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Seizures sparked by spironolactone: A case report 由螺内酯引起的癫痫发作:一例报告。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-11 DOI: 10.1016/j.seizure.2025.11.012
Mazieyar Azad , Thao Nguyen , Suparna Krishnaiengar , Katherine Zarroli
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引用次数: 0
Brivaracetam vs. Oxcarbazepine in childhood self-limited focal epilepsies (BRAVO-SeLFEs): A pilot randomized controlled trial 布瓦西坦与奥卡西平治疗儿童自限性局灶性癫痫(BRAVO-SeLFEs):一项随机对照试验
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-09 DOI: 10.1016/j.seizure.2025.11.011
Fathima Fasin , Ankit Kumar Meena , Saurabh Agarwal , Lokesh Saini , Sujatha Manjunathan , Tanu Gupta , Rahul Gupta , Ashwini Chityala , Jagdish Prasad Goyal , Naresh Nebhinani , Kuldeep Singh

Purpose

To compare the efficacy, safety, and behavioral outcomes of brivaracetam with oxcarbazepine in children with self-limited focal epilepsies (SeLFEs).

Methods

This is an open-label pilot randomized controlled trial conducted at a tertiary referral center in India. 50 children aged 2–18 years with SeLFEs were enrolled. They were randomized (1:1) to receive either brivaracetam (intervention) or oxcarbazepine (control) for 6 months. The primary endpoint was seizure freedom at 6 months. The secondary end points included epilepsy severity assessment using the Early Childhood Epilepsy Severity Scale (E-Chess), behavioral evaluation with the Child Behavior Checklist (CBCL), and functional assessment via the Vineland Social Maturity Scale (VSMS). Safety outcomes and feasibility parameters were recorded.

Results

At 6 months, seizure freedom was similar in the intervention (92 %) and control (86 %) groups with similar median cumulative seizures (16 vs. 22, p = 0.37). Both groups showed within-group reductions in median E-Chess scores [baseline, 5 (IQR 5–7); at 6 months, 3 (IQR 3–3)] but no intergroup differences at 6 months. The group x time interaction was not significant (ß = 0.44, p = 0.26). The median VSMS scores were comparable in both control [95 (IQR 91.5–97) to 96 (IQR 93–97)] and intervention [96 (IQR 93.5–98) to 97 (IQR 95–99.5)] groups. No behavioral abnormalities were noted in any participant (CBCL, T < 60). One child in the oxcarbazepine group developed a skin rash requiring drug withdrawal; no adverse effects were reported in the brivaracetam group. The retention rate was high (96 %) with comparable safety and feasibility.

Conclusion

Brivaracetam and oxcarbazepine achieved similar rates of seizure freedom at 6 months without causing any clinically significant behavioral abnormalities in either group.
目的比较布瓦西坦与奥卡西平治疗儿童自限性局灶性癫痫的疗效、安全性和行为结局。方法:这是一项在印度三级转诊中心进行的开放标签试点随机对照试验,招募了50名2-18岁的自恋儿童。他们随机(1:1)接受布伐西坦(干预)或奥卡西平(对照)治疗6个月。主要终点是6个月时癫痫发作自由。次要终点包括使用早期儿童癫痫严重程度量表(E-Chess)进行癫痫严重程度评估,使用儿童行为检查表(CBCL)进行行为评估,以及通过Vineland社会成熟度量表(VSMS)进行功能评估。记录安全结果和可行性参数。结果6个月时,干预组(92%)和对照组(86%)的癫痫发作自由度相似,中位累积癫痫发作相似(16 vs. 22, p = 0.37)。两组均显示组内电子象棋分数中位数下降[基线,5 (IQR 5 - 7);6个月时,3 (IQR 3 - 3)],但6个月时各组间无差异。组x时间交互作用不显著(ß = 0.44, p = 0.26)。对照组[95 (IQR 91.5-97)至96 (IQR 93-97)]和干预组[96 (IQR 93.5-98)至97 (IQR 95 - 99.5)]的中位VSMS评分具有可比性。未发现任何参与者的行为异常(CBCL, T < 60)。奥卡西平组的一名儿童出现皮疹,需要停药;布瓦西坦组未见不良反应。保留率高(96%),安全性和可行性相当。结论布瓦西坦和奥卡西平在6个月时癫痫发作自由率相似,两组患者均未出现明显的临床行为异常。
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引用次数: 0
Exploration of the mechanism of action of cenobamate cenobamate的作用机制探讨。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-07 DOI: 10.1016/j.seizure.2025.11.010
Raman Sankar , Louis Ferrari , Marc Kamin
Antiseizure medications have varied effects on neurotransmitter receptors and ion channels in the brain, yet how these mechanisms of action (MoAs) translate to clinical efficacy is not well understood. Cenobamate, a tetrazole alkyl carbamate antiseizure medication (ASM), has a dual MoA: preferential inhibition of the persistent sodium current (INaP) while sparing the transient sodium current (INaT), combined with extrasynaptic tonic inhibition mediated by positive allosteric modulation of gamma-aminobutyric acid type A (GABAA) receptors. In preclinical studies, cenobamate demonstrated broad-spectrum activity across various animal models of focal and generalized seizures. In clinical trials, cenobamate demonstrated rates of seizure freedom not observed with other voltage-gated sodium channel blockers (SCBs), whose main MoA involves modulating INaT or GABAA. In addition, real-world evidence suggests cenobamate may have efficacy in adult Dravet syndrome, a loss-of-function sodium channelopathy typically aggravated by SCBs. Cenobamate’s selectivity for INaP occurs at therapeutic concentrations, a characteristic seemingly unique among ASMs. Moreover, cenobamate preferentially modulates tonic (extrasynaptic) currents over phasic (synaptic) GABAA currents. These combined mechanistic effects may represent an emerging class of ASMs and could explain cenobamate’s broad-spectrum effect in animal seizure models and its efficacy for focal seizures in humans. In this review, we examine how cenobamate interacts with sodium currents and GABA receptor physiology and review cenobamate’s efficacy profile in humans. Finally, we will postulate how specific aspects of cenobamate’s dual MoA may contribute to its efficacy in comparison to other ASMs with similar MoAs.
抗癫痫药物对大脑中的神经递质受体和离子通道有不同的影响,但这些作用机制(MoAs)如何转化为临床疗效尚不清楚。Cenobamate是一种四唑烷基氨基甲酸酯类抗癫痫药物(ASM),具有双重MoA:优先抑制持续钠电流(INaP),同时保留瞬态钠电流(INaT),结合γ -氨基丁酸a型(GABAA)受体的正变构调节介导的突触外紧张性抑制。在临床前研究中,cenobamate在各种动物模型局灶性和全局性癫痫发作中表现出广谱活性。在临床试验中,cenobamate表现出的癫痫发作自由率与其他电压门控钠通道阻滞剂(scb)不同,后者的主要MoA涉及调节INaT或GABAA。此外,实际证据表明,cenobamate可能对成人Dravet综合征有效,这是一种功能丧失的钠通道病变,通常由scb加重。在治疗浓度下,Cenobamate对INaP的选择性发生,这似乎是asm中独有的特征。此外,相较于相位(突触)GABAA电流,左脑突起优先调节强直性(突触外)电流。这些综合的机制作用可能代表了一种新兴的asm,并可以解释cenobamate在动物癫痫模型中的广谱效应及其对人类局灶性癫痫发作的疗效。在这篇综述中,我们研究了cenobamate如何与钠电流和GABA受体生理相互作用,并回顾了cenobamate在人体中的疗效。最后,我们将假设与其他具有类似MoA的asm相比,cenobamate的双MoA的特定方面如何有助于其疗效。
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引用次数: 0
Educational attainment of children with self-limited epilepsy with CentroTemporal spikes (SELECTS), other epilepsies, and without epilepsy: A retrospective cohort study 伴有中央颞叶尖峰(SELECTS)、其他癫痫和无癫痫的自限性癫痫患儿的受教育程度:一项回顾性队列研究
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-07 DOI: 10.1016/j.seizure.2025.11.009
Arron S Lacey , Carys B Jones , Christopher J Weir , Jacqueline Stephen , William Owen Pickrell , Richard F Chin

Background

Children with epilepsy may have poorer educational outcomes—this may not be true for all epilepsy syndromes. We investigate educational attainment of children with Self-Limited Epilepsy with CentroTemporal Spikes (SELECTS) in Wales.

Method

A retrospective cohort study using routinely-collected data for children in Wales. We used primary care diagnosis codes to identify children (0–16 years) with SELECTS, other epilepsies, and children without epilepsy (comparators). We linked these records to Key Stage (KS) 2, 3 and 4 (ages 11,14, and 16) national educational test results (2003–2021). We performed logistic regression to analyse attainment (proportion achieving required attainment) in children with SELECTS, other epilepsies, and comparators.

Results

At KS 2,3 and 4: 101,92 and 81 children with SELECTS were matched to 299,274 and 243 children with other epilepsies and comparators. A lower proportion of the SELECTS and other epilepsies groups achieved required attainment than the comparators across all key stages.
After adjusting for sex, deprivation, year of study and Anti-Seizure Medications (ASM), children with SELECTS had similar achievement to comparators in KS2 and KS3:adjusted Odds Ratio (aOR,[95 %CI]) for achieving requirement:KS2:aOR=0.97[0.87–1.09];KS3:aOR=0.99[0.88–1.10]; but slightly reduced KS4 achievement:aOR=0.89,[0.80–1.00]. Children with other epilepsies were significantly less likely to achieve the requirement than comparators:KS2:aOR=0.79[0.72–0.87], KS3:aOR=0.78[0.71–0.86],KS4:aOR=0.72[0.65–0.80].

Conclusions

There was a trend for poorer educational achievement for children with SELECTS at KS4; this was only borderline statistically significant in the adjusted model. Children with other epilepsies had an increased risk of poorer attainment across all ages when compared to children without epilepsy.
背景:癫痫患儿的学习成绩可能较差,但并非所有癫痫综合征患者都是如此。我们调查了威尔士有中央颞叶尖峰(SELECTS)的自限性癫痫患儿的受教育程度。方法回顾性队列研究,使用常规收集的威尔士儿童数据。我们使用初级保健诊断代码来识别患有select、其他癫痫和无癫痫的儿童(0-16岁)(比较者)。我们将这些记录与关键阶段(KS) 2,3和4(11岁,14岁和16岁)国家教育考试结果(2003-2021)联系起来。我们采用逻辑回归分析select患儿、其他癫痫患儿和比较者的学业成就(达到要求学业成就的比例)。结果sks 2、3和4:select患儿101例、92例和81例与其他癫痫患儿299274例和243例相匹配。在所有关键阶段,与比较组相比,select组和其他癫痫组达到所需成就的比例较低。在调整性别、剥夺、学习年份和抗癫痫药物(ASM)后,select儿童在KS2和KS3方面的成就与比较者相似:达到要求的调整优势比(aOR,[95% CI]):KS2:aOR=0.97[0.87-1.09], KS3:aOR=0.99[0.88-1.10];但KS4成绩略有下降:aOR=0.89,[0.80-1.00]。其他癫痫患儿达到要求的可能性明显低于对照组:KS2:aOR=0.79[0.72 - 0.87], KS3:aOR=0.78[0.71-0.86],KS4:aOR=0.72[0.65-0.80]。结论小学四年级select儿童的学习成绩有较差的趋势;在调整后的模型中,这仅具有临界统计学意义。与没有癫痫的儿童相比,患有其他癫痫的儿童在所有年龄段的学习成绩较差的风险增加。
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引用次数: 0
Modified atkins diet in children with developmental and epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS): A prospective interventional, non-randomized, single-arm study 改良阿特金斯饮食在发展性和癫痫性脑病伴睡眠尖波激活(D/EE-SWAS)患儿中的应用:一项前瞻性、非随机、单臂研究
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-06 DOI: 10.1016/j.seizure.2025.11.007
Shagun Singh , Arushi Gahlot Saini , Deepika Puri , Smita Pattanaik , Rajni Sharma , Prahbhjot Malhi , Renu Suthar , Jitendra K Sahu , Naveen Sankhyan

Purpose

Developmental and Epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) is a rare and severe childhood epilepsy syndrome often associated with cognitive and language regression. While the ketogenic diet has shown efficacy in drug-resistant epilepsy, its role in D/EE-SWAS remains inadequately studied. This prospective study aimed to evaluate the efficacy and tolerability of the modified Atkins diet (MAD) in children with D/EE-SWAS refractory to standard therapies.

Methods

This was a single-arm, prospective interventional study conducted at a tertiary pediatric neurology unit from January 2022 to June 2023. Children aged 2–16 years with a confirmed diagnosis of D/EE-SWAS, with SWI ≥50 % during NREM sleep on EEG, and clinical evidence of seizure or neurodevelopmental regression despite treatment with at least two ASMs and corticosteroids were enrolled. The primary outcome was the change in SWI at 12 weeks. Secondary outcomes included seizure burden, cognitive outcomes (social quotient), and parent-reported language and behaviour changes at 24 weeks.

Results

Twenty-two children were enrolled; 10 completed 12 weeks and five completed 24 weeks of MAD. At 12 weeks, only 33 % showed a good EEG response (>50 % SWI reduction); one child achieved complete resolution at 24 weeks. Seizure remission (including maintenance of remission in those with no clinical seizures) was observed in 82 %, 90 %, and 100 % at 4, 12, and 24 weeks, respectively. No significant change in cognitive scores was seen. However, 60 % and 40 % of parents reported improvements in language and behaviour. Adverse effects were mostly mild, and compliance was a major barrier, with 54 % discontinuing the diet before 12 weeks.

Conclusion

The MAD was safe but not effective in the treatment of D/EE-SWAS in the short-term, with improvements only seen in seizure control. Its impact on EEG and cognition appears limited in the short-term and poor adherence poses significant challenges.
目的:发展性和癫痫性脑病伴睡眠尖波激活(D/EE-SWAS)是一种罕见且严重的儿童癫痫综合征,常伴有认知和语言退化。虽然生酮饮食已显示出对耐药癫痫的疗效,但其在D/EE-SWAS中的作用仍未得到充分研究。这项前瞻性研究旨在评估改良阿特金斯饮食(MAD)对标准治疗难治性D/EE-SWAS患儿的疗效和耐受性。方法:这是一项单臂前瞻性介入研究,于2022年1月至2023年6月在一所三级儿科神经内科进行。年龄2-16岁的儿童,确诊为D/EE-SWAS,脑电图非快速眼动睡眠期间SWI≥50%,尽管接受了至少两次asm和皮质类固醇治疗,但仍有癫痫发作或神经发育倒退的临床证据。主要终点是12周时SWI的变化。次要结果包括癫痫发作负担、认知结果(社会商数)和父母报告的24周时的语言和行为变化。结果:22名儿童入组;10人完成了12周的MAD, 5人完成了24周的MAD。12周时,只有33%的患者表现出良好的脑电图反应(SWI减少50%);一个孩子在24周时完全康复。在第4周、第12周和第24周,癫痫发作缓解(包括无临床癫痫发作患者的缓解维持)分别为82%、90%和100%。认知评分没有明显变化。然而,60%和40%的父母报告说,他们的语言和行为有所改善。副作用大多是轻微的,依从性是主要障碍,54%的患者在12周前停止饮食。结论:MAD在短期内治疗D/EE-SWAS是安全的,但并不有效,仅在癫痫发作控制方面有所改善。它对脑电图和认知的影响在短期内似乎有限,依从性差带来了重大挑战。
{"title":"Modified atkins diet in children with developmental and epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS): A prospective interventional, non-randomized, single-arm study","authors":"Shagun Singh ,&nbsp;Arushi Gahlot Saini ,&nbsp;Deepika Puri ,&nbsp;Smita Pattanaik ,&nbsp;Rajni Sharma ,&nbsp;Prahbhjot Malhi ,&nbsp;Renu Suthar ,&nbsp;Jitendra K Sahu ,&nbsp;Naveen Sankhyan","doi":"10.1016/j.seizure.2025.11.007","DOIUrl":"10.1016/j.seizure.2025.11.007","url":null,"abstract":"<div><h3>Purpose</h3><div>Developmental and Epileptic encephalopathy with spike-wave activation in sleep (D/EE-SWAS) is a rare and severe childhood epilepsy syndrome often associated with cognitive and language regression. While the ketogenic diet has shown efficacy in drug-resistant epilepsy, its role in D/EE-SWAS remains inadequately studied. This prospective study aimed to evaluate the efficacy and tolerability of the modified Atkins diet (MAD) in children with D/EE-SWAS refractory to standard therapies.</div></div><div><h3>Methods</h3><div>This was a single-arm, prospective interventional study conducted at a tertiary pediatric neurology unit from January 2022 to June 2023. Children aged 2–16 years with a confirmed diagnosis of D/EE-SWAS, with SWI ≥50 % during NREM sleep on EEG, and clinical evidence of seizure or neurodevelopmental regression despite treatment with at least two ASMs and corticosteroids were enrolled. The primary outcome was the change in SWI at 12 weeks. Secondary outcomes included seizure burden, cognitive outcomes (social quotient), and parent-reported language and behaviour changes at 24 weeks.</div></div><div><h3>Results</h3><div>Twenty-two children were enrolled; 10 completed 12 weeks and five completed 24 weeks of MAD. At 12 weeks, only 33 % showed a good EEG response (&gt;50 % SWI reduction); one child achieved complete resolution at 24 weeks. Seizure remission (including maintenance of remission in those with no clinical seizures) was observed in 82 %, 90 %, and 100 % at 4, 12, and 24 weeks, respectively. No significant change in cognitive scores was seen. However, 60 % and 40 % of parents reported improvements in language and behaviour. Adverse effects were mostly mild, and compliance was a major barrier, with 54 % discontinuing the diet before 12 weeks.</div></div><div><h3>Conclusion</h3><div>The MAD was safe but not effective in the treatment of D/EE-SWAS in the short-term, with improvements only seen in seizure control. Its impact on EEG and cognition appears limited in the short-term and poor adherence poses significant challenges.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"134 ","pages":"Pages 37-44"},"PeriodicalIF":2.8,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145574881","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
Cytotoxic lesions of the corpus callosum related to epilepsy or anti-seizure medications: a systematic review 与癫痫或抗癫痫药物相关的胼胝体细胞毒性病变:系统综述
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-06 DOI: 10.1016/j.seizure.2025.11.008
Gianni Cutillo , Guido Bonelli , Martina Rubin , Giordano Cecchetti , Jacopo Lanzone , Davide G. Curti , Anna Bellini , Giovanna F. Fanelli , Massimo Filippi

Background

Cytotoxic lesions of the corpus callosum (CLOCCs) are unusual MRI findings in epilepsy patients, often related to sudden anti-seizure medication (ASM) discontinuation or seizures. We reviewed the data of patients with epilepsy and/or under ASMs who developed CLOCCs, defining their clinical course focusing on therapies and intercurrent seizures.

Methods

A systematic review was performed including articles reporting on adult and pediatric patients who developed a CLOCC following ASMs modification and/or related to epilepsy.

Results

80 patients from 45 studies were included (44 females), 19 (24%) pediatric patients and 61 (76%) adults. 86% lesions were classified as Starkey A. CLOCCs were related to ASM withdrawal in 27 (34%) patients, seizure activity in 23 (29%), seizure activity with concomitant ASM variation in 18 (23%), ASM initiation in 5 (6%), ASM switch in 3 (4%) cases. The most frequent ASMs used in the sample were carbamazepine (30), phenytoin (14) and lamotrigine (13) however, non-sodium-channel blockers, e.g., levetiracetam (9) and valproate (8) were reported. CLOCCS regressed in a median time of 15 days (interquartile range [IQR]=14-25) in pediatric patients and 42 days (IQR=28-120) in adults (p<0.01).

Conclusion

CLOCCS are associated to sudden ASM modification or seizures. Regression time may vary widely and seems to be faster in children; moreover, non-sodium channel blockers are an increasingly recognized association.
背景:胼胝体细胞毒性病变(CLOCCs)是癫痫患者罕见的MRI表现,通常与突然停用抗癫痫药物(ASM)或癫痫发作有关。我们回顾了发生CLOCCs的癫痫和/或asm患者的数据,定义了他们的临床过程,重点是治疗和并发性癫痫发作。方法系统回顾了成人和儿童患者在asm修改后发生CLOCC和/或与癫痫相关的文章。结果45项研究共纳入80例患者,其中女性44例,儿童19例(24%),成人61例(76%)。86%的病变被归类为Starkey a。CLOCCs与ASM戒断相关27例(34%),癫痫发作活性23例(29%),癫痫发作活性伴ASM变化18例(23%),ASM开始5例(6%),ASM切换3例(4%)。样本中最常使用的ASMs是卡马西平(30),苯妥英(14)和拉莫三嗪(13),然而,非钠通道阻滞剂,如左乙拉西坦(9)和丙戊酸(8)被报道。儿童患者CLOCCS的中位回归时间为15天(四分位数间距[IQR]=14-25),成人患者为42天(IQR=28-120) (p<0.01)。结论cloccs与突发性ASM改变或癫痫发作有关。回归时间可能差别很大,在儿童中似乎更快;此外,非钠通道阻滞剂也越来越被认可。
{"title":"Cytotoxic lesions of the corpus callosum related to epilepsy or anti-seizure medications: a systematic review","authors":"Gianni Cutillo ,&nbsp;Guido Bonelli ,&nbsp;Martina Rubin ,&nbsp;Giordano Cecchetti ,&nbsp;Jacopo Lanzone ,&nbsp;Davide G. Curti ,&nbsp;Anna Bellini ,&nbsp;Giovanna F. Fanelli ,&nbsp;Massimo Filippi","doi":"10.1016/j.seizure.2025.11.008","DOIUrl":"10.1016/j.seizure.2025.11.008","url":null,"abstract":"<div><h3>Background</h3><div>Cytotoxic lesions of the corpus callosum (CLOCCs) are unusual MRI findings in epilepsy patients, often related to sudden anti-seizure medication (ASM) discontinuation or seizures. We reviewed the data of patients with epilepsy and/or under ASMs who developed CLOCCs, defining their clinical course focusing on therapies and intercurrent seizures.</div></div><div><h3>Methods</h3><div>A systematic review was performed including articles reporting on adult and pediatric patients who developed a CLOCC following ASMs modification and/or related to epilepsy.</div></div><div><h3>Results</h3><div>80 patients from 45 studies were included (44 females), 19 (24%) pediatric patients and 61 (76%) adults. 86% lesions were classified as Starkey A. CLOCCs were related to ASM withdrawal in 27 (34%) patients, seizure activity in 23 (29%), seizure activity with concomitant ASM variation in 18 (23%), ASM initiation in 5 (6%), ASM switch in 3 (4%) cases. The most frequent ASMs used in the sample were carbamazepine (30), phenytoin (14) and lamotrigine (13) however, non-sodium-channel blockers, e.g., levetiracetam (9) and valproate (8) were reported. CLOCCS regressed in a median time of 15 days (interquartile range [IQR]=14-25) in pediatric patients and 42 days (IQR=28-120) in adults (p&lt;0.01).</div></div><div><h3>Conclusion</h3><div>CLOCCS are associated to sudden ASM modification or seizures. Regression time may vary widely and seems to be faster in children; moreover, non-sodium channel blockers are an increasingly recognized association.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"133 ","pages":"Pages 261-267"},"PeriodicalIF":2.8,"publicationDate":"2025-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145520669","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
Minimally invasive ictal EEG source localization including sphenoidal electrodes – a retrospective study and a meta-analysis 微创脑电图源定位包括蝶窦电极-回顾性研究和荟萃分析。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-05 DOI: 10.1016/j.seizure.2025.11.005
Kara Goetz , Jakob I. Doerrfuss , Martin Holtkamp , Bernd J. Vorderwülbecke
Background: In patients with pharmacoresistant temporal lobe epilepsy, minimally invasive sphenoidal electrodes can enhance presurgical video-EEG monitoring. Placed into the infratemporal fossa, these electrodes record EEG activity from the mesial temporal lobe. We hypothesized that inclusion of the signal from sphenoidal electrodes would improve the diagnostic sensitivity of ictal EEG source localization (ESL).
Methods: We retrospectively included patients with pharmacoresistant focal epilepsy, presurgical low-density EEG including bilateral sphenoidal electrodes, subsequent resective surgery and favorable 1-year seizure outcome (ILAE class 1-2). Per seizure, ESL was conducted separately with vs. without signals from sphenoidal electrodes. Source maxima were compared to the resected zone. For comparison with the literature, we added a univariate random-effects meta-analysis of previous ictal ESL studies.
Results: ESL was applied to 184 seizures of 42 patients (39 with anterior temporal lobe epilepsy). At seizure level, ESL with vs. without sphenoidal electrodes achieved diagnostic sensitivities of 68 % (95 %-CI: 61–75 %) vs. 46 % (39–53 %; p < 0.001). Patient-level diagnostic sensitivity was 50 % (35–65 %) vs. 19 % (7–31 %; p = 0.002). The meta-analysis of ten studies on 233 patients resulted in a patient-level summary sensitivity of 68 % (44–84 %) albeit with substantial heterogeneity (I2=0.689).
Conclusions: As a proof of principle, sphenoidal electrodes significantly improved the diagnostic sensitivity of ictal ESL in absence of subtemporal scalp electrodes. Previous studies on ictal ESL yielded substantially heterogenous results, depending on their specific methodology. In presurgical epilepsy cases evaluated with both sphenoidal electrodes and ictal ESL, both techniques should be combined as presented here.
背景:在耐药颞叶癫痫患者中,微创蝶窦电极可以增强术前视频-脑电图监测。这些电极被放置在颞下窝,记录来自颞叶内侧的脑电图活动。我们假设包含蝶窦电极的信号可以提高颅电源定位(ESL)的诊断灵敏度。方法:我们回顾性地纳入了顽抗局灶性癫痫患者,术前低密度脑电图包括双侧蝶窦电极,随后切除手术和良好的1年癫痫发作结果(ILAE 1-2级)。每次癫痫发作,ESL分别进行,有蝶窦电极信号和没有蝶窦电极信号。将源最大值与切除区域进行比较。为了与文献进行比较,我们对之前的关键ESL研究进行了单变量随机效应荟萃分析。结果:应用ESL治疗42例癫痫发作184次,其中颞叶前癫痫39例。在癫痫发作水平,使用与不使用蝶骨电极的ESL的诊断敏感性分别为68% (95% -CI: 61- 75%)和46% (39- 53%;p < 0.001)。患者水平的诊断敏感性为50% (35- 65%)vs. 19% (7- 31%; p = 0.002)。对233例患者进行的10项研究的荟萃分析结果显示,患者水平的总敏感性为68%(44- 84%),尽管存在很大的异质性(I2=0.689)。结论:作为一个原理证明,蝶窦电极在没有颞下头皮电极的情况下显著提高了急性ESL的诊断敏感性。先前关于临界ESL的研究根据其具体的研究方法,得出了大量不同的结果。在术前癫痫患者同时使用蝶窦电极和临界ESL进行评估时,这两种技术应结合使用。
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引用次数: 0
The structure-function dissociation in mesial temporal lobe epilepsy with temporopolar blurring 中颞叶癫痫伴颞极模糊的结构-功能分离
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-04 DOI: 10.1016/j.seizure.2025.11.006
Yuming Li , Peiwen Liu , Qiuxing Lin , Wei Li , Yingying Zhang , Xiuli Li , Danyang Cao , Xiang Huang , Kailing Huang , Qiyong Gong , Dong Zhou , Dongmei An

Objectives

While ipsilateral temporopolar blurring (TPB) has been associated with white matter degeneration in temporal lobe epilepsy with hippocampal sclerosis (TLE-HS), its influence within the large-scale neural networks remains unclear. This study investigates the network-level topological alterations associated with TPB through multilayer network analysis.

Methods

This retrospective study investigated the impact of TPB on topological architecture of structural (SCN) and functional connectivity networks (FCN) in patients with TLE-HS using graph theory analysis. We further calculated the weighted multiplex participation coefficient (wMxP) to quantify the SCN-FCN interaction in a multiplex connectome.

Results

In total, 105 patients with TLE-HS (60 TPB+; 45 TPB-) and 61 healthy controls were enrolled. We found similar global network organization but altered nodal topological properties between TPB+ and TPB- in single-layer analysis, predominantly across the default mode network, visual network, and subcortical ROIs. Additionally, TPB+ showed decreased average wMxP (t(df=97) = -2.265, p < 2.2e-16) and lower nodal wMxP in default mode network and ipsilateral thalamus (t(df=97)=-2.404, pfwer=0.0408) than those in TPB-.

Conclusions

We demonstrate that disrupted topological architecture associated with TPB in TLE-HS were primarily and locally distributed in brain regions with rich connections to the temporal pole. The dissociation between SN and FN may indicate compensatory functional adjustments in TLE.
虽然同侧颞极模糊(TPB)与颞叶癫痫合并海马硬化(TLE-HS)的白质变性有关,但其在大规模神经网络中的影响尚不清楚。本研究通过多层网络分析探讨了与TPB相关的网络级拓扑变化。方法回顾性研究TPB对TLE-HS患者结构(SCN)和功能连接网络(FCN)拓扑结构的影响。我们进一步计算加权多路参与系数(wMxP)来量化多路连接组中SCN-FCN的相互作用。结果共纳入105例TLE-HS患者(TPB+ 60例,TPB- 45例)和61例健康对照。在单层分析中,我们发现TPB+和TPB-之间的全局网络组织相似,但节点拓扑特性发生了变化,主要分布在默认模式网络、视觉网络和皮层下roi中。与TPB-相比,TPB+组平均wMxP降低(t(df=97)=- 2.265, p < 2.2e-16),默认模式网络和同侧丘脑节点wMxP降低(t(df=97)=-2.404, pfwer=0.0408)。结论与TPB相关的拓扑结构紊乱主要和局部分布于与颞极有丰富连接的脑区。SN和FN之间的分离可能表明TLE的代偿功能调节。
{"title":"The structure-function dissociation in mesial temporal lobe epilepsy with temporopolar blurring","authors":"Yuming Li ,&nbsp;Peiwen Liu ,&nbsp;Qiuxing Lin ,&nbsp;Wei Li ,&nbsp;Yingying Zhang ,&nbsp;Xiuli Li ,&nbsp;Danyang Cao ,&nbsp;Xiang Huang ,&nbsp;Kailing Huang ,&nbsp;Qiyong Gong ,&nbsp;Dong Zhou ,&nbsp;Dongmei An","doi":"10.1016/j.seizure.2025.11.006","DOIUrl":"10.1016/j.seizure.2025.11.006","url":null,"abstract":"<div><h3>Objectives</h3><div>While ipsilateral temporopolar blurring (TPB) has been associated with white matter degeneration in temporal lobe epilepsy with hippocampal sclerosis (TLE-HS), its influence within the large-scale neural networks remains unclear. This study investigates the network-level topological alterations associated with TPB through multilayer network analysis.</div></div><div><h3>Methods</h3><div>This retrospective study investigated the impact of TPB on topological architecture of structural (SCN) and functional connectivity networks (FCN) in patients with TLE-HS using graph theory analysis. We further calculated the weighted multiplex participation coefficient (wMxP) to quantify the SCN-FCN interaction in a multiplex connectome.</div></div><div><h3>Results</h3><div>In total, 105 patients with TLE-HS (60 TPB+; 45 TPB-) and 61 healthy controls were enrolled. We found similar global network organization but altered nodal topological properties between TPB+ and TPB- in single-layer analysis, predominantly across the default mode network, visual network, and subcortical ROIs. Additionally, TPB+ showed decreased average wMxP (t(df=97) = -2.265, <em>p</em> &lt; 2.2e-16) and lower nodal wMxP in default mode network and ipsilateral thalamus (t(df=97)=-2.404, p<sub>fwer</sub>=0.0408) than those in TPB-.</div></div><div><h3>Conclusions</h3><div>We demonstrate that disrupted topological architecture associated with TPB in TLE-HS were primarily and locally distributed in brain regions with rich connections to the temporal pole. The dissociation between SN and FN may indicate compensatory functional adjustments in TLE.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"134 ","pages":"Pages 70-78"},"PeriodicalIF":2.8,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145580514","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
Does 7 T MRI offer an added value in drug resistant temporal lobe epilepsy? 7 T MRI对耐药颞叶癫痫是否有附加价值?
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-04 DOI: 10.1016/j.seizure.2025.11.004
Ariadne Zampeli , Miroslav Malac , Isabella M. Björkman-Burtscher , Boel Hansson , Linda Wennberg , Karin Markenroth Bloch , Kristina Källén , Maria Compagno Strandberg

Purpose

To evaluate the added value of 7 Tesla (T) magnetic resonance imaging (MRI) in the presurgical assessment of patients with temporal lobe epilepsy (TLE), considering its overall impact on clinical decision-making, including both MRI-positive and MRI-negative cases, and its timing during the evaluation process.

Methods

Sixty-one patients with drug-resistant TLE, enrolled in this study underwent both 3 T and 7 T MRI, along with other diagnostic assessments (video electroencephalography, positron emission tomography, stereo electroencephalography). Patients were categorized into three phases of the presurgical evaluation: basic, intermediate, and advanced. The added value of 7 T MRI was assessed based on its ability to confirm, exclude, or detect new epileptogenic lesions, as well as its influence on surgical decision-making.

Results

7 T MRI provided added clinical value in 51 % of cases, improving lesion detection and contributing to clinical decisions, particularly in patients with unclear or ambiguous findings. In some instances, it confirmed previously suspected lesions or revealed previously undetected lesions, leading to surgical interventions. It also helped exclude possible lesions, influencing decisions to avoid unnecessary surgery. Notably, hippocampal malrotation (HIMAL) was identified in several patients, warranting further investigation regarding its potential epileptogenic role.

Conclusion

7 T MRI enhances the presurgical evaluation of TLE by improving lesion detection, clarifying ambiguous findings, and aiding clinical decision-making. Its use should be considered at various stages of the evaluation process, as it offers valuable diagnostic information for both MRI-positive and MRI-negative patients. Further research is needed to explore the possible epileptogenic significance of HIMAL.
目的:探讨7特斯拉(T)磁共振成像(MRI)在颞叶癫痫(TLE)患者术前评估中的附加价值,考虑其对临床决策的整体影响,包括MRI阳性和MRI阴性病例,以及在评估过程中的时机。方法:纳入本研究的61例耐药TLE患者接受了3t和7t MRI检查,并进行了其他诊断评估(视频脑电图、正电子发射断层扫描、立体脑电图)。患者被分为三个阶段的术前评估:基础,中期和晚期。7 T MRI的附加价值是根据其确认、排除或发现新的癫痫性病变的能力以及对手术决策的影响来评估的。结果:7t MRI在51%的病例中提供了额外的临床价值,改善了病变检测并有助于临床决策,特别是在发现不明确或模棱两可的患者中。在某些情况下,它证实了以前怀疑的病变或发现了以前未发现的病变,导致手术干预。它还有助于排除可能的病变,影响决定避免不必要的手术。值得注意的是,在一些患者中发现了海马旋转不良(HIMAL),需要进一步研究其潜在的致痫作用。结论:7 T MRI通过改善病变发现、澄清模糊表现、辅助临床决策,增强了TLE的术前评估。应在评估过程的各个阶段考虑其使用,因为它为mri阳性和mri阴性患者提供了有价值的诊断信息。HIMAL可能的致痫意义有待进一步研究。
{"title":"Does 7 T MRI offer an added value in drug resistant temporal lobe epilepsy?","authors":"Ariadne Zampeli ,&nbsp;Miroslav Malac ,&nbsp;Isabella M. Björkman-Burtscher ,&nbsp;Boel Hansson ,&nbsp;Linda Wennberg ,&nbsp;Karin Markenroth Bloch ,&nbsp;Kristina Källén ,&nbsp;Maria Compagno Strandberg","doi":"10.1016/j.seizure.2025.11.004","DOIUrl":"10.1016/j.seizure.2025.11.004","url":null,"abstract":"<div><h3>Purpose</h3><div>To evaluate the added value of 7 Tesla (T) magnetic resonance imaging (MRI) in the presurgical assessment of patients with temporal lobe epilepsy (TLE), considering its overall impact on clinical decision-making, including both MRI-positive and MRI-negative cases, and its timing during the evaluation process.</div></div><div><h3>Methods</h3><div>Sixty-one patients with drug-resistant TLE, enrolled in this study underwent both 3 T and 7 T MRI, along with other diagnostic assessments (video electroencephalography, positron emission tomography, stereo electroencephalography). Patients were categorized into three phases of the presurgical evaluation: basic, intermediate, and advanced. The added value of 7 T MRI was assessed based on its ability to confirm, exclude, or detect new epileptogenic lesions, as well as its influence on surgical decision-making.</div></div><div><h3>Results</h3><div>7 T MRI provided added clinical value in 51 % of cases, improving lesion detection and contributing to clinical decisions, particularly in patients with unclear or ambiguous findings. In some instances, it confirmed previously suspected lesions or revealed previously undetected lesions, leading to surgical interventions. It also helped exclude possible lesions, influencing decisions to avoid unnecessary surgery. Notably, hippocampal malrotation (HIMAL) was identified in several patients, warranting further investigation regarding its potential epileptogenic role.</div></div><div><h3>Conclusion</h3><div>7 T MRI enhances the presurgical evaluation of TLE by improving lesion detection, clarifying ambiguous findings, and aiding clinical decision-making. Its use should be considered at various stages of the evaluation process, as it offers valuable diagnostic information for both MRI-positive and MRI-negative patients. Further research is needed to explore the possible epileptogenic significance of HIMAL.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"134 ","pages":"Pages 27-36"},"PeriodicalIF":2.8,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145574784","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
Characteristics of epilepsy associated with hemophagocytic lymphohistiocytosis 与噬血细胞性淋巴组织细胞增多症相关的癫痫特征
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-11-03 DOI: 10.1016/j.seizure.2025.11.003
Xue Wang, Hongyan Bi, Yingying Zhao, Yongbo Zhang

Objective

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome frequently complicated by severe neurological manifestations, including epilepsy. This study aimed to characterize the clinical phenotype, identify risk factors, and determine outcomes specific to HLH-associated epilepsy.

Methods

We conducted a retrospective cohort study of 76 patients with HLH, comparing those with epilepsy (n = 25) and those without (n = 51). Comprehensive clinical, laboratory, genetic, electroencephalogram, and neuroimaging data were analyzed.

Results

Patients with HLH-associated epilepsy were significantly younger and had a higher prevalence of primary HLH. They demonstrated a more severe inflammatory profile, marked by elevated ferritin, interleukin-6, and cerebrospinal fluid (CSF) pleocytosis. Multivariate analysis identified age ≤25 years, ferritin >20,000 ng/mL, CSF pleocytosis, and evolution to cortical atrophy as independent risk factors for epilepsy. Neuroimaging revealed a characteristic "enhancement-to-atrophy" sequence, where acute contrast-enhancing lesions (64.0 % vs. 29.4 %) often progressed to cortical atrophy (56.0 % vs. 19.6 %), highlighting a trajectory from acute neuroinflammation to permanent structural injury. The epilepsy group had significantly poorer survival (median 19 vs. 23 months). Although systemic HLH remission was achieved, the majority of patients (68.0 %) developed refractory epilepsy, necessitating long-term antiseizure medication.

Significance

HLH-associated epilepsy constitutes a severe clinical entity driven by intense neuroinflammation, which frequently results in irreversible brain damage. Early identification of its hallmark features, including young age, extreme hyperferritinemia, CSF pleocytosis, and the distinctive "enhancement-to-atrophy" neuroimaging sequence, is crucial for prompt intervention and long-term neurological management.
目的:噬细胞性淋巴组织细胞增多症(HLH)是一种危及生命的高炎症综合征,常伴有严重的神经系统症状,包括癫痫。本研究旨在描述临床表型,确定危险因素,并确定特定于hlh相关癫痫的结果。方法对76例HLH患者进行回顾性队列研究,比较癫痫患者(n = 25)和非癫痫患者(n = 51)。综合临床、实验室、遗传、脑电图和神经影像学资料进行分析。结果HLH相关性癫痫患者明显年轻化,且原发性HLH患病率较高。他们表现出更严重的炎症特征,表现为铁蛋白、白细胞介素-6升高和脑脊液(CSF)多细胞增多。多因素分析发现,年龄≤25岁、铁蛋白≤20000 ng/mL、脑脊液多细胞增多、向皮质萎缩演变为癫痫的独立危险因素。神经影像学显示了特征性的“增强到萎缩”序列,其中急性对比增强病变(64.0%对29.4%)通常进展为皮质萎缩(56.0%对19.6%),突出了从急性神经炎症到永久性结构损伤的轨迹。癫痫组的生存期明显较差(中位19个月vs. 23个月)。虽然达到了全身HLH缓解,但大多数患者(68.0%)发展为难治性癫痫,需要长期抗癫痫药物治疗。hlh相关癫痫是一种由强烈神经炎症驱动的严重临床症状,经常导致不可逆的脑损伤。早期识别其标志性特征,包括年轻,极端高铁蛋白血症,脑脊液多细胞症,以及独特的“增强-萎缩”神经成像序列,对于及时干预和长期神经系统管理至关重要。
{"title":"Characteristics of epilepsy associated with hemophagocytic lymphohistiocytosis","authors":"Xue Wang,&nbsp;Hongyan Bi,&nbsp;Yingying Zhao,&nbsp;Yongbo Zhang","doi":"10.1016/j.seizure.2025.11.003","DOIUrl":"10.1016/j.seizure.2025.11.003","url":null,"abstract":"<div><h3>Objective</h3><div>Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome frequently complicated by severe neurological manifestations, including epilepsy. This study aimed to characterize the clinical phenotype, identify risk factors, and determine outcomes specific to HLH-associated epilepsy.</div></div><div><h3>Methods</h3><div>We conducted a retrospective cohort study of 76 patients with HLH, comparing those with epilepsy (<em>n</em> = 25) and those without (<em>n</em> = 51). Comprehensive clinical, laboratory, genetic, electroencephalogram, and neuroimaging data were analyzed.</div></div><div><h3>Results</h3><div>Patients with HLH-associated epilepsy were significantly younger and had a higher prevalence of primary HLH. They demonstrated a more severe inflammatory profile, marked by elevated ferritin, interleukin-6, and cerebrospinal fluid (CSF) pleocytosis. Multivariate analysis identified age ≤25 years, ferritin &gt;20,000 ng/mL, CSF pleocytosis, and evolution to cortical atrophy as independent risk factors for epilepsy. Neuroimaging revealed a characteristic \"enhancement-to-atrophy\" sequence, where acute contrast-enhancing lesions (64.0 % vs. 29.4 %) often progressed to cortical atrophy (56.0 % vs. 19.6 %), highlighting a trajectory from acute neuroinflammation to permanent structural injury. The epilepsy group had significantly poorer survival (median 19 vs. 23 months). Although systemic HLH remission was achieved, the majority of patients (68.0 %) developed refractory epilepsy, necessitating long-term antiseizure medication.</div></div><div><h3>Significance</h3><div>HLH-associated epilepsy constitutes a severe clinical entity driven by intense neuroinflammation, which frequently results in irreversible brain damage. Early identification of its hallmark features, including young age, extreme hyperferritinemia, CSF pleocytosis, and the distinctive \"enhancement-to-atrophy\" neuroimaging sequence, is crucial for prompt intervention and long-term neurological management.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"133 ","pages":"Pages 275-280"},"PeriodicalIF":2.8,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145520658","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
期刊
Seizure-European Journal of Epilepsy
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