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Corrigendum to “Does 7 T MRI offer an added value in drug resistant temporal lobe epilepsy?” [Seizure: European Journal of Epilepsy 134 (2026) 27–36] “7t MRI对耐药颞叶癫痫是否有附加价值?”的更正。[癫痫:欧洲癫痫杂志134(2026)27-36]。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-14 DOI: 10.1016/j.seizure.2026.02.008
Ariadne Zampeli , Miroslav Malac , Isabella M. Björkman-Burtscher , Boel Hansson , Linda Wennberg , Karin Markenroth Bloch , Kristina Källén , Maria Compagno Strandberg
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引用次数: 0
Lamotrigine as an antiseizure medication in pregnancy 拉莫三嗪作为妊娠期抗癫痫药物。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-02 DOI: 10.1016/j.seizure.2026.02.001
Frank J.E. Vajda , Terence J. O’Brien , Janet E. Graham , Alison E. Hitchcock , Piero Perucca , Cecilie M. Lander , Mervyn J. Eadie

Aim

To try to ascertain in APR data why lamotrigine was the preferred ASM in treating women with epilepsy,

Method

Statistical analysis of foetal malformation and seizure free pregnancy occurrence rates associated with lamotrigine use in monotherapy and polytherapy

Results

Among commonly used ASMs, lamotrigine (4.6%) and levetiracetam (4.5%) in monotherapy had the lowest associated foetal malformation rates, compared with a 3.2% rate in ASM-untreated pregnancy in women with epilepsy. The lamotrigine monotherapy seizure freedom rate during pregnancy (56.6%) was lower than that associated with all other ASM monotherapy combined (68.7%; R.R. 1.21, 95% C.I. 1.12, 1.32).

Conclusion

Those prescribing ASM therapy for women capable of pregnancy seem to have given priority to avoiding foetal malformation over achieving seizure freedom in pregnancy.
方法:统计分析拉莫三嗪单药和多药治疗中胎儿畸形和无癫痫发作妊娠发生率。结果:在常用的抗痉挛药物中,拉莫三嗪单药治疗(4.6%)和左乙拉西坦单药治疗(4.5%)的胎儿畸形发生率最低,而未使用抗痉挛药物治疗的癫痫孕妇胎儿畸形发生率为3.2%。拉莫三嗪单药治疗妊娠期癫痫发作自由率(56.6%)低于其他ASM单药联合治疗(68.7%;rr 1.21, 95% C.I. 1.12, 1.32)。结论:对于有妊娠能力的妇女,ASM治疗似乎优先考虑避免胎儿畸形而不是实现妊娠期间癫痫的自由发作。
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引用次数: 0
Efficacy and safety of cenobamate-based combination therapy in drug-resistant epilepsy: Secondary analysis by mechanisms of action of concomitant antiseizure medications 以辛奥巴马酸为基础的联合治疗耐药癫痫的疗效和安全性:联合抗癫痫药物作用机制的二次分析。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-03-01 Epub Date: 2026-02-13 DOI: 10.1016/j.seizure.2026.02.013
Magdalena Bosak , Hanna Podraza , Dorota Włoch-Kopeć , Andrzej Rysz , Kamil Wężyk , Katarzyna Grabska-Radzikowska , Piotr Sobolewski , Tomasz Siwek , Iwona Kurkowska-Jastrzębska , Monika Służewska-Niedźwiedź , Katarzyna Sulima , Lech Kipiński , Lidia Kiryła , Katarzyna Stopińska , Elżbieta Płonka-Półtorak , Justyna Tabaka-Pradela , Magdalena Konopko , Agnieszka Meller , Monika Chorąży , Maja Kopytek-Beuzen , Katarzyna Zawiślak-Fornagiel

Introduction

In a secondary analysis of a cohort of 475 adults with drug-resistant epilepsy treated with adjunctive cenobamate, we assessed whether treatment outcomes were influenced by concomitant antiseizure medication class and cenobamate dose.

Materials and methods

Concomitant antiseizure medications (ASMs) were grouped as sodium channel blockers (SCBs), SV2A ligands, valproate, carbonic anhydrase inhibitors, or GABA analogs. Efficacy and safety outcomes were analyzed using multivariate logistic regression, adjusting for confounders.

Results

Baseline use of valproate was associated with higher odds of achieving ≥50 % seizure reduction and seizure freedom, whereas SCB co-therapy was associated with lower odds of seizure freedom. At the final follow-up, these efficacy differences largely disappeared, except that SCB use modestly favored ≥50 % response. SCB co-medication was associated with a decreased risk of somnolence, while the presence of an SV2A ligands, valproate, or carbonic anhydrase inhibitors was correlated with lower treatment discontinuation rates. Cenobamate dose showed no significant association with achieving ≥50 % response, seizure freedom, or overall AE occurrence, reflecting the pragmatic character of the studied cohort.

Conclusion

Cenobamate demonstrated optimal efficacy when combined with valproate and broad-spectrum agents, and suboptimal efficacy when coadministered with SCB. Substantial seizure improvements were often achieved at moderate doses of cenobamate, underscoring the value of individualized titration and comedication management.
在一项对475名接受辅助辛奥巴酸治疗的成人耐药癫痫患者的队列分析中,我们评估了伴随抗癫痫药物类别和辛奥巴酸剂量是否影响治疗结果。材料和方法:同时使用抗癫痫药物(asm)分为钠通道阻滞剂(scb)、SV2A配体、丙戊酸盐、碳酸酐酶抑制剂或GABA类似物。使用多变量逻辑回归分析疗效和安全性结果,调整混杂因素。结果:基线使用丙戊酸盐与实现≥50%癫痫发作减少和癫痫发作自由的几率较高相关,而SCB联合治疗与癫痫发作自由的几率较低相关。在最后的随访中,这些疗效差异基本上消失了,除了SCB使用适度有利于≥50%的反应。SCB联合用药与嗜睡风险降低相关,而SV2A配体、丙戊酸盐或碳酸酐酶抑制剂的存在与较低的停药率相关。Cenobamate剂量与达到≥50%的反应、癫痫发作自由度或总体AE发生率无显著相关性,反映了研究队列的实用性。结论:Cenobamate与丙戊酸盐和广谱药物合用时疗效最佳,与SCB合用时疗效次优。在中等剂量的cenobamate治疗下,癫痫发作通常得到显著改善,这强调了个体化滴定和药物管理的价值。
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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 Pub Date : 2026-03-01 Epub 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。
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引用次数: 0
Mitochondrial complex assembly in epilepsy of primary mitochondrial disease origin. 原发性线粒体疾病起源癫痫的线粒体复合体组装。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-18 DOI: 10.1016/j.seizure.2026.02.016
Gavin P McStay

Primary mitochondrial diseases are caused by mutations in genes required for expression, function or assembly of the mitochondrial oxidative phosphorylation system. The pathology of primary mitochondrial diseases is varied and a subset of these are associated with epilepsy and seizures. Mutations are found in each of the 5 complexes of the oxidative phosphorylation system in both structural subunits and assembly factors along with mitochondrially encoded components of the protein synthesis machinery. This review will highlight the mutations identified in clinical case studies that are associated with epilepsy and seizures and include the studies using cell systems and other model organisms where molecular characterisation of oxidative phosphorylation is more extensive. The molecular causes of epilepsy have not been well characterised in the relevant cells. This review identifies gaps in knowledge and suggestions for future studies to advance the understanding of the molecular pathogenesis of epilepsy that is associated with primary mitochondrial disease.

原发性线粒体疾病是由线粒体氧化磷酸化系统的表达、功能或组装所需的基因突变引起的。原发性线粒体疾病的病理是多种多样的,其中一部分与癫痫和癫痫发作有关。在氧化磷酸化系统的5个复合物中,在结构亚基和组装因子以及蛋白质合成机制的线粒体编码成分中都发现了突变。本综述将重点介绍在与癫痫和癫痫发作相关的临床病例研究中发现的突变,并包括使用细胞系统和其他模式生物的研究,其中氧化磷酸化的分子特征更广泛。癫痫的分子成因尚未在相关细胞中得到很好的表征。这篇综述指出了知识上的差距和对未来研究的建议,以促进对与原发性线粒体疾病相关的癫痫分子发病机制的理解。
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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 Pub Date : 2026-02-01 Epub 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 ,&nbsp;Seung Hwan Moon ,&nbsp;Hea Ree Park ,&nbsp;Eun Yeon Joo ,&nbsp;Dae-Won Seo ,&nbsp;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":"2026-02-01","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
Onchocerciasis-associated epilepsy: Charting a path forward 盘尾丝虫病相关癫痫:探索前进之路
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-01 Epub Date: 2024-04-21 DOI: 10.1016/j.seizure.2024.04.018
G Van Cutsem , JN Siewe Fodjo , A Hadermann , L-J Amaral , C Trevisan , S Pion , R Colebunders
This narrative review intends to inform neurologists and public health professionals about Onchocerciasis-Associated Epilepsy (OAE), a neglected public health problem in many remote onchocerciasis-endemic areas. For epidemiological purposes, we define OAE as sudden-onset of convulsive and non-convulsive seizure types, including head nodding seizures (nodding syndrome) in a previously healthy child aged 3 to 18 years in the absence of any other obvious cause for epilepsy, all happening within an area with high ongoing Onchocerca volvulus transmission. Several OAE pathophysiological mechanisms have been proposed, but none has been proven yet. Recent population-based studies showed that strengthening onchocerciasis elimination programs was followed by a significant reduction in the incidence of OAE and nodding syndrome. Treating epilepsy in onchocerciasis-endemic regions is challenging. More advocacy is needed to provide uninterrupted, free access to anti-seizure medication to persons with epilepsy in these remote, impoverished areas. It is crucial todevelop policies and increase funding for the prevention and treatment of OAE to reduce the associated burden of disease, notably via the establishment of morbidity management and disability prevention programs (MMDP). Moreover, effective collaboration between onchocerciasis elimination and mental health programs is imperative to alleviate the burden of OAE. This synergy promises reciprocal advantages and underscores the need for a comprehensive approach to address this multifaceted challenge.
这篇叙述性综述旨在告知神经科医生和公共卫生专业人员盘尾丝虫病相关癫痫(OAE),这是许多偏远盘尾丝虫病流行地区被忽视的公共卫生问题。出于流行病学目的,我们将OAE定义为突然发作的惊厥和非惊厥发作类型,包括在没有任何其他明显癫痫病因的3至18岁健康儿童中发生的头部点头发作(点头综合征),所有这些都发生在盘尾丝虫高度传播的区域内。已经提出了几种OAE的病理生理机制,但尚未得到证实。最近基于人群的研究表明,加强盘尾丝虫病消除计划之后,OAE和点头综合征的发生率显著降低。在盘尾丝虫病流行地区治疗癫痫具有挑战性。需要更多的宣传,为这些偏远贫困地区的癫痫患者提供不间断、免费的抗癫痫药物。制定政策和增加资金用于OAE的预防和治疗,以减少相关的疾病负担至关重要,特别是通过建立发病率管理和残疾预防计划(MMDP)。此外,消除盘尾丝虫病与心理健康项目之间的有效合作对于减轻盘尾丝虫病的负担至关重要。这种协同作用保证了互惠互利,并强调需要采取综合办法来应对这一多方面的挑战。
{"title":"Onchocerciasis-associated epilepsy: Charting a path forward","authors":"G Van Cutsem ,&nbsp;JN Siewe Fodjo ,&nbsp;A Hadermann ,&nbsp;L-J Amaral ,&nbsp;C Trevisan ,&nbsp;S Pion ,&nbsp;R Colebunders","doi":"10.1016/j.seizure.2024.04.018","DOIUrl":"10.1016/j.seizure.2024.04.018","url":null,"abstract":"<div><div>This narrative review intends to inform neurologists and public health professionals about Onchocerciasis-Associated Epilepsy (OAE), a neglected public health problem in many remote onchocerciasis-endemic areas. For epidemiological purposes, we define OAE as sudden-onset of convulsive and non-convulsive seizure types, including head nodding seizures (nodding syndrome) in a previously healthy child aged 3 to 18 years in the absence of any other obvious cause for epilepsy, all happening within an area with high ongoing <em>Onchocerca volvulus</em> transmission. Several OAE pathophysiological mechanisms have been proposed, but none has been proven yet. Recent population-based studies showed that strengthening onchocerciasis elimination programs was followed by a significant reduction in the incidence of OAE and nodding syndrome. Treating epilepsy in onchocerciasis-endemic regions is challenging. More advocacy is needed to provide uninterrupted, free access to anti-seizure medication to persons with epilepsy in these remote, impoverished areas. It is crucial todevelop policies and increase funding for the prevention and treatment of OAE to reduce the associated burden of disease, notably via the establishment of morbidity management and disability prevention programs (MMDP). Moreover, effective collaboration between onchocerciasis elimination and mental health programs is imperative to alleviate the burden of OAE. This synergy promises reciprocal advantages and underscores the need for a comprehensive approach to address this multifaceted challenge.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 105-114"},"PeriodicalIF":2.8,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140766218","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
Poor neurodevelopment, nutritional and physical growth outcomes among children born to mothers with nodding syndrome 患有点头综合征的母亲所生子女的神经发育、营养和身体发育状况不佳。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-01 Epub Date: 2024-09-19 DOI: 10.1016/j.seizure.2024.09.012
Mary Nyakato , Shubaya Kasule Naggayi , Pamela Rosemary Akun , Sam Ononge , Alfred Odong , Erias Adams Baguma , Hellen Nansiiro , Dennis Kalibbala , Simple Ouma , Innocent Besigye , Richard Idro

Introduction

Nodding syndrome (NS), a poorly understood severe neurological disorder develops in children. In Uganda, some NS cases have grown into child-bearing adults. Babies born to mothers with NS may be prone to impaired neurodevelopmental outcomes. Cognitive deficits in mothers with NS may further inhibit care offered to their children hence compromising neurocognitive development, physical growth, and behaviour.

Objectives

The study aimed to determine the neurodevelopmental, behavioural, nutritional, and physical growth outcomes of children whose mothers have nodding syndrome.

Methods

A comparative cross-sectional study was conducted between May 2021 and April 2022 in Northern Uganda. Children aged 0-5 years of mothers with NS were compared to those of mothers without NS, matched by age, gender and neighbouring residence. Neurodevelopment, behaviour, nutrition, and physical growth were assessed using standardized measures and t-tests employed for group comparisons of outcomes.

Results

Overall,106 children participated. Fifty-three (53) were offspring of mothers with NS and 53 of mothers without NS; having a mean age of 26.9 (2.22) and 27.5(2.12) months respectively. Children whose mothers have NS had significantly lower neurodevelopmental scores than those of NS-unaffected mothers in fine motor (37.5(12.1) vs 44.2(14.3), p = 0.011), receptive language (37.8(10.8) vs 43.9(12.9), p = 0.010), overall cognitive development (74.36(17.8) vs 83.34(19.6), p = 0.015), and attention (0.64(0.20) vs 0.76(0.15), p = 0.001). There were no differences in the behaviour scores. Children of mothers with NS also had significantly lower weight-for-age z scores (WAZ) (p = 0.003) and length/height-for-age z scores (LAZ/HAZ) (p = 0.001); with 19(35.9 %) of them stunted.

Conclusion

Children whose mothers have NS have poorer neurodevelopmental, nutritional, and physical growth outcomes. Interventions to improve outcomes in these children are warranted.
简介点头综合征(NS)是一种在儿童中发病的严重神经系统疾病,人们对其了解甚少。在乌干达,一些 NS 病例已成长为有生育能力的成年人。患有 NS 的母亲所生的婴儿可能容易出现神经发育障碍。患有 NS 的母亲在认知方面的缺陷可能会进一步影响对其子女的照顾,从而影响神经认知发育、身体发育和行为:本研究旨在确定母亲患有点头综合征的儿童在神经发育、行为、营养和身体发育方面的结果:2021 年 5 月至 2022 年 4 月在乌干达北部进行了一项横断面比较研究。母亲患有点头综合征的 0-5 岁儿童与母亲未患有点头综合征的 0-5 岁儿童进行了比较,两者的年龄、性别和居住地相匹配。采用标准化方法对神经发育、行为、营养和身体发育情况进行评估,并采用 t 检验对结果进行分组比较:共有 106 名儿童参加了研究。53名儿童的母亲患有NS,53名儿童的母亲未患有NS;他们的平均年龄分别为26.9(2.22)个月和27.5(2.12)个月。母亲患有 NS 的儿童在精细运动(37.5(12.1) vs 44.2(14.3),P = 0.011)、接受性语言(37.8(10.8) vs 43.9(12.9),p = 0.010)、整体认知发展(74.36(17.8) vs 83.34(19.6),p = 0.015)和注意力(0.64(0.20) vs 0.76(0.15),p = 0.001)。行为评分方面没有差异。母亲患有 NS 的儿童的年龄体重 z 分数(WAZ)(p = 0.003)和年龄身长/身高 z 分数(LAZ/HAZ)(p = 0.001)也明显较低;其中有 19 人(35.9%)发育不良:结论:母亲患有 NS 的儿童在神经发育、营养和体格生长方面都较差。结论:母亲患有 NS 的儿童在神经发育、营养和身体发育方面都较差,因此有必要采取干预措施来改善这些儿童的发育状况。
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引用次数: 0
Long-term observation and mortality of children with nodding syndrome in western Uganda, 1994 – 2018 1994-2018年乌干达西部点头综合征儿童的长期观察结果和死亡率。
IF 2.8 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2026-02-01 Epub Date: 2024-06-26 DOI: 10.1016/j.seizure.2024.06.019
Christoph Kaiser , Walter Kipp , Nolbert Gumisiriza , George Asaba

Purpose

To assess long-term mortality and causes of death in children with nodding syndrome, an epileptic disorder of sub-Sahara Africa.

Methods

Ten children with nodding syndrome were followed over 24 years. The mortality rate was determined as the number of deaths per 1000 person-years of observation. The standard mortality ratio (SMR) was calculated as the number of observed deaths divided by the number of expected deaths in the general population. Patients were started on phenobarbital and treatment response was monitored during the first 20 months of follow-up.

Results

During an observation period of 89.8 person-years, eight patients had died, one patient was found alive, and one patient had been lost to follow-up. This corresponded to a mortality rate of 89.1 deaths per 1000 person-years and a SMR of 21.4 (95 % CI 6.6–36.2). Five deaths were related to status epilepticus, in two cases occurring after inadvertent drug withdrawal. All patients responded on phenobarbital with a reduction of seizure frequency but only four reached a seizure-free period of at least 6 months.

Conclusions

This long-term follow-up demonstrated high mortality in patients with nodding syndrome. Anti-seizure treatment with phenobarbital was of moderate efficacy. Abrupt interruption of phenobarbital was found leading to seizure aggravation, status epilepticus, and death. Our findings point out the importance of securing continuity of treatment access once anti-seizure therapy is included in health services in resource-poor settings. More rigorous observations and controlled studies are needed to improve the therapeutic options for nodding syndrome.
目的:评估撒哈拉以南非洲地区一种癫痫疾病--点头综合征患儿的长期死亡率和死亡原因:对 10 名患有点头综合征的儿童进行了长达 24 年的跟踪调查。死亡率按每 1000 人观察年的死亡人数确定。标准死亡率(SMR)的计算方法是:观察到的死亡人数除以普通人群的预期死亡人数。患者开始服用苯巴比妥,并在最初 20 个月的随访期间监测治疗反应:在 89.8 人年的观察期内,8 名患者死亡,1 名患者存活,1 名患者失去随访。这相当于每千人年的死亡率为 89.1 例,SMR 为 21.4 (95 % CI 6.6-36.2)。五例死亡与癫痫状态有关,其中两例是在不慎停药后发生的。所有患者服用苯巴比妥后癫痫发作频率都有所下降,但只有四名患者在至少 6 个月的时间内没有癫痫发作:这项长期随访表明,点头综合征患者的死亡率很高。使用苯巴比妥抗癫痫治疗的疗效一般。突然中断苯巴比妥可导致癫痫发作加重、癫痫状态和死亡。我们的研究结果表明,在资源匮乏的环境中,一旦抗癫痫治疗被纳入医疗服务,确保治疗连续性的重要性不言而喻。需要进行更严格的观察和对照研究,以改进对点头综合征的治疗方案。
{"title":"Long-term observation and mortality of children with nodding syndrome in western Uganda, 1994 – 2018","authors":"Christoph Kaiser ,&nbsp;Walter Kipp ,&nbsp;Nolbert Gumisiriza ,&nbsp;George Asaba","doi":"10.1016/j.seizure.2024.06.019","DOIUrl":"10.1016/j.seizure.2024.06.019","url":null,"abstract":"<div><h3>Purpose</h3><div>To assess long-term mortality and causes of death in children with nodding syndrome, an epileptic disorder of sub-Sahara Africa.</div></div><div><h3>Methods</h3><div>Ten children with nodding syndrome were followed over 24 years. The mortality rate was determined as the number of deaths per 1000 person-years of observation. The standard mortality ratio (SMR) was calculated as the number of observed deaths divided by the number of expected deaths in the general population. Patients were started on phenobarbital and treatment response was monitored during the first 20 months of follow-up.</div></div><div><h3>Results</h3><div>During an observation period of 89.8 person-years, eight patients had died, one patient was found alive, and one patient had been lost to follow-up. This corresponded to a mortality rate of 89.1 deaths per 1000 person-years and a SMR of 21.4 (95 % CI 6.6–36.2). Five deaths were related to status epilepticus, in two cases occurring after inadvertent drug withdrawal. All patients responded on phenobarbital with a reduction of seizure frequency but only four reached a seizure-free period of at least 6 months.</div></div><div><h3>Conclusions</h3><div>This long-term follow-up demonstrated high mortality in patients with nodding syndrome. Anti-seizure treatment with phenobarbital was of moderate efficacy. Abrupt interruption of phenobarbital was found leading to seizure aggravation, status epilepticus, and death. Our findings point out the importance of securing continuity of treatment access once anti-seizure therapy is included in health services in resource-poor settings. More rigorous observations and controlled studies are needed to improve the therapeutic options for nodding syndrome.</div></div>","PeriodicalId":49552,"journal":{"name":"Seizure-European Journal of Epilepsy","volume":"135 ","pages":"Pages 123-128"},"PeriodicalIF":2.8,"publicationDate":"2026-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141499452","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 Pub Date : 2026-02-01 Epub 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,&nbsp;Miray Atacan Yaşgüçlükal,&nbsp;Cengiz Yalçınkaya,&nbsp;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":"2026-02-01","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}
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Seizure-European Journal of Epilepsy
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