Epileptic Disorders最新文献

{"title":"Practical management of repeated life-threatening status epilepticus in Alternating Hemiplegia of Childhood: Case report and literature review.","authors":"Ramona Cordani, Livia Pisciotta, Michela Stagnaro, Maria Margherita Mancardi, Elisabetta Lampugnani, Luca Manfredini, Lino Nobili, Edvige Veneselli, Elisa De Grandis","doi":"10.1002/epd2.70031","DOIUrl":"https://doi.org/10.1002/epd2.70031","url":null,"abstract":"<p><strong>Background: </strong>Alternating Hemiplegia of Childhood (AHC) is a severe channelopathy that manifests before 18 months of age, primarily caused by pathogenic variants in the ATP1A3 gene. It is characterized by recurrent and disabling episodes of plegia, dystonia, dysautonomia, along with chronic neurological features and cardiac arrhythmias. About 50% of AHC patients have epilepsy, and a subset of them may develop refractory or super-refractory status epilepticus. Currently, there are no specific available therapeutic guidelines, particularly for managing life-threatening paroxysmal and epileptic episodes in the Intensive Care Unit (ICU).</p><p><strong>Case report: </strong>This report aims to illustrate a comprehensive treatment approach and clinical management of a young boy with AHC and multiple episodes of super-refractory status epilepticus throughout his lifetime. The patient was diagnosed with AHC at the age of 6 months, carrying the most severe ATP1A3 gene mutation-p.Glu815Lys. Epileptic seizures started at 24 months of age, and after a relatively stable clinical course, he experienced a first super-refractory status epilepticus at the age of 16. Subsequently, he presented two additional episodes of status epilepticus and faced progressive neurological deterioration until death at the age of 20.</p><p><strong>Discussion: </strong>AHC management is a substantial challenge due to the coexistence of multiple paroxysmal events, epilepsy, chronic neurological impairments, and the possibility of cardiac abnormalities, including short QTc or cardiac rhythm complications. Tailored treatment and deep knowledge of this complex disease are imperative for effective management.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144063170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effect of stereo-EEG versus subdural EEG on functional and seizure outcome in pediatric and adult epilepsy surgery: A 21-year single-center experience.","authors":"Ahmed Gaballa, Christian G Bien, Philip Grewe, Anne Hagemann, Thilo Kalbhenn, Tilman Polster, Friedrich G Woermann, Thomas Cloppenborg","doi":"10.1002/epd2.70025","DOIUrl":"https://doi.org/10.1002/epd2.70025","url":null,"abstract":"<p><strong>Objective: </strong>(1) To compare use, benefits, and complications of stereo-EEG (SEEG) and subdural EEG (SD) in presurgical epilepsy candidates. (2) To evaluate the effectiveness of both methods in delineation of the epileptogenic zone (EZ) and guiding tailored resective surgery.</p><p><strong>Methods: </strong>We included patients with SEEG or SD evaluations in children and adults at the Bethel Epilepsy Centre, Germany, between 2000 and 2020. We retrospectively assessed epilepsy related parameters, main indication of iEEG (intracranial EEG, for identification of the EZ and functional mapping), complications, seizure, and functional outcome. The second objective was addressed by a subgroup analysis in patients with focal cortical dysplasia (FCD) and mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE) for delineation of the EZ.</p><p><strong>Results: </strong>Two hundred and nineteen iEEG explorations in 215 patients (age range: 1.3-64.6 years) were assessed (SEEG, n = 78; SD, n = 141). We observed a change in iEEG usage away from SD towards SEEG. In the SEEG group, the decision rate against surgery was higher (SEEG: 23.1% vs. SE: 3.5%, p < .001) and more patients achieved seizure freedom 2 years after surgery (SEEG: 67.8% vs. SD: 46.5%, p = .008). For all iEEG, complications were rare; symptomatic intracranial bleeding only occurred in the SD group (n = 6, 4.3%). In patients with FCD/MOGHE for delineation of the EZ, all patients were operated with favorable seizure freedom after SEEG, but with acceptance of planned neurological deficits (SEEG n = 8, SD n = 20, seizure freedom rate SEEG 87.5% vs. SD 30%, p = .011; neurological deficits: SEEG n = 3 planned motor deficits vs. SD n = 1 unplanned motor deficit due to ischemia).</p><p><strong>Significance: </strong>SEEG has largely replaced SD in iEEG for the identification of the EZ. It is better tolerated and allows more freedom of choice with respect to resective surgery, resulting in the better identification of surgical candidates and delineation of the EZ with favorable seizure outcomes.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144004290","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Surface-electromyography characteristics of clonic seizures with no scalp-EEG correlate: A comparative analysis with tremors.","authors":"Veena V Kumar, Akshaya R Sivaji, Shwetank Singh, Zachary Scicchitano, Brandy Woods, Roohi Katyal, Neel Fotedar","doi":"10.1002/epd2.70035","DOIUrl":"https://doi.org/10.1002/epd2.70035","url":null,"abstract":"<p><strong>Introduction: </strong>Clonic seizures are characterized by twitching movements at a frequency of 0.2-5 Hz. The clonic \"twitch\" is produced by a brief synchronized contraction of agonist and antagonist muscles, followed by a synchronized silent period. In this study, we aimed to compare the surface-electromyography (sEMG) characteristics of scalp-EEG negative clonic seizures with those of nonepileptic movements like tremors that can resemble clonic seizures.</p><p><strong>Methods: </strong>We retrospectively identified patients who were diagnosed with scalp-EEG negative clonic seizures or tremors. We only included patients (n = 6) who were monitored simultaneously with video-EEG and sEMG electrodes. sEMG was placed on agonist and antagonist muscles of the affected extremity using a standardized placement system developed at our institution. We analyzed the following characteristics of sEMG bursts: the relationship between agonist and antagonist muscles and the temporal evolution of burst duration, burst amplitude, and burst frequency.</p><p><strong>Results: </strong>The following sEMG characteristics were observed: (i) sEMG bursts and corresponding silent periods were synchronous between agonist and antagonist muscles in clonic seizures. In tremors, an alternating pattern was seen. (ii) sEMG burst amplitude increased during the first 10 s of clonic seizures. There was no significant change in tremors. (iii) sEMG burst duration increased from the beginning to end of clonic seizures. There was no significant change in tremors. (iv) sEMG burst frequency decreased from the beginning to end of clonic seizures due to increased burst and silent period duration. There was no consistent change in burst frequency in tremors. (v) sEMG burst duration of ≥250 ms was indicative of a clonic seizure with a >90% positive predictive value.</p><p><strong>Conclusions: </strong>Our study describes characteristic sEMG features of clonic seizures without scalp-EEG correlates, which can be used as an objective biomarker in distinguishing these from nonepileptic movements such as tremors.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144046718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Absence-to-bilateral-tonic-clonic seizure with focal features: A diagnostic challenge.","authors":"Alioth Guerrero-Aranda, Fabrizio A Mortola, Tania P Sánchez-Murguía, Fridha V Villalpando-Vargas","doi":"10.1002/epd2.70040","DOIUrl":"https://doi.org/10.1002/epd2.70040","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144043858","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thank you to our reviewers in 2024","authors":"Sándor Beniczky,&nbsp;Birgit Frauscher","doi":"10.1002/epd2.70017","DOIUrl":"https://doi.org/10.1002/epd2.70017","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":"27 2","pages":"141-143"},"PeriodicalIF":1.9,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143930281","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Reduction of interictal epileptic burden by pulsatile corticoid therapy in children with drug-resistant epilepsy-How stable is the effect?","authors":"Katharina Schiller, Tamir Avigdor, John Thomas, Daniel Mansilla, Chifaou Abdallah, Aline Kortas, Gabriele Unterholzner, Raluca Pana, Markus Rauchenzauner, Birgit Frauscher","doi":"10.1002/epd2.70036","DOIUrl":"https://doi.org/10.1002/epd2.70036","url":null,"abstract":"<p><strong>Objective: </strong>The systematic use of pulsatile corticosteroid therapy (PCT) in children with drug-resistant epilepsy has been shown to reduce epileptic activity. However, it remains unclear how long this effect will last. The objective of this study was therefore to evaluate the stability of the effect of PCT by assessing the interictal epileptic activity burden (% of electroencephalography (EEG) with interictal epileptic activity, IEA) one year after the end of treatment.</p><p><strong>Methods: </strong>The final study cohort consisted of 20 children (9 females; mean age 7.6 ± 3.5 years) with various drug-resistant surgically nonremediable epilepsies who underwent systematic treatment with PCT (20 mg/m² body surface per treatment cycle). EEG recordings during sleep and wakefulness were obtained at baseline, after PCT, and at one-year follow-up. IEA burden and sleep spindles (rate per minute) to evaluate sleep physiology were compared between the different time points.</p><p><strong>Results: </strong>IEA burden was significantly reduced after PCT treatment (4.9% [2.4-20.4] vs. .9% [.2-5.5], p = .005, d = -.47) and this effect continued to persist at follow-up (.9% [.2-5.5] vs. 2.7% [.2-7.4], p = .99, d = .02). At time of follow-up, 33.3% patients showed a relapse defined by an increase in IEA burden after an initial decrease of at least 50% through PCT. Fast spindle rate (12-16 Hz) tended to be higher after PCT (1.0 ± .8 vs. 1.6 ± .8, p = .08, d = .59) and remained stable between the end of PCT and follow-up (1.6 ± .8 vs. 1.6 ± .4, p = .98, d = .01).</p><p><strong>Significance: </strong>Our findings suggest that in the majority of patients, PCT led to long-lasting benefits not only by reducing epileptic activity but also by improving sleep, important for cognitive functions.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144056908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Localizing value of ictal hypersalivation/sialorrhea and ictal spitting: A systematic review.","authors":"Petia S Dimova, Yoana Milenova, Denitza Stoyanova, Petar Karazapryanov, Krassimir Minkin, Kaloyan Gabrovski","doi":"10.1002/epd2.70030","DOIUrl":"https://doi.org/10.1002/epd2.70030","url":null,"abstract":"<p><strong>Objective: </strong>We performed a systematic review on the localizing value of ictal hypersalivation and ictal spitting in focal epilepsy with the aim of summarizing the state-of-the-art anatomo-clinical correlations in the field and aiding the interpretation of ictal semiology within the framework of pre-surgical evaluation.</p><p><strong>Methods: </strong>We conducted a review and meta-analysis of the published evidence and reported the results according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) statement. We searched in PubMed for relevant articles using the following key words: (hyper)salivation, sialorrhea, drooling, spitting, insular, opercular, epilepsy, seizure, ictal, EEG, and surgery. We evaluated the risk of bias and the diagnostic accuracy quality of each publication using the Quality of Diagnostic Accuracy Studies (QUADAS)-2-adapted tool. We assessed the level of confidence (very high, high, moderate, and low) in the reported epileptogenic zone (EZ) based on the availability of findings from MRI, intracerebral EEG, and postoperative outcomes.</p><p><strong>Results: </strong>We reviewed the full text of 65 articles that fulfilled the criteria for evidence synthesis. Thirty-two studies and 11 case reports (173 patients) on ictal hypersalivation and 15 studies and 13 case reports (54 patients) on ictal spitting were eligible. Ictal hypersalivation was reported in approximately 11% of (mesio)-temporal and approximately 23% of insular, opercular, and insulo-opercular epilepsies with >70% high-confidence cases due to seizure-free postsurgical outcomes. Ictal spitting was very rarely observed in focal epilepsies, including mesio-temporal epilepsies (incidence of 1% and 1.6%, respectively), but was significantly associated with a nondominant mesio-temporal EZ.</p><p><strong>Significance: </strong>There is strong evidence that ictal hypersalivation is more common in insular, opercular, and insulo-opercular seizures than in temporal lobe seizures, with the mid-insula and central operculum most consistently identified as symptomatogenic zones. Ictal spitting is a rare complex motor automatism pointing to seizures originating in nondominant mesio-temporal structures.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144006631","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ILAE neuroimaging task force highlight: MRI detection of early life epilepsy caused by focal cortical dysplasia.","authors":"Nathan T Cohen, L Gilbert Vezina, Chima Oluigbo, Tayyba Anwar, John Archer, Boris C Bernhardt, Lorenzo Caciagli, Fernando Cendes, Yotin Chinvarun, Luis Concha, Paolo Federico, Eliane Kobayashi, Godwin Ogbole, Stefan Rampp, Anna Elisabetta Vaudano, Z Irene Wang, Shuang Wang, Gavin P Winston, William D Gaillard","doi":"10.1002/epd2.70038","DOIUrl":"https://doi.org/10.1002/epd2.70038","url":null,"abstract":"<p><p>The ILAE Neuroimaging Task Force aims to publish educational case reports that highlight basic aspects of neuroimaging in epilepsy consistent with the ILAE's educational mission. Here, we describe a case series of three neonates with focal cortical dysplasia (FCD)-related drug-resistant epilepsy who underwent surgical intervention. The purpose of this series is to demonstrate the difficulties of identifying FCD in this age group and to highlight the potential added value of arterial spin labeling MRI for delineation of the epileptogenic zone. This series also supports the importance of the 2019 ILAE recommendations for structural imaging in epilepsy, the 2009 ILAE recommendations on imaging infants and children with recent-onset epilepsy, and the 2022 ILAE consensus classification of FCD.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-05-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144024463","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Features of cognitive dysfunction in late-onset temporal lobe epilepsy.","authors":"Naoya Hasegawa, Natsumi Saito, Masafumi Fukuda","doi":"10.1002/epd2.70034","DOIUrl":"https://doi.org/10.1002/epd2.70034","url":null,"abstract":"<p><strong>Objective: </strong>A global increase in the aging population has resulted in more patients with late-onset epilepsy. Late-onset temporal lobe epilepsy (LOTLE) is one of the most common types of late-onset epilepsy and is frequently accompanied by memory impairments. Here, we investigated the relationships between memory function and clinical characteristics, including findings from long-term video-electroencephalograph (LVEEG) monitoring, in LOTLE.</p><p><strong>Methods: </strong>We evaluated patients who had been diagnosed with LOTLE and Underwent LVEEG and neuropsychological tests (e.g., the Wechsler Memory Scale-Revised [WMS-R]) before the introduction of anti-seizure medications at our epilepsy center. We then performed multiple regression analyses using patients' clinical characteristics, including LVEEG data, as the independent variables, and cognitive scores as the dependent variables.</p><p><strong>Results: </strong>We included 17 patients with LOTLE. The mean age at onset was 65.18 ± 8.35 (47-78) years old. Among the WMS-R domains, verbal memory (β = -.79, p < .001), visual memory (β = -.645, p = .005), general memory (β = -.838, p < .001), and attention (β = -1.334, p < .001) showed negative relationships with the number of seizures of left temporal origin captured by LVEEG. Furthermore, patients who experienced transient amnestic episodes had relatively better delayed recall scores at 30 min (β = -.606, p = .005).</p><p><strong>Significance: </strong>Our findings suggest that frequent seizures originating from the left temporal lobe impair various cognitive functions. They also indicate that the patterns of cognitive dysfunction in LOTLE may be divided into two types: one showing preserved delayed recall with transient epileptic amnesia, and one showing impaired delayed recall.</p>","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144051383","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction to \"Two familial cases of infantile epileptic spasms syndrome associated with UDP-glucose-6-dehydrogenase deficiency\".","authors":"","doi":"10.1002/epd2.70028","DOIUrl":"https://doi.org/10.1002/epd2.70028","url":null,"abstract":"","PeriodicalId":50508,"journal":{"name":"Epileptic Disorders","volume":" ","pages":""},"PeriodicalIF":1.9,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144065166","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}