EpilepsiaPub Date : 2026-04-01Epub Date: 2025-12-23DOI: 10.1002/epi.70063
Loan Samalens, Camille Beets, Clothilde Courivaud, Sarvenaz Keshmiri, Jean-François Adam, Paolo Pellicioli, Emmanuel Luc Barbier, Raphaël Serduc, Antoine Depaulis
{"title":"Synchrotron-generated microbeams as a radiosurgical alternative for drug-resistant epilepsies: Proof of concept in a mouse model of mesiotemporal lobe epilepsy.","authors":"Loan Samalens, Camille Beets, Clothilde Courivaud, Sarvenaz Keshmiri, Jean-François Adam, Paolo Pellicioli, Emmanuel Luc Barbier, Raphaël Serduc, Antoine Depaulis","doi":"10.1002/epi.70063","DOIUrl":"10.1002/epi.70063","url":null,"abstract":"<p><strong>Objective: </strong>One-third of patients with epilepsy, particularly those with mesial temporal lobe epilepsy (MTLE), remain resistant to medication. Resective surgery, the gold standard, is highly invasive and carries significant risks. Here, using a mouse model, we explored the potential of microbeam radiation therapy (MRT), a new technique based on the spatial microfractionation of high-flux X-rays, as a non-invasive alternative for treating MTLE.</p><p><strong>Methods: </strong>MTLE was modeled in male C57BL6/J mice via unilateral kainate injection in the dorsal hippocampus. Mice with magnetic resonance imaging (MRI)-validated hippocampal sclerosis (HS) were irradiated at the European Synchrotron Radiation Facility (ESRF) using MRT with either a single trajectory (peak doses = 125-500 Gy) or multiple trajectories (2 or 5 ports at a cumulated peak dose of 125 Gy at the target). Their focal seizures were then monitored with use of electroencephalography (EEG) during 8 weeks, upon which immunochemistry was performed to assess potential tissular toxicity.</p><p><strong>Results: </strong>Anteroposterior 1-port MRT significantly reduced EEG-recorded focal seizures at 125 and 250 Gy, in a dose-dependent manner. However, increased mortality was observed at 500 Gy. Immunohistolabeling revealed neuronal loss (revealed by NeuN staining), microgliosis (revealed by Iba1 straining), and astrogliosis (revealed by GFAP staining) limited to the microbeam tracks at all doses in the injected hippocampus and adjacent brain structures (e.g., cortex). Five-port MRT with a peak dose of 125 Gy at the target improved the antiepileptic effect, whereas no significant tissue alterations outside the microbeam tracks were detected by histological assessment.</p><p><strong>Significance: </strong>This proof-of-concept study highlights MRT as a promising non-invasive therapy for drug-resistant focal epilepsies with optimal peak doses of 125-250 Gy, and it suggests that distributing the dose through multiple angles optimizes the therapeutic effect. MRT could provide a safer alternative to surgery, warranting further investigations.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1614-1626"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075611/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145809611","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2025-12-24DOI: 10.1002/epi.70078
Noémie Donnard, Blandine Dozières-Puyravel, Katia Geraldes, Céline Perrot, Virginie Quéméner, Estelle Goujon, Pierre Truchy, Hala Nasser, François-Xavier Mauvais, Marion Danse, Stéphane Auvin
{"title":"Weaning from ketogenic diet therapy in children with epilepsy: Insights from a retrospective study.","authors":"Noémie Donnard, Blandine Dozières-Puyravel, Katia Geraldes, Céline Perrot, Virginie Quéméner, Estelle Goujon, Pierre Truchy, Hala Nasser, François-Xavier Mauvais, Marion Danse, Stéphane Auvin","doi":"10.1002/epi.70078","DOIUrl":"10.1002/epi.70078","url":null,"abstract":"<p><strong>Objective: </strong>This study was undertaken to describe weaning practices following ketogenic diet therapy (KDT) in children with epilepsy and to identify clinical factors associated with seizure exacerbation or antiseizure medication adjustments during or after weaning from KDT.</p><p><strong>Methods: </strong>This retrospective observational study examined patients who initiated and discontinued KDT between 2016 and 2022 at a tertiary epilepsy center. Patients with GLUT1 deficiency, ongoing KDT, or less than 1 year of follow-up postdiscontinuation were excluded. Clinical, electroencephalographic (EEG), magnetic resonance imaging, KDT response, weaning duration, and seizure outcomes were analyzed. Responders were defined by ≥50% seizure reduction. Seizure worsening and/or antiseizure medication (ASM) adjustments during weaning were the primary outcomes.</p><p><strong>Results: </strong>Among 57 evaluable patients, 49% were responders and 25% became seizure-free. During weaning, 62% experienced seizure exacerbation or required ASM modifications. Unfavorable outcomes were significantly associated with shorter KDT duration, shorter weaning periods, higher ASM burden, and abnormal preweaning EEG. Among responders, seizure worsening was not significantly linked to weaning speed alone. At 1 year, seizure freedom was more common in patients who did not experience any issue during the weaning (65% vs. 30%, p = .009).</p><p><strong>Significance: </strong>Our findings underscore the importance of individualized KDT discontinuation plans based on efficacy, EEG activity, and ASM burden. Although prolonged weaning was more common in responders, weaning duration alone did not predict outcomes. These results highlight the need for prospective studies to define optimal weaning strategies for KDT.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1817-1826"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075600/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145826990","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2025-12-17DOI: 10.1002/epi.70059
Zihan Wei, Xinpei Wang, Yonghong Liu, Hongmin Bai
{"title":"Brain-heart functional network dysfunction in temporal lobe epilepsy: A microstate-based analysis.","authors":"Zihan Wei, Xinpei Wang, Yonghong Liu, Hongmin Bai","doi":"10.1002/epi.70059","DOIUrl":"10.1002/epi.70059","url":null,"abstract":"<p><strong>Objective: </strong>Epilepsy patients face significantly elevated cardiovascular risks, with cardiac arrhythmias occurring 2-3 times more frequently than in the general population. Current knowledge of brain-heart functional coupling abnormalities in epilepsy, particularly during interictal periods, remains limited. We investigated brain-heart interplay characteristics in temporal lobe epilepsy through synchronized electroencephalographic-electrocardiographic analysis using a synthetic data generation model and microstate analysis.</p><p><strong>Methods: </strong>We enrolled 52 patients with temporal lobe epilepsy (mean age = 33.4 ± 12.7 years) and 42 age-matched healthy controls (mean age = 31.95 ± 11.03 years). Twenty-minute artifact-free electroencephalographic segments were analyzed during resting states. Four directional brain-heart coupling sequences were extracted: C<sub>Brain→HF</sub>, C<sub>Brain→LF</sub>, C<sub>HF→Brain</sub>, and C<sub>LF→Brain</sub>, representing bidirectional interactions between brain activity and cardiac components.</p><p><strong>Results: </strong>Six microstate topologies were consistently identified across all brain-heart interplay sequences, with temporal lobe epilepsy patients demonstrating significantly more complex and unstable topological characteristics compared to healthy controls. For C<sub>Brain→HF</sub> coupling, patients exhibited significantly reduced mean duration of microstate 3 (.34 ± .09 s vs. .38 ± .07 s, p = .02), increased occurrence rates of microstates 3 and 4 (both p < .001), and altered temporal coverage patterns. Similar abnormalities were observed across all sequences, with patients showing shortened microstate durations, altered occurrence rates, and disrupted temporal coverage. Spatial dissimilarity analysis revealed significant topological abnormalities across all microstates. A logistic regression model incorporating microstate parameters achieved 94.7% diagnostic accuracy for temporal lobe epilepsy, with an F1 score of .952 and an area under the curve of .932.</p><p><strong>Significance: </strong>Temporal lobe epilepsy is characterized by profound disruptions in brain-heart functional coupling during interictal periods, manifesting as altered microstate topographies and temporal dynamics. These findings establish microstate-based analysis as a promising framework for characterizing brain-heart axis dysfunction in epilepsy.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1837-1848"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145767450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2025-12-24DOI: 10.1002/epi.70065
Yingfan Wang, Minghao Li, Huijun Li, Wei Yu, Peilin Jiang, Xinyi Zhou, Ke Hu, Feng Yang, Jiu Chen, Ming Yang, Xiaoshan Wang
{"title":"Altered neurostructural development in magnetic resonance imaging-negative pediatric epilepsy: A large-scale multicenter study of 1919 children.","authors":"Yingfan Wang, Minghao Li, Huijun Li, Wei Yu, Peilin Jiang, Xinyi Zhou, Ke Hu, Feng Yang, Jiu Chen, Ming Yang, Xiaoshan Wang","doi":"10.1002/epi.70065","DOIUrl":"10.1002/epi.70065","url":null,"abstract":"<p><strong>Objective: </strong>Addressing the poorly understood impact of pediatric epilepsy on neurodevelopment, this large-scale study delineates age- and sex-stratified neurostructural trajectories in magnetic resonance imaging (MRI)-negative pediatric epilepsy to identify periods of maximal developmental divergence from healthy controls.</p><p><strong>Methods: </strong>In this multicenter, cross-sectional study, we analyzed T1-weighted MRI from 957 patients with MRI-negative epilepsy and 962 controls (aged 4-12 years). Generalized additive models for location, scale, and shape modeled sex-stratified developmental trajectories of global brain metrics. Voxel- and surface-based morphometry compared cortical morphology and regional gray matter volume (GMV) between groups across yearly age bins (familywise error-corrected p < .05).</p><p><strong>Results: </strong>Compared to controls, patients showed reduced total intracranial volume, GMV, cerebrospinal fluid volume, and cortical thickness and significantly increased white matter hyperintensity burden. Key findings on developmental trajectories include an atypical trajectory of total surface area, a premature cortical thickness peak at approximately age 7 years, and a white matter hyperintensity (WMH) burden peak at approximately age 8 years. From ages 4 to 9 years, patients displayed widespread cortical morphological delays, most prominently affecting limbic and sensorimotor networks, which appeared to normalize after age 10 years. Unlike the GMV atrophy seen in adults, pediatric patients showed limbic expansion (5-6 years), thalamic hypertrophy (9-12 years), and cerebellar volumetric shifts.</p><p><strong>Significance: </strong>Our findings indicate that pediatric epilepsy is a disorder of aberrant neurodevelopment with two distinct signatures. First, we identify a critical 4-9-year vulnerability window characterized by profound but transient deviations, including atypical cortical maturation, increased WMH burden, and widespread morphological delays. These delays appeared to normalize after age 10 years, a finding that requires longitudinal validation. Second, we uncover a progressive, potentially persistent alteration: a hierarchical expansion of gray matter initiating in the limbic system and later involving the thalamus. These signatures provide distinct biomarkers to differentiate transient disruption from ongoing network reorganization, offering new targets for timed interventions.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1873-1886"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075624/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145826982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2026-01-10DOI: 10.1002/epi.70098
Philip Lederer, Muhammet Dogan, Dorian Hirschmann, Beate Kranawetter, Anna Cho, Philipp Goebl, Christian Dorfer, Brigitte Gatterbauer, Karl Rössler, Philippe Dodier, Wei-Te Wang, Gerhard Bavinzski, Arthur Hosmann, Wolfgang Serles, Josa M Frischer
{"title":"Seizure control after radiosurgical treatment in patients with cerebral arteriovenous malformations: An observational study.","authors":"Philip Lederer, Muhammet Dogan, Dorian Hirschmann, Beate Kranawetter, Anna Cho, Philipp Goebl, Christian Dorfer, Brigitte Gatterbauer, Karl Rössler, Philippe Dodier, Wei-Te Wang, Gerhard Bavinzski, Arthur Hosmann, Wolfgang Serles, Josa M Frischer","doi":"10.1002/epi.70098","DOIUrl":"10.1002/epi.70098","url":null,"abstract":"<p><strong>Objective: </strong>Seizures are among the most common symptoms of cerebral arteriovenous malformations (AVMs). Although the main goal of AVM treatment remains complete obliteration, seizure control has become an important treatment aspect in recent years. Thus, we analyzed seizure control following Gamma Knife Radiosurgery (GKRS) treatment at our department in patients with AVMs.</p><p><strong>Methods: </strong>We analyzed 130 patients with AVMs who had at least one seizure before treatment with GKRS at our tertiary referral center between 1992 and 2022. We analyzed predictors for treatment success, including AVM size, Virginia Radiosurgery AVM Scale, Radiosurgery-Based AVM Score, Spetzler-Ponce class, Spetzler-Martin grade, localization, and identified independent predictors of seizure persistence.</p><p><strong>Results: </strong>Median follow-up was 9.7 years. Improved seizure control was documented for 89% of patients. Seizure-free status after radiosurgery was achieved for 78% of patients. Spetzler-Ponce class C (odds ratio [OR] = 4.6) and having multiple seizures before the first GKRS treatment (OR = 3.5) were independent predictors for seizure persistence. Still, 72% of patients with multiple seizures prior to AVM treatment were seizure-free at last follow-up. Among patients with multiple seizures, having a high-grade AVM was identified as an independent predictor of seizure persistence (OR = 12.1).</p><p><strong>Significance: </strong>GKRS, as a stand-alone management option or in combination with endovascular therapy, is an effective treatment option in AVM management, not only to achieve AVM obliteration but also to control AVM-related seizures. The Spetzler-Ponce class is a powerful predictor of seizure persistence at last follow-up, especially in patients with multiple seizures prior to treatment.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1627-1638"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075625/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145942938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2025-12-31DOI: 10.1002/epi.70083
Chi-Yuan Chang, Robert Moss, M Brandon Westover, Daniel M Goldenholz
{"title":"Deployable seizure forecasting requires clinically meaningful performance: Response to Stirling et al.","authors":"Chi-Yuan Chang, Robert Moss, M Brandon Westover, Daniel M Goldenholz","doi":"10.1002/epi.70083","DOIUrl":"10.1002/epi.70083","url":null,"abstract":"","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1587-1588"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12854151/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145862518","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2026-02-18DOI: 10.1002/epi.70154
Kaley J Marcinski Nascimento, Fábio A Nascimento, Torie Robinson, Birgit Frauscher, Eugen Trinka, Sándor Beniczky
{"title":"Persons with epilepsy and their caregivers understand the definition of ictal impairment of consciousness.","authors":"Kaley J Marcinski Nascimento, Fábio A Nascimento, Torie Robinson, Birgit Frauscher, Eugen Trinka, Sándor Beniczky","doi":"10.1002/epi.70154","DOIUrl":"10.1002/epi.70154","url":null,"abstract":"<p><p>The term consciousness has been reintroduced in the updated seizure classification. Concerns have been raised that \"ictal impaired consciousness\" may be misunderstood by persons with epilepsy (PWEs) and their caregivers, particularly that English-speaking individuals might equate it with complete loss of consciousness. We conducted an online survey distributed via epilepsy organizations and social media to assess whether English-speaking PWEs and caregivers could understand and apply a simple medical definition of consciousness: \"In medical terms, consciousness means being able to remember things and respond to what is happening around you. During a seizure, consciousness is considered affected if the person cannot remember what happened and if they are unable to respond normally when people try to interact with them.\" The survey was completed by 253 respondents (148 caregivers, 105 PWEs). Almost all participants (97%) found the definition clear, 90% reported they could apply it to their seizures, and 99% demonstrated comprehension by correctly interpreting an example question. No significant differences were observed between PWEs and caregivers, across education levels, or by seizure characteristics. These findings indicate that PWEs and caregivers can readily understand and apply the concept of ictal impairment of consciousness when given a concise, patient-friendly definition.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"e39-e45"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075620/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146219015","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2026-01-03DOI: 10.1002/epi.70079
Donald J Phillips, Autumn S Ivy, Samuel Guzman, Saman Hazany, Xiangmin Xu
{"title":"Spatial transcriptomics in epilepsy research: Early successes, opportunities, and challenges.","authors":"Donald J Phillips, Autumn S Ivy, Samuel Guzman, Saman Hazany, Xiangmin Xu","doi":"10.1002/epi.70079","DOIUrl":"10.1002/epi.70079","url":null,"abstract":"","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1589-1600"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075609/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145892455","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
EpilepsiaPub Date : 2026-04-01Epub Date: 2026-03-03DOI: 10.1002/epi.70170
Lena Bender, Wolf Lagrèze, Martin Hirsch, Andreas Schulze-Bonhage
{"title":"Lenses protecting against photosensitivity violate international driving regulations.","authors":"Lena Bender, Wolf Lagrèze, Martin Hirsch, Andreas Schulze-Bonhage","doi":"10.1002/epi.70170","DOIUrl":"10.1002/epi.70170","url":null,"abstract":"","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"2035-2037"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13075617/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147343842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The symmetrical claustrum sign in pediatric febrile infection-related epilepsy syndrome: Diagnostic value and clinical implications.","authors":"Xiaodi Han, Changhong Ren, Hua Cheng, Shuhua Chen, Jiuwei Li, Fang Fang, Changhong Ding, Xiaotun Ren, Hui Xiong, Weihua Zhang","doi":"10.1002/epi.70064","DOIUrl":"10.1002/epi.70064","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to assess the diagnostic value of symmetrical claustrum sign (SCS) in children with febrile infection-related epilepsy syndrome (FIRES), and describe the clinical features, treatment responses, and prognosis of children with FIRES accompanied by symmetrical claustrum lesions (FIRES-C).</p><p><strong>Methods: </strong>A retrospective cohort study conducted at Beijing Children's Hospital from January 2015 to April 2024 included children with \"fever and convulsions\" who met the inclusion criteria. The diagnostic efficacy of SCS was calculated by analyzing the sensitivity and specificity.</p><p><strong>Results: </strong>The study enrolled 1105 patients, including 129 with FIRES, 35 of whom were diagnosed with FIRES-C (median onset age: 8.3 years; 18 male). In the cohort of patients with status epilepticus (n = 347), the sensitivity of SCS was 27.1% (95% confidence interval [CI], 19.8%-35.9%), and the specificity was 95.4% (95% CI, 91.8%-97.4%). The median time to develop bilateral claustrum lesions was 15.8 days (interquartile range [IQR] 11-19). The results of the magnetic resonance imaging (MRI) scans revealed that 7 patients (20%) had isolated claustrum lesions, whereas 28 patients (80%) had extra-claustrum lesions. After a median follow-up of 42 months, 14 of the 29 patients (48.3%) who survived exhibited moderate to severe deficits (modified Rankin scale [mRS] ≥3) and 2 patients died. When compared to FIRES patients without claustrum lesions (n = 94), those with FIRES-C (n = 35) demonstrated a tendency toward milder disease severity, characterized by higher Glasgow Coma Scale (GCS) scores and lower incidence of non-midazolam anesthetic use and respiratory support requirements (p < .001). However, no significant differences in prognosis were observed between the two groups (p = .322).</p><p><strong>Significance: </strong>SCS exhibit high specificity but limited sensitivity in the diagnosis of FIRES in pediatric patients. SCS may serve as a biomarker to assist in the diagnostic process and is typically associated with a less severe clinical progression.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":"1737-1747"},"PeriodicalIF":6.6,"publicationDate":"2026-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145803058","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}