , Tiejia Jiang , Wencong Ruan , Haifeng Li , Ning Ma","doi":"10.1016/j.seizure.2026.01.009","DOIUrl":"10.1016/j.seizure.2026.01.009","url":null,"abstract":"<div><h3>Purpose</h3><div>To develop effective visual AI recognition models for epileptic spasm (ES), and to promote the professionalism and convenience of ES detection.</div></div><div><h3>Method</h3><div>We collected about 330 hours of infant motion videos with epileptic spasm in Children's Hospital, Zhejiang University School of Medicine from November 2022 to October 2024. A video-centered AI model was constructed, with a pre-trained Vision Transformer (ViT) via Contrastive Language-Image Pre-training (CLIP), serving as the core to extract spatial features. Additionally, a temporal convolution module was integrated to extract temporal information and a multi-layer perceptron was used to perform a normal-abnormal binary classification task. Focal loss was applied to mitigate class imbalance, prioritizing the learning of hard-to-classify samples. The model was trained for 100 epochs with 5-times random dataset splitting, in which the dataset was partitioned by individual infants to ensure disjoint training and test sets. Model performance was validated using metrics (including Precision, Recall, F-score, Accuracy, AUROC) based on test set results.</div></div><div><h3>Results</h3><div>The median age of ES onset was 0.4 (0.3, 0.7) years. All patients exhibited isolated or clustered epileptic spasms. By employing a CLIP-based classifier, the system reached a recall rate of 1.00 ± 0.00, a precision of 0.78 ± 0.01, an F-score of 0.87 ± 0.01, an accuracy of 0.98 ± 0.01, and an AUROC of 0.99 ± 0.01 in detecting epileptic spasm — outperforming previously reported methods. Case studies involving four infants’ motion videos showed a high degree of consistency between the model’s predictions and expert annotations. The model effectively distinguished ES episodes from normal patterns, even in videos with multiple intermittent ES segments.</div></div><div><h3>Conclusion</h3><div>We developed an automatic motion recognition model that holds significant potential in early automated detection of ES.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"136 ","pages":"Pages 23-30"},"PeriodicalIF":2.8,"publicationDate":"2026-01-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146144495","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}

引用次数: 0

An e-Delphi approach to develop a patient-reported outcome measure to assess functional/dissociative seizures severity e-Delphi方法开发患者报告的结果测量来评估功能性/解离性癫痫发作的严重程度。

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-15 DOI: 10.1016/j.seizure.2025.12.015

Gregg Harry Rawlings , Laura Whitaker , Chris Gaskell , Markus Reuber

Objectives

Functional / dissociative seizures (FDS) are a debilitating condition for which there are no validated, reliable, or co-produced condition-specific severity measures. Such a measure would be of value for clinical and research applications. Here, we conduct a three-round electronic-Delphi survey with the aim of achieving consensus on questions that could be used to assess FDS severity as part of a patient reported outcome measure.

Methods

e-Delphi members consisted of individuals living with FND, professional experts in the condition (e.g., healthcare providers, researchers), and nonprofessional caregivers. Participants were recruited via international organisations linked with FND. The purpose and tasks of each round were iteratively developed and based on participants’ responses to the previous round.

Results

In total, 90 people participated in round one (54 individuals with FDS, 32 professionals and 4 nonprofessional cares). This reduced to 67 and 55 in rounds two and three, respectively. Overall, 136 candidate items proposed by the current authors or participants were initially considered for inclusion. The final measure consisted of three sections with 29 items assessing severity of FDS, six exploring frequency and duration of seizures, and a checklist of symptoms commonly associated with FDS. 90% of those in round three were “happy” with the proposed measure.

Conclusions

Notwithstanding the challenges of operationalising severity and the inter- and intra-individual variability of the condition, consensus on items to include was achieved by experts by experience or training. Future scale development will evaluate the proposed measure prior to its implementation in clinical and research practice.

目的：功能性/解离性癫痫发作（FDS）是一种衰弱性疾病，没有经过验证的、可靠的或共同产生的疾病特定的严重程度措施。这种测量方法对临床和研究应用具有一定的价值。在这里，我们进行了三轮电子德尔菲调查，目的是就可用于评估FDS严重程度的问题达成共识，作为患者报告结果测量的一部分。方法：e-Delphi成员包括患有FND的个体、该疾病的专业专家（如医疗保健提供者、研究人员）和非专业护理人员。参与者是通过与FND有联系的国际组织招募的。每一轮的目的和任务都是根据参与者对前一轮的反应迭代开发的。结果：共有90人参加了第一轮（54名FDS患者，32名专业护理人员和4名非专业护理人员）。第二轮和第三轮分别减少到67和55。总的来说，目前的作者或参与者提出的136个候选项目最初被考虑纳入。最后的测量包括三个部分，29个项目评估FDS的严重程度，6个项目探索癫痫发作的频率和持续时间，以及一个与FDS相关的症状清单。在第三轮投票中，90%的人对提议的措施表示“满意”。结论：尽管存在操作的严重度以及个体间和个体内部条件的可变性方面的挑战，专家通过经验或培训达成了关于纳入项目的共识。未来的规模开发将在其在临床和研究实践中实施之前评估所提议的措施。

{"title":"An e-Delphi approach to develop a patient-reported outcome measure to assess functional/dissociative seizures severity","authors":"Gregg Harry Rawlings , Laura Whitaker , Chris Gaskell , Markus Reuber","doi":"10.1016/j.seizure.2025.12.015","DOIUrl":"10.1016/j.seizure.2025.12.015","url":null,"abstract":"<div><h3>Objectives</h3><div>Functional / dissociative seizures (FDS) are a debilitating condition for which there are no validated, reliable, or co-produced condition-specific severity measures. Such a measure would be of value for clinical and research applications. Here, we conduct a three-round electronic-Delphi survey with the aim of achieving consensus on questions that could be used to assess FDS severity as part of a patient reported outcome measure.</div></div><div><h3>Methods</h3><div>e-Delphi members consisted of individuals living with FND, professional experts in the condition (e.g., healthcare providers, researchers), and nonprofessional caregivers. Participants were recruited via international organisations linked with FND. The purpose and tasks of each round were iteratively developed and based on participants’ responses to the previous round.</div></div><div><h3>Results</h3><div>In total, 90 people participated in round one (54 individuals with FDS, 32 professionals and 4 nonprofessional cares). This reduced to 67 and 55 in rounds two and three, respectively. Overall, 136 candidate items proposed by the current authors or participants were initially considered for inclusion. The final measure consisted of three sections with 29 items assessing severity of FDS, six exploring frequency and duration of seizures, and a checklist of symptoms commonly associated with FDS. 90% of those in round three were “happy” with the proposed measure.</div></div><div><h3>Conclusions</h3><div>Notwithstanding the challenges of operationalising severity and the inter- and intra-individual variability of the condition, consensus on items to include was achieved by experts by experience or training. Future scale development will evaluate the proposed measure prior to its implementation in clinical and research practice.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 19-27"},"PeriodicalIF":2.8,"publicationDate":"2026-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146012938","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}

引用次数: 0

Evolution into spike-and-wave activation in sleep in patients with self-limited focal epilepsies 自限性局灶性癫痫患者睡眠中向峰波激活的演变

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-08 DOI: 10.1016/j.seizure.2026.01.006

Merve İriş, Miray Atacan Yaşgüçlükal, Cengiz Yalçınkaya, Veysi Demirbilek

Purposes

Self-limited focal epilepsies of childhood (SeLFE), while predominantly considered benign, are known to potentially manifest with spike-and-wave activation in sleep (SWAS) in a minority of patients

Methods

The medical records of individuals diagnosed with one of the SeLFE syndromes according to the ILAE 2022 diagnostic criteria, who were followed in our center between 1989–2023, were retrospectively analyzed. At least two awake and sleep EEGs were performed during a minimum 2-year follow-up. SWAS is considered as spike and wave discharges occupying ≥50% of NREM sleep with symmetrical or mildly asymmetrical bilateral or unilateral hemispheric distribution.

Results

Among 144 patients with SeLFE, 57(39.6%) were diagnosed with self-limited epilepsy with centrotemporal spikes (SeLECTS); 65(45.1%) with self-limited epilepsy with autonomic seizures (SeLEAS); and 22 (15.3%) with childhood occipital visual epilepsy (COVE). The mean age of seizure onset was 7.6, 5.6, and 8.5 years, respectively. Twelve (8.3%) evolved into SWAS (5 from SeLECTS, 6 from SeLEAS, 1 from COVE). Time elapsed between onset of first seizure and evolution into SWAS ranged from 5.2 to 75 months (mean: 26.8±19.8), 6.2–42.8 months (mean: 20.1±14.7 for patients with SeLECTS; 5.2–75.0 months (mean: 32.7±24.5) with SeLEAS, and 25.0 months with COVE). All except two patients had also cognitive or behavioral regression and were diagnosed as epileptic encephalopathy with spike-wave activation in sleep(EE-SWAS) and one patient was diagnosed with Landau–Kleffner syndrome.

Conclusions

The most recent definition of ILAE highlights that SeLFEs are no longer recognized as “benign” epilepsies. Even with a low incidence rate, clinicians should always be cautious about the risk of SWAS development in these syndromes.

目的儿童自限性局灶性癫痫（SeLFE），虽然主要被认为是良性的，但已知在少数患者中可能表现为睡眠中的峰波激活（SWAS）。方法回顾性分析1989-2023年间在本中心随访的根据ILAE 2022诊断标准诊断为其中一种SeLFE综合征的个体的病历。在至少2年的随访期间，至少进行了两次清醒和睡眠脑电图检查。SWAS被认为是峰值和波放电占据NREM睡眠的50%以上，具有对称或轻度不对称的双侧或单侧半球分布。结果144例SeLFE患者中，57例（39.6%）诊断为伴中央颞叶尖峰（SeLECTS）的自限性癫痫；自限性癫痫伴自主神经发作（SeLEAS） 65例（45.1%）；儿童枕部视觉癫痫22例（15.3%）。癫痫发作的平均年龄分别为7.6岁、5.6岁和8.5岁。12例（8.3%）进化为SWAS（5例来自SeLECTS， 6例来自SeLEAS， 1例来自COVE）。从首次发作到发展为SWAS的时间范围为5.2至75个月（平均：26.8±19.8），选择组为6.2至42.8个月（平均：20.1±14.7），SeLEAS组为5.2至75.0个月（平均：32.7±24.5），COVE组为25.0个月)。除2例患者外，其余患者均有认知或行为倒退，并被诊断为癫痫性脑病伴睡眠尖波激活（EE-SWAS）， 1例患者被诊断为Landau-Kleffner综合征。ILAE的最新定义强调，self不再被认为是“良性”癫痫。即使发病率较低，临床医生也应始终警惕SWAS在这些综合征中发展的风险。

{"title":"Evolution into spike-and-wave activation in sleep in patients with self-limited focal epilepsies","authors":"Merve İriş, Miray Atacan Yaşgüçlükal, Cengiz Yalçınkaya, Veysi Demirbilek","doi":"10.1016/j.seizure.2026.01.006","DOIUrl":"10.1016/j.seizure.2026.01.006","url":null,"abstract":"<div><h3>Purposes</h3><div>Self-limited focal epilepsies of childhood (SeLFE), while predominantly considered benign, are known to potentially manifest with spike-and-wave activation in sleep (SWAS) in a minority of patients</div></div><div><h3>Methods</h3><div>The medical records of individuals diagnosed with one of the SeLFE syndromes according to the ILAE 2022 diagnostic criteria, who were followed in our center between 1989–2023, were retrospectively analyzed. At least two awake and sleep EEGs were performed during a minimum 2-year follow-up. SWAS is considered as spike and wave discharges occupying ≥50% of NREM sleep with symmetrical or mildly asymmetrical bilateral or unilateral hemispheric distribution.</div></div><div><h3>Results</h3><div>Among 144 patients with SeLFE, 57(39.6%) were diagnosed with self-limited epilepsy with centrotemporal spikes (SeLECTS); 65(45.1%) with self-limited epilepsy with autonomic seizures (SeLEAS); and 22 (15.3%) with childhood occipital visual epilepsy (COVE). The mean age of seizure onset was 7.6, 5.6, and 8.5 years, respectively. Twelve (8.3%) evolved into SWAS (5 from SeLECTS, 6 from SeLEAS, 1 from COVE). Time elapsed between onset of first seizure and evolution into SWAS ranged from 5.2 to 75 months (mean: 26.8±19.8), 6.2–42.8 months (mean: 20.1±14.7 for patients with SeLECTS; 5.2–75.0 months (mean: 32.7±24.5) with SeLEAS, and 25.0 months with COVE). All except two patients had also cognitive or behavioral regression and were diagnosed as epileptic encephalopathy with spike-wave activation in sleep(EE-SWAS) and one patient was diagnosed with Landau–Kleffner syndrome.</div></div><div><h3>Conclusions</h3><div>The most recent definition of ILAE highlights that SeLFEs are no longer recognized as “benign” epilepsies. Even with a low incidence rate, clinicians should always be cautious about the risk of SWAS development in these syndromes.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 34-38"},"PeriodicalIF":2.8,"publicationDate":"2026-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023818","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}

引用次数: 0

Superior localizing and prognostic value of statistical parametric SPECT analysis in temporal lobe epilepsy: A comparative study of ISAS and SISCOM 统计参数SPECT分析在颞叶癫痫中的优越定位和预后价值：ISAS和SISCOM的比较研究

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-07 DOI: 10.1016/j.seizure.2026.01.001

Jeongsik Kim , Seung Hwan Moon , Hea Ree Park , Eun Yeon Joo , Dae-Won Seo , Young-Min Shon

Purpose

This study aimed to compare the localizing accuracy, interobserver reliability, and prognostic value of ictal SPECT analyzed by Statistical Parametric Mapping (ISAS) versus Subtraction Ictal SPECT Co-registered to MRI (SISCOM) in patients with drug-resistant temporal lobe epilepsy (TLE).

Methods

Sixty consecutive TLE patients who underwent resective epilepsy surgery were analyzed. All ictal SPECT studies were processed by ISAS and SISCOM. Two blinded reviewers independently identified the most prominent hyperperfusion regions across 40 predefined brain subregions. Localization concordance with the actual resection site was scored at both lobar and sublobar levels. Interobserver agreement and the correlation between imaging localization and postsurgical seizure outcomes were analyzed.

Results

ISAS achieved significantly higher interobserver agreement than SISCOM for the localization (κ = 0.34–0.88 vs. 0.14–0.69). ISAS concordance with surgical resection was 88.7% at the lobar level and 79.7% at the sublobar level, compared to 71.7% and 60.9% for SISCOM, respectively. Logistic regression revealed that only ISAS localization at both scales significantly predicted seizure-free outcome, whereas SISCOM did not.

Conclusions

ISAS outperforms SISCOM in both localizing accuracy and observer reliability, and its concordance with surgical resection robustly predicts postoperative seizure freedom. These findings support the adoption of ISAS as the first-line SPECT postprocessing technique for presurgical evaluation of TLE.

目的：本研究旨在比较统计参数映射（ISAS）与减相颅面SPECT （SISCOM）在耐药颞叶癫痫（TLE）患者中的定位准确性、观察者间可靠性和预后价值。方法对60例连续接受癫痫切除手术的TLE患者进行分析。所有关键性SPECT研究均由ISAS和SISCOM处理。两名盲法审稿人独立确定了40个预先定义的脑亚区中最突出的高灌注区。在大叶和叶下水平对与实际切除部位的定位一致性进行评分。分析了观察者之间的一致性以及成像定位与术后癫痫发作结果的相关性。结果isas的定位一致性显著高于SISCOM （κ = 0.34-0.88 vs. 0.14-0.69）。ISAS与手术切除在大叶水平和叶下水平的一致性分别为88.7%和79.7%，而SISCOM的一致性分别为71.7%和60.9%。逻辑回归显示，只有ISAS定位在两个尺度上显著预测无癫痫发作的结果，而SISCOM没有。结论sisas在定位准确性和观察者可靠性方面优于SISCOM，其与手术切除的一致性有力地预测了术后癫痫发作的自由度。这些发现支持采用ISAS作为手术前评估TLE的一线SPECT后处理技术。

{"title":"Superior localizing and prognostic value of statistical parametric SPECT analysis in temporal lobe epilepsy: A comparative study of ISAS and SISCOM","authors":"Jeongsik Kim , Seung Hwan Moon , Hea Ree Park , Eun Yeon Joo , Dae-Won Seo , Young-Min Shon","doi":"10.1016/j.seizure.2026.01.001","DOIUrl":"10.1016/j.seizure.2026.01.001","url":null,"abstract":"<div><h3>Purpose</h3><div>This study aimed to compare the localizing accuracy, interobserver reliability, and prognostic value of ictal SPECT analyzed by Statistical Parametric Mapping (ISAS) versus Subtraction Ictal SPECT Co-registered to MRI (SISCOM) in patients with drug-resistant temporal lobe epilepsy (TLE).</div></div><div><h3>Methods</h3><div>Sixty consecutive TLE patients who underwent resective epilepsy surgery were analyzed. All ictal SPECT studies were processed by ISAS and SISCOM. Two blinded reviewers independently identified the most prominent hyperperfusion regions across 40 predefined brain subregions. Localization concordance with the actual resection site was scored at both lobar and sublobar levels. Interobserver agreement and the correlation between imaging localization and postsurgical seizure outcomes were analyzed.</div></div><div><h3>Results</h3><div>ISAS achieved significantly higher interobserver agreement than SISCOM for the localization (κ = 0.34–0.88 vs. 0.14–0.69). ISAS concordance with surgical resection was 88.7% at the lobar level and 79.7% at the sublobar level, compared to 71.7% and 60.9% for SISCOM, respectively. Logistic regression revealed that only ISAS localization at both scales significantly predicted seizure-free outcome, whereas SISCOM did not.</div></div><div><h3>Conclusions</h3><div>ISAS outperforms SISCOM in both localizing accuracy and observer reliability, and its concordance with surgical resection robustly predicts postoperative seizure freedom. These findings support the adoption of ISAS as the first-line SPECT postprocessing technique for presurgical evaluation of TLE.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 39-45"},"PeriodicalIF":2.8,"publicationDate":"2026-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146023819","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}

引用次数: 0

Association of systemic inflammatory markers with post-stroke epilepsy after ischemic stroke: A competing risk analysis 全身性炎症标志物与缺血性卒中后癫痫的关联：一项竞争风险分析。

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-07 DOI: 10.1016/j.seizure.2026.01.004

Pannaporn Imemkamon , Somjet Tosamran , Sununtha Jankaew , Nattawut Unwanatham , Chusak Limotai

Purpose

Post-stroke epilepsy (PSE) is a serious long-term complication of ischemic stroke, yet early identification of patients at risk remains challenging. Systemic inflammatory biomarkers may reflect underlying epileptogenic processes. This study explored the association between neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and hemoglobin-to-lymphocyte ratio (HLR) and the subsequent development of PSE in a large, long-term cohort.

Methods

We retrospectively analyzed 1445 adult patients hospitalized for acute ischemic stroke between 2014 and 2017 at a university-affiliated center. Patients with prior epilepsy were excluded. Blood counts within 2 days of admission were used to calculate NLR, PLR, and HLR. PSE was defined as the occurrence of at least one unprovoked seizure beyond 7 days post-stroke. A competing risk model was used to assess associations, accounting for death as a competing event.

Results

Over a median follow-up of nearly 7 years, 43 patients (2.98 %) developed PSE. Median NLR was higher in the PSE group than in the non-PSE group (3.45 vs. 2.94; nominal p = 0.036). In multivariable competing risk analysis, continuous NLR was nominally associated with PSE (subdistribution hazard ratio [SHR] = 1.048, 95 % CI 1.002–1.100; p = 0.040). As an exploratory sensitivity analysis, NLR > 7 was also associated with increased PSE incidence (SHR = 2.20, 95 % CI 1.11–4.35; p = 0.024). PLR and HLR did not show associations with PSE. The PSE group also experienced higher mortality and longer hospital stays.

Conclusion

NLR, an inexpensive and readily available inflammatory marker, was nominally associated with the development of PSE after ischemic stroke. These findings are exploratory and hypothesis-generating, supporting further investigation into the role of systemic inflammation in post-stroke epileptogenesis.

目的：卒中后癫痫（PSE）是缺血性卒中的一种严重的长期并发症，但早期识别患者的风险仍然具有挑战性。全身性炎症生物标志物可能反映潜在的癫痫发生过程。本研究在一个大型长期队列中探讨了中性粒细胞与淋巴细胞比率（NLR）、血小板与淋巴细胞比率（PLR）和血红蛋白与淋巴细胞比率（HLR）与PSE后续发展之间的关系。方法：回顾性分析2014年至2017年在某大学附属中心因急性缺血性脑卒中住院的1445例成人患者。排除既往有癫痫的患者。入院2天内的血液计数用于计算NLR、PLR和HLR。PSE定义为卒中后7天内至少发生一次非诱发性癫痫发作。使用竞争风险模型来评估关联，将死亡视为竞争事件。结果：在近7年的中位随访中，43例（2.98%）患者发生PSE。PSE组的中位NLR高于非PSE组（3.45 vs. 2.94；名义p = 0.036）。在多变量竞争风险分析中，连续NLR名义上与PSE相关（亚分布风险比[SHR] = 1.048, 95% CI 1.002-1.100; p = 0.040）。作为一项探索性敏感性分析，NLR bb0 7也与PSE发病率增加相关（SHR = 2.20, 95% CI 1.11-4.35; p = 0.024）。PLR和HLR与PSE无相关性。PSE组也经历了更高的死亡率和更长的住院时间。结论：NLR是一种廉价且容易获得的炎症标志物，名义上与缺血性卒中后PSE的发生有关。这些发现是探索性的和假设生成的，支持进一步研究全身性炎症在卒中后癫痫发生中的作用。

{"title":"Association of systemic inflammatory markers with post-stroke epilepsy after ischemic stroke: A competing risk analysis","authors":"Pannaporn Imemkamon , Somjet Tosamran , Sununtha Jankaew , Nattawut Unwanatham , Chusak Limotai","doi":"10.1016/j.seizure.2026.01.004","DOIUrl":"10.1016/j.seizure.2026.01.004","url":null,"abstract":"<div><h3>Purpose</h3><div>Post-stroke epilepsy (PSE) is a serious long-term complication of ischemic stroke, yet early identification of patients at risk remains challenging. Systemic inflammatory biomarkers may reflect underlying epileptogenic processes. This study explored the association between neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and hemoglobin-to-lymphocyte ratio (HLR) and the subsequent development of PSE in a large, long-term cohort.</div></div><div><h3>Methods</h3><div>We retrospectively analyzed 1445 adult patients hospitalized for acute ischemic stroke between 2014 and 2017 at a university-affiliated center. Patients with prior epilepsy were excluded. Blood counts within 2 days of admission were used to calculate NLR, PLR, and HLR. PSE was defined as the occurrence of at least one unprovoked seizure beyond 7 days post-stroke. A competing risk model was used to assess associations, accounting for death as a competing event.</div></div><div><h3>Results</h3><div>Over a median follow-up of nearly 7 years, 43 patients (2.98 %) developed PSE. Median NLR was higher in the PSE group than in the non-PSE group (3.45 vs. 2.94; nominal <em>p</em> = 0.036). In multivariable competing risk analysis, continuous NLR was nominally associated with PSE (subdistribution hazard ratio [SHR] = 1.048, 95 % CI 1.002–1.100; <em>p</em> = 0.040). As an exploratory sensitivity analysis, NLR > 7 was also associated with increased PSE incidence (SHR = 2.20, 95 % CI 1.11–4.35; <em>p</em> = 0.024). PLR and HLR did not show associations with PSE. The PSE group also experienced higher mortality and longer hospital stays.</div></div><div><h3>Conclusion</h3><div>NLR, an inexpensive and readily available inflammatory marker, was nominally associated with the development of PSE after ischemic stroke. These findings are exploratory and hypothesis-generating, supporting further investigation into the role of systemic inflammation in post-stroke epileptogenesis.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 11-18"},"PeriodicalIF":2.8,"publicationDate":"2026-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146004809","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}

引用次数: 0

IF 2.8 3区医学 Q2 CLINICAL NEUROLOGY

Seizure-European Journal of Epilepsy

Pub Date : 2026-01-07 DOI: 10.1016/j.seizure.2026.01.005

Thuppanattumadam Ananthasubramanian Sangeeth , LG Viswanathan , Hansashree Padmanabha , Akshaya Janardhanan , Ajay Asranna , Gautham Arunachal , Raghavendra Kenchaiah , Ravindranadh CM , Jitender Saini , Sanjib Sinha

Background

Progressive myoclonic epilepsies (PMEs) are severe epileptic encephalopathies characterized by drug-resistant seizures, myoclonus, neuroregression, and ataxia. Biallelic variants in KCTD7 cause a rare autosomal recessive PME (MIM #611726).

Methods

We retrospectively analysed six unrelated children with genetically confirmed KCTD7-related PME diagnosed at a quaternary referral centre in South India (2018–2025). Clinical features, EEG, SSEP, MRI, and genetic results were reviewed. Variant pathogenicity was assessed per ACMG guidelines.

Results

Six patients (3 male, 3 female; median onset 11 months, range 6–18 months) were included. Initial symptoms were seizures (four patients) or developmental delay (two patients), with regression in five patients. Fever-triggered worsening was noted in all patients. Ataxia was a common symptom (five patients). EEG showed generalized or multifocal epileptiform discharges, often posterior-predominant. MRI demonstrated diffuse cerebral/cerebellar atrophy and characteristic thalamic T2 hypo-intensity in three patients. Genetic analysis identified seven variants: five missense and two frame-shift, including three novel variants (p.Arg279Cys, p.Asp115Profs88, and p.Cys71fs*130). The recurrent p.Ala178Val variant was observed in two patients. One patient had epilepsia partialis continua responsive to corticosteroids.

Conclusions

This series expands the phenotypic and genotypic spectrum of KCTD7-related PME in India. Key clinical clues include developmental regression, seizures, cortical myoclonus, fever-provoked worsening, posterior-dominant epileptiform discharges, and early ataxia. The study highlights the importance of comprehensive genetic testing for accurate diagnosis, prognostication, and counselling in early-onset epileptic encephalopathies.

背景：进行性肌阵挛性癫痫（PMEs）是一种以耐药癫痫发作、肌阵挛、神经退化和共济失调为特征的严重癫痫性脑病。KCTD7的双等位基因变异导致罕见的常染色体隐性PME （mim# 611726）。方法：我们回顾性分析了2018-2025年在南印度一家四级转诊中心诊断的6名无亲缘关系的kctd7相关PME患儿。本文回顾了临床特征、脑电图、SSEP、MRI和遗传结果。根据ACMG指南评估变异致病性。结果：纳入6例患者（男3例，女3例；中位发病11个月，范围6-18个月）。最初症状为癫痫发作（4例）或发育迟缓（2例），其中5例出现发育迟缓。所有患者均出现发热引发的病情恶化。共济失调是常见症状（5例）。脑电图显示全身性或多灶性癫痫样放电，常后侧为主。MRI显示3例患者弥漫性脑/小脑萎缩和特征性丘脑T2低强度。遗传分析发现7个变异：5个错义和2个帧移位，包括3个新变异（p.Arg279Cys、p.Asp115Profs88和p.Cys71fs*130）。2例患者出现p.a ala178val变异体复发。1例患者患有持续部分性癫痫，对皮质类固醇有反应。结论：该系列扩大了印度kctd7相关PME的表型和基因型谱。关键的临床线索包括发育倒退、癫痫发作、皮质肌阵挛、发热引起的恶化、后显性癫痫样放电和早期共济失调。该研究强调了全面的基因检测对早发性癫痫性脑病的准确诊断、预测和咨询的重要性。

{"title":"KCTD7-related progressive myoclonic epilepsy: Clinical and genetic characterization of six Indian patients and review of literature","authors":"Thuppanattumadam Ananthasubramanian Sangeeth , LG Viswanathan , Hansashree Padmanabha , Akshaya Janardhanan , Ajay Asranna , Gautham Arunachal , Raghavendra Kenchaiah , Ravindranadh CM , Jitender Saini , Sanjib Sinha","doi":"10.1016/j.seizure.2026.01.005","DOIUrl":"10.1016/j.seizure.2026.01.005","url":null,"abstract":"<div><h3>Background</h3><div>Progressive myoclonic epilepsies (PMEs) are severe epileptic encephalopathies characterized by drug-resistant seizures, myoclonus, neuroregression, and ataxia. Biallelic variants in <em>KCTD7</em> cause a rare autosomal recessive PME (MIM #611726).</div></div><div><h3>Methods</h3><div>We retrospectively analysed six unrelated children with genetically confirmed <em>KCTD7</em>-related PME diagnosed at a quaternary referral centre in South India (2018–2025). Clinical features, EEG, SSEP, MRI, and genetic results were reviewed. Variant pathogenicity was assessed per ACMG guidelines.</div></div><div><h3>Results</h3><div>Six patients (3 male, 3 female; median onset 11 months, range 6–18 months) were included. Initial symptoms were seizures (four patients) or developmental delay (two patients), with regression in five patients. Fever-triggered worsening was noted in all patients. Ataxia was a common symptom (five patients). EEG showed generalized or multifocal epileptiform discharges, often posterior-predominant. MRI demonstrated diffuse cerebral/cerebellar atrophy and characteristic thalamic T2 hypo-intensity in three patients. Genetic analysis identified seven variants: five missense and two frame-shift, including three novel variants (p.Arg279Cys, p.Asp115Profs88, and p.Cys71fs*130). The recurrent p.Ala178Val variant was observed in two patients. One patient had epilepsia partialis continua responsive to corticosteroids.</div></div><div><h3>Conclusions</h3><div>This series expands the phenotypic and genotypic spectrum of <em>KCTD7</em>-related PME in India. Key clinical clues include developmental regression, seizures, cortical myoclonus, fever-provoked worsening, posterior-dominant epileptiform discharges, and early ataxia. The study highlights the importance of comprehensive genetic testing for accurate diagnosis, prognostication, and counselling in early-onset epileptic encephalopathies.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 28-33"},"PeriodicalIF":2.8,"publicationDate":"2026-01-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146020411","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}

引用次数: 0