Seizure outcomes after resection of temporal encephalocele in patients with drug-resistant epilepsy: A systematic review and meta-analysis

耐药性癫痫患者颞叶脑膨出切除术后的癫痫发作结局：一项系统评价和荟萃分析

Temporal encephaloceles (TEs) are seen in patients with drug-resistant epilepsy (DRE); yet they are also common incidental findings. Variability in institutional pre-surgical epilepsy practices and interpretation of epileptogenic network localization contributes to bias in existing epilepsy cohorts with TE, and therefore the relevance of TE in DRE remains controversial. We sought to estimate effect sizes and sample sizes necessary to demonstrate clinically relevant improvements in seizure outcome with different surgical approaches. Retrospective cohort studies demonstrate good outcomes after TE resection regardless of the extent of resection. Prohibitively large sample sizes necessary to observe outcome differences between surgical approaches and presurgical predictors indicate that improved biomarkers and mechanistic understanding of TE epileptogenicity are needed.

颞叶脑膨出（TEs）可见于耐药性癫痫（DRE）患者；不过，它们也是常见的偶然发现。各机构术前癫痫诊疗实践的差异以及对致痫网络定位的不同解读，导致现有的合并颞叶脑膨出的癫痫队列研究存在偏差，因此颞叶脑膨出在耐药性癫痫中的相关性仍存在争议。我们试图估算采用不同手术方法在改善癫痫发作结局方面具有临床意义所需的效应量和样本量。回顾性队列研究显示，无论切除范围如何，切除颞叶脑膨出后预后良好。要观察不同手术方法及术前预测因素之间的预后差异，所需的样本量极大，这表明需要改进生物标志物，并加深对颞叶脑膨出致痫性机制的理解。

REF: Khoudari H, Alabbas M, Tobochnik S, Burneo J, Cox B, Lemus HN. Seizure outcomes after resection of temporal encephalocele in patients with drug-resistant epilepsy: A systematic review and meta-analysis. Epilepsia. 2025;66(4):945-954. doi:10.1111/epi.18264 PMID: 39817419

Antiseizure medication use in acute symptomatic seizures: A narrative review

急性症状性癫痫发作中的抗癫痫药物使用：一篇综述性述评 注：“narrative review”一般可译为“综述性述评” ，它是一种对某一主题的相关文献进行综合叙述和分析的文章类型。

Acute symptomatic seizures, occurring shortly after a central nervous system insult, constitute nearly half of all seizure cases. However, there is a conspicuous absence of clear, comprehensive, and cohesive guidelines for the management of these seizures with antiseizure medications, especially their duration of use. This lack of consensus on the optimal duration of therapy leads to prolonged treatments that may carry adverse consequences. The primary objective of this narrative review is to present the existing evidence-based literature on the management of acute symptomatic seizures within the context of the underlying pathologies that trigger them. We explore the risk of developing epilepsy for each specific etiology and identify the factors that influence this risk. Finally, to facilitate decision-making regarding treatment duration, we categorize acute seizures based on the temporal characteristics of hyperexcitability as acute, subacute, and prolonged. Such a rubric may offer clarity in an area where consensus and guidelines are lacking.

急性症状性癫痫发作是指在中枢神经系统受损后短期内发生的发作，这类发作几乎占所有癫痫发作病例的一半。然而，目前明显缺乏关于使用抗癫痫药物治疗这些发作的清晰、全面且连贯的指南，尤其是在用药时长方面。对于最佳治疗时长缺乏共识，导致治疗时间延长，而这可能会带来不良后果。 本述评的主要目的是结合引发急性症状性癫痫发作的潜在病理状况，介绍现有的基于循证医学的相关文献。我们将探讨每种特定病因引发癫痫的风险，并确定影响该风险的因素。最后，为了便于就治疗时长做出决策，我们根据过度兴奋性的时间特征，将急性发作分为急性、亚急性和持续性三类。在缺乏共识和指南的这一领域，这样的分类或许能带来一些清晰的思路。

REF: Yardi R, Vasireddy RP, Galovic M, Punia V. Antiseizure medication use in acute symptomatic seizures: A narrative review. Epilepsia. 2025;66(4):955-969. doi:10.1111/epi.18275 PMID: 39841056

The epilepsy–autism phenotype associated with developmental and epileptic encephalopathies: New mechanism-based therapeutic options

与发育性癫痫性脑病相关的癫痫 - 自闭症表型：基于新机制的治疗选择

Epilepsy and autism often co-occur in genetic developmental and epileptic encephalopathies (DEEs), but their underlying neurobiological processes remain poorly understood, complicating treatment. Advances in molecular genetics and understanding the neurodevelopmental pathogenesis of the epilepsy-autism phenotype may lead to mechanism-based treatments for children with DEEs and autism. Several genes, including the newly reported PPFIA3, MYCBP2, DHX9, TMEM63B, and RELN, are linked to various neurodevelopmental and epileptic disorders, intellectual disabilities, and autistic features. These findings underscore the clinical heterogeneity of genetic DEEs and suggest diverse neurobiological mechanisms influenced by genetic, epigenetic, and environmental factors. Mechanisms linking epilepsy and autism include γ-aminobutyric acidergic (GABAergic) signaling dysregulation, synaptic plasticity, disrupted functional connectivity, and neuroinflammatory responses. GABA system abnormalities, critical for inhibitory neurotransmission, contribute to both conditions. Dysregulation of the mechanistic target of rapamycin (mTOR) pathway and neuroinflammation are also pivotal, affecting seizure generation, drug resistance, and neuropsychiatric comorbidities. Abnormal synaptic function and connectivity further underscore the epilepsy-autism phenotype. New treatment options targeting specific mechanisms linked to the epilepsy-autism phenotype are emerging. Genetic variants in potassium channel genes like KCNQ2 and KCNT1 are frequent causes of early onset DEEs. Personalized treatments like retigabine and quinidine have been explored with heterogeneous responses. Efforts are ongoing to develop more effective KCNQ activators and KCNT1 blockers. SCN1A genetic variants, particularly in Dravet syndrome, show potential for treatment of autistic symptoms with low-dose clonazepam, fenfluramine, and cannabidiol, although human trials have yet to consistently replicate animal model successes. Early intervention before the age of 3 years, particularly in SCN1A- and tuberous sclerosis complex-related DEEs, is crucial. Additionally, targeting the mTOR pathway shows promise for seizure control and managing epilepsy-associated comorbidities. Understanding the distinct autism spectrum disorder phenotype in DEEs and implementing early behavioral interventions are essential for improving outcomes. Despite genetic advances, significant challenges persist in diagnosing and treating DEE-associated epilepsy-autism phenotypes. Future clinical trials should adopt precision health approaches to improve neurodevelopmental outcomes.

癫痫和自闭症常在遗传性发育性癫痫性脑病（DEEs）中并发，但其潜在的神经生物学过程仍不甚明晰，这给治疗带来了困难。分子遗传学的进展以及对癫痫 - 自闭症表型神经发育发病机制的深入理解，可能会为患有DEEs和自闭症的儿童带来基于机制的治疗方法。包括新报道的PPFIA3、MYCBP2、DHX9、TMEM63B和RELN在内的多个基因，与各种神经发育和癫痫性疾病、智力障碍以及自闭症特征相关。这些发现凸显了遗传性DEEs的临床异质性，并表明其受遗传、表观遗传和环境因素影响，存在多种神经生物学机制。 将癫痫和自闭症联系起来的机制包括γ - 氨基丁酸能（GABA能）信号传导失调、突触可塑性异常、功能连接中断和神经炎症反应。对于抑制性神经传递至关重要的GABA系统异常，会导致这两种病症的发生。雷帕霉素机制靶点（mTOR）通路失调和神经炎症也起着关键作用，影响癫痫发作的产生、药物耐药性以及神经精神共病。异常的突触功能和连接性进一步体现了癫痫 - 自闭症表型。 针对与癫痫 - 自闭症表型相关的特定机制的新治疗方案正在不断涌现。钾通道基因（如KCNQ2和KCNT1）中的遗传变异是早发性DEEs的常见原因。像瑞替加滨和奎尼丁等个性化治疗方法已被探索，但疗效存在异质性。目前正在努力开发更有效的KCNQ激活剂和KCNT1阻滞剂。SCN1A基因变异，特别是在大田原综合征中，低剂量氯硝西泮、芬氟拉明和大麻二酚显示出治疗自闭症症状的潜力，尽管人体试验尚未能始终如一地重现动物模型中的成功。3岁之前的早期干预，特别是对于与SCN1A和结节性硬化症复合体相关的DEEs，至关重要。此外，靶向mTOR通路在控制癫痫发作和管理癫痫相关共病方面显示出前景。 了解DEEs中独特的自闭症谱系障碍表型并实施早期行为干预，对于改善预后至关重要。尽管遗传学取得了进展，但在诊断和治疗DEE相关的癫痫 - 自闭症表型方面仍存在重大挑战。未来的临床试验应采用精准医疗方法，以改善神经发育结局。

REF: Specchio N, Di Micco V, Aronica E, et al. The epilepsy-autism phenotype associated with developmental and epileptic encephalopathies: New mechanism-based therapeutic options. Epilepsia. 2025;66(4):970-987. doi:10.1111/epi.18209 PMID: 39985505

New onset refractory status epilepticus: Long-term outcomes beyond seizures

新发难治性癫痫持续状态：癫痫发作之外的长期结局

We propose and prioritize important outcome domains that should be considered for future research investigating long-term outcomes (LTO) after new onset refractory status epilepticus (NORSE). The study was led by the international NORSE Institute LTO Working Group. First, literature describing the LTO of NORSE survivors was identified using a PubMed search and summarized to identify knowledge gaps. Subsequently, a consensus-building process was performed to prioritize and rank important LTO domains for further research. The prioritization of LTO domains was qualitative, enabling the expert panel to generate ideas, share opinions, and provide reasons for the rankings. A second round took place to allow expansion and agreement regarding specific details for each domain. Outcomes were classified into eight main domains: (1) Function: Neuropsychological, Neurological (other than seizures), and Psychiatric (mood and behavior); (2) Quality of Life; (3) Epilepsy; (4) Nonneurological (medical); (5) Social; (6) Caregiver Burden; (7) Long-Term Mortality; and (8) Health Care System Impact. In addition, the working group suggested obtaining outcome measures for each domain at 6 months and 1 year after discharge and annually thereafter until stability has been reached. There are no currently established time frames set for when LTO in NORSE begin or plateau, and previously there existed no consensus regarding which LTO should be considered. This consensus process identifies and recommends NORSE LTO domains that should be considered in future research studies to provide more consistent results that can be compared between studies. Survivors of NORSE should be evaluated serially and at fixed points over time to maximize our understanding of the recovery trajectory for all LTO domains. Establishing reliable and standardized data describing LTO (beyond seizures) after NORSE will support discussions with families during the acute stages, prognostication, the development of targeted management strategies for survivors, and future comparative research globally helping to identify biomarkers that may predict LTO.

我们提出并确定了重要结局领域的优先级，这些领域应在未来研究新发性难治性癫痫持续状态（NORSE）长期结局（LTO）时予以考虑。本研究由国际NORSE研究所长期结局工作组牵头开展。首先，通过PubMed搜索确定了描述NORSE幸存者长期结局的文献，并进行总结以找出知识空白。随后，开展了一项达成共识的流程，以确定并排列重要的长期结局领域，供进一步研究。长期结局领域的优先级确定是定性的，这使专家小组能够提出想法、分享观点，并为排名提供理由。进行了第二轮讨论，以就每个领域的具体细节展开拓展并达成一致。结局被分为八个主要领域：（1）功能：神经心理学、神经学（癫痫发作以外的情况）和精神科（情绪与行为）；（2）生活质量；（3）癫痫；（4）非神经学（医学方面）；（5）社会方面；（6）照顾者负担；（7）长期死亡率；（8）医疗系统影响。此外，工作组建议在出院后6个月和1年以及此后每年获取每个领域的结局指标，直至情况稳定。目前尚无关于NORSE长期结局何时开始或达到稳定期的确切时间框架，此前对于应考虑哪些长期结局也未达成共识。这一共识流程确定并推荐了未来研究中应考虑的NORSE长期结局领域，以便提供更具一致性的研究结果，从而在不同研究间进行比较。应定期并在固定时间点对NORSE幸存者进行评估，以最大限度地了解所有长期结局领域的康复轨迹。建立可靠且标准化的描述NORSE后长期结局（癫痫发作以外）的数据，将有助于在急性期与患者家属进行沟通、进行预后判断、为幸存者制定有针对性的管理策略，以及开展全球范围内的未来对比研究，有助于识别可能预测长期结局的生物标志物。

REF: Espino PH, Eschbach K, Blank LJ, et al. New onset refractory status epilepticus: Long-term outcomes beyond seizures. Epilepsia. 2025;66(4):988-1005. doi:10.1111/epi.18267 PMID: 39825688

When patients with Creutzfeldt–Jakob disease are misdiagnosed as having nonconvulsive status epilepticus

当克雅氏病（Creutzfeldt - Jakob disease）患者被误诊为非惊厥性癫痫持续状态时

Contemporary studies report nonconvulsive status epilepticus (NCSE) in Creutzfeldt-Jakob disease (CJD), based on benzodiazepine (BZP)-responsive epileptiform discharges on the electroencephalogram (EEG), with the following false syllogism: (1) intravenous (IV) administration of BZPs usually suppress ictal activity in NCSE; (2) in CJD, periodic sharp wave complexes (PSWCs) are suppressed by IV BZPs; (3) therefore, these patients have NCSE. This is a simplistic and invalid conclusion, because authors of 20th-century science reports have clearly shown that IV BZPs, short-acting barbiturates, and drugs with no antiseizure effects, such as chloral hydrate and IV naloxone, suppress PSWCs, but patients fall asleep with no clinical improvement. In contrast, IV methylphenidate transiently improves both the EEG and clinical states. Unlike NCSE, which is unlikely to be stopped by external stimuli, PSWCs can be transiently stopped by sensory or painful stimulation. Since the end of the 1970s, the effect of spontaneous sleep on the disappearance of PSWCs has been well documented, with a description of a cycling alternating pattern. Phase A features periodic discharges and is associated with increased arousal, whereas phase B exhibits a reduction or suppression of the PSWCs and is associated with a reduction in the arousal level and hypotonia (non-rapid eye movement sleep). When considering the use of IV BZP administration during EEG as a diagnostic test, the sequence of disappearance of PSWCs at sleep onset and reappearance after each stimulation or sleep apnea episode is compelling evidence against NCSE, as is the observation of a pattern of stimulus-induced wakefulness with transient improvement of the EEG. The cycling alternating pattern during sleep and reactivity to sensory or painful stimulation disappear with increasing disease severity; however, this occurs in the later stages of the disease, where there is no diagnostic doubt.

当代研究报告称克雅病（CJD）中存在非惊厥性癫痫持续状态（NCSE），其依据是脑电图（EEG）上出现对苯二氮䓬类药物（BZP）有反应的癫痫样放电，这存在如下错误的三段论推理：（1）静脉注射（IV）BZP通常可抑制NCSE的发作活动；（2）在CJD中，静脉注射BZP可抑制周期性尖波复合波（PSWC）；（3）因此，这些患者患有NCSE。这是一个简单且站不住脚的结论，因为20世纪的科研报告作者已明确指出，静脉注射BZP、短效巴比妥类药物以及诸如水合氯醛和静脉注射纳洛酮等无抗癫痫作用的药物均可抑制PSWC，但患者会入睡且临床症状并无改善。相反，静脉注射哌甲酯可使脑电图和临床状态得到短暂改善。与不太可能被外部刺激终止的NCSE不同，PSWC可被感觉或疼痛刺激短暂终止。自20世纪70年代末以来，已有充分文献记载了自然睡眠对PSWC消失的影响，并对循环交替模式进行了描述。A期的特征是周期性放电，且与觉醒增加有关；而B期表现为PSWC减少或受抑制，且与觉醒水平降低和肌张力低下（非快速眼动睡眠）有关。当将脑电图检查期间静脉注射BZP作为一种诊断性测试时，睡眠开始时PSWC消失以及每次刺激或睡眠呼吸暂停发作后PSWC再次出现的情况，有力地反驳了NCSE的诊断，脑电图因刺激而短暂改善所呈现的刺激诱导觉醒模式的观察结果同样如此。随着疾病严重程度的增加，睡眠期间的循环交替模式以及对感觉或疼痛刺激的反应性会消失；不过，这发生在疾病的后期阶段，此时诊断已无疑问。

REF: Gélisse P, Crespel A. When patients with Creutzfeldt-Jakob disease are misdiagnosed as having nonconvulsive status epilepticus. Epilepsia. 2025;66(4):1006-1013. doi:10.1111/epi.18259 PMID: 39754446

The effect of epilepsy surgery on tonic–clonic seizures

癫痫手术对强直 - 阵挛性发作的影响

Epilepsy surgery outcomes tend to be judged by the percentage in seizure reduction without considering the effect on specific seizure types, particularly tonic-clonic seizures, which produce the greatest morbidity and mortality. We assess how often focal to bilateral tonic-clonic seizures (BTCS) stop and how often they appear de novo after epilepsy surgery. Epilepsy surgery markedly reduces or eliminates BTCS, which should have a potential positive impact on morbidity and mortality. This favors offering surgery even if the chance of seizure freedom is not high and calls for a new surgical outcome scale to factor in seizure severity reduction.

癫痫手术效果往往以癫痫发作减少的百分比来评判，而未考虑对特定癫痫发作类型的影响，尤其是强直 - 阵挛性发作，这类发作造成的发病率和死亡率最高。我们评估了局灶性至双侧强直 - 阵挛性发作（BTCS）在癫痫手术后停止发作的频率，以及新发该类发作的频率。癫痫手术能显著减少或消除 BTCS，这可能会对降低发病率和死亡率产生积极影响。即便无癫痫发作的概率不高，也应考虑实施手术，并且需要制定一种新的手术效果评估量表，将癫痫发作严重程度的降低纳入考量。

REF: Alcala-Zermeno JL, Romozzi M, Sperling MR. The effect of epilepsy surgery on tonic-clonic seizures. Epilepsia. 2025;66(4):1048-1058. doi:10.1111/epi.18243 PMID: 39754525

Efficacy of neuromodulation of the pulvinar nucleus for drug-resistant epilepsy

丘脑枕核神经调控治疗耐药性癫痫的疗效

The pulvinar nucleus of the thalamus has extensive cortical connections with the temporal, parietal, and occipital lobes. Deep brain stimulation (DBS) targeting the pulvinar nucleus, therefore, carries the potential for therapeutic benefit in patients with drug-resistant posterior quadrant epilepsy (PQE) and neocortical temporal lobe epilepsy (TLE). Here, we present a single-center experience of patients managed via bilateral DBS of the pulvinar nucleus. In this first ever report on a series of patients undergoing bilateral pulvinar DBS for drug-resistant epilepsy, we demonstrate that stimulation of the pulvinar and in particular the MPN is a safe and viable option for patients with nonlesional PQE or TLE. The optimal target for stimulation and relative merits of open versus closed loop stimulation should be delineated in future studies.

丘脑枕核与颞叶、顶叶和枕叶存在广泛的皮质连接。因此，针对丘脑枕核的深部脑刺激（DBS）有望为药物难治性后象限癫痫（PQE）和新皮质颞叶癫痫（TLE）患者带来治疗益处。在此，我们介绍了单中心采用双侧丘脑枕核DBS治疗患者的经验。这是首篇关于一系列接受双侧丘脑枕核DBS治疗药物难治性癫痫患者的报告，我们证实，刺激丘脑枕核，尤其是丘脑枕内侧核（MPN），对于无病灶的PQE或TLE患者而言，是一种安全且可行的治疗选择。未来的研究应明确最佳的刺激靶点以及开环与闭环刺激的相对优势。

REF: Chandran AS, Joshi S, Suresh S, et al. Efficacy of neuromodulation of the pulvinar nucleus for drug-resistant epilepsy. Epilepsia. 2025;66(4):1059-1070. doi:10.1111/epi.18244 PMID: 39797738

User-defined virtual sensors: A new solution to the problem of temporal plus epilepsy sources

用户自定义虚拟传感器：一种解决颞叶癫痫源问题的新方案 注：这里“temporal plus epilepsy”可能表述有误，常见的是“temporal lobe epilepsy”（颞叶癫痫） ，你可根据实际情况确认。

The most common medically resistant epilepsy (MRE) involves the temporal lobe (TLE), and children designated as temporal plus epilepsy (TLE+) have a five-times increased risk of postoperative surgical failure. This retrospective, blinded, cross-sectional study aimed to correlate visual and computational analyses of magnetoencephalography (MEG) virtual sensor waveforms with surgical outcome and epilepsy classification (TLE and TLE+). This study demonstrates a concordance between UDvs beamforming and iEEG that is related to both postsurgical seizure outcome and presurgical classification of epilepsy (TLE and TLE+). UDvs beamforming could be a complementary approach to the well-established ECD, improving invasive electrode and surgical resection planning for patients undergoing epilepsy surgery evaluations and treatments.

最常见的药物难治性癫痫（MRE）累及颞叶（TLE），被诊断为颞叶附加型癫痫（TLE+）的儿童术后手术失败风险增加五倍。这项回顾性、盲法、横断面研究旨在将脑磁图（MEG）虚拟传感器波形的视觉分析和计算分析与手术结果及癫痫分类（TLE和TLE+）相关联。本研究显示，无偏导向矢量（UDvs）波束成形技术与颅内脑电图（iEEG）之间存在一致性，而这种一致性与术后癫痫发作结局以及术前癫痫分类（TLE和TLE+）均相关。UDvs波束成形技术可能是对成熟的等效电流偶极子（ECD）法的一种补充方法，有助于改善接受癫痫手术评估和治疗患者的侵入性电极置入及手术切除方案的规划。

REF: Tenney J, Fujiwara H, Skoch J, et al. User-defined virtual sensors: A new solution to the problem of temporal plus epilepsy sources. Epilepsia. 2025;66(4):1071-1083. doi:10.1111/epi.18247 PMID: 39740248

Favorable seizure and developmental outcomes without preoperative intracranial electroencephalography in pediatric patients following epilepsy surgery: A single epilepsy center retrospective study

小儿癫痫手术后未进行术前颅内脑电图监测仍获得良好的癫痫发作控制及发育结局：一项单癫痫中心回顾性研究

At our institute, most pediatric patients undergo epilepsy surgery following a thorough presurgical evaluation without intracranial electroencephalography (EEG). We conducted an initial validation of our noninvasive presurgical strategy by assessing the seizure and developmental outcomes of 135 children. Thorough noninvasive presurgical evaluation enables detection of subtle MRI lesions and curative epilepsy surgery without intracranial EEG in most patients, including those with FCD type II and type I, and leads to favorable seizure and developmental/neuropsychological outcomes.

在我们研究所，大多数儿科患者在经过全面的术前评估（不进行颅内脑电图[EEG]监测）后接受癫痫手术。我们通过评估135名儿童的癫痫发作情况和发育结局，对我们的无创术前评估策略进行了初步验证。全面的无创术前评估能够发现细微的磁共振成像（MRI）病变，使大多数患者（包括伴有II型和I型局灶性皮质发育不良[FCD]的患者）无需进行颅内脑电图监测即可接受治愈性癫痫手术，并且能带来良好的癫痫发作控制效果以及发育/神经心理学结局。

REF: Okumura T, Usui N, Kondo A, et al. Favorable seizure and developmental outcomes without preoperative intracranial electroencephalography in pediatric patients following epilepsy surgery: A single epilepsy center retrospective study. Epilepsia. 2025;66(4):1084-1096. doi:10.1111/epi.18249 PMID: 39729026