Epilepsia最新文献

{"title":"A review of the putative antiseizure and antiepileptogenic mechanisms of action for soticlestat.","authors":"Shinichi Kondo, Venkatesha Murthy, Mahnaz Asgharnejad, Arturo Benitez, Kosuke Nakashima, Nicole Hawkins, H Steve White","doi":"10.1111/epi.18287","DOIUrl":"https://doi.org/10.1111/epi.18287","url":null,"abstract":"<p><p>Soticlestat (TAK-935) is a potent and selective inhibitor of cholesterol 24-hydroxylase (CYP46A1), an enzyme primarily expressed in the brain that catabolizes cholesterol to 24S-hydroxycholesterol (24HC). In the ELEKTRA phase II clinical trial, soticlestat reduced seizure frequency in patients with developmental and epileptic encephalopathies, and two phase III studies evaluating the safety and efficacy of soticlestat in Dravet syndrome (SKYLINE) and Lennox-Gastaut syndrome (SKYWAY) have recently been completed. The exact mechanism of action by which soticlestat exerts pharmacological benefits remains undetermined. In this review, we assess the available preclinical evidence and present a working hypothesis for the antiseizure effects of soticlestat. The data support three potential mechanisms of action: (1) normalization of the seizure threshold via reduction of 24HC levels in the brain; as 24HC acts as a potent and selective positive allosteric modulator of glutamate N-methyl-D-aspartate receptors, reduction of 24HC levels by soticlestat may lead to decreased hyperexcitability and elevated seizure thresholds; (2) restoration of glutamate sequestration from the synaptic cleft; accumulation of glutamate in the synaptic cleft enhances neural excitation and can contribute to neurotoxicity; soticlestat may inhibit conversion of cholesterol to 24HC in the membrane lipid raft microdomain and help to preserve it, consequently reducing excessive glutamate excitation; and (3) suppression of neuroinflammation via reduction of inflammatory cytokine release. These potential mechanisms of action warrant further investigation.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440333","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}

{"title":"Differential functional connectivity of amygdala in drug-resistant temporal lobe epilepsy.","authors":"Thandar Aung, Lilly W Tang, Jonathan Ho, Luke C Henry, Naoki Ikegaya, Michel Modo, Jorge Gonzalez Martinez","doi":"10.1111/epi.18317","DOIUrl":"https://doi.org/10.1111/epi.18317","url":null,"abstract":"<p><strong>Objective: </strong>Recent studies highlight the amygdala's crucial role in temporal lobe epilepsy (TLE), particularly in magnetic resonance imaging-negative cases and new TLE subtypes with structural amygdala changes. This study aims to investigate the electrophysiological properties and connectivity patterns of the amygdaloid complex in TLE patients using stereoelectroencephalography (SEEG).</p><p><strong>Methods: </strong>From March 2020 to December 2023, we collected data from nine patients with drug-resistant TLE who underwent SEEG with dual amygdala trajectories: dorsal amygdala (DA) targeting medial and central nuclei, and ventral amygdala (VA) targeting basal and lateral nuclei. We analyzed interictal and ictal activities, focusing on power spectral density, interictal epileptiform discharges (IEDs), and coherence between DA and VA regions and ipsilateral regions of the temporal-limbic network, including the hippocampus, superior temporal gyrus, entorhinal cortex, fusiform gyrus, orbitofrontal cortex, temporal pole, and ventral insula.</p><p><strong>Results: </strong>IEDs were significantly higher in the DA, particularly in patients with epileptogenic zones involving the amygdala. We identified frequency-dependent connectivity patterns between DA and VA with ipsilateral cortical areas of interest during ictal activity. The VA exhibited higher connectivity with the limbic network in a greater number of seizures, particularly with the hippocampal head, entorhinal cortex, fusiform gyrus, and temporal pole in delta and theta frequencies. In contrast, DA connectivity was mainly confined to the hippocampus in theta and high-gamma frequency ranges.</p><p><strong>Significance: </strong>Our findings reveal distinct functional connectivity patterns and potentially divergent roles of DA and VA in TLE. These insights could refine intracranial sampling protocols of the amygdaloid complex, guiding more precise strategies for resection and neuromodulation in TLE patients.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440355","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}

{"title":"Correction to \"Health care utilization of Hispanic/Latino veterans with epilepsy: A national population-based study\".","authors":"","doi":"10.1111/epi.18327","DOIUrl":"https://doi.org/10.1111/epi.18327","url":null,"abstract":"","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440345","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}

{"title":"Amitriptyline use in individuals with KCNQ2/3 gain-of-function variants: A retrospective cohort study.","authors":"Matthias De Wachter, Charissa Millevert, Joost Nicolai, Elisabeth Cats, Gerhard Kluger, Mathieu Milh, Robin Cloarec, Steffen Syrbe, Katrijn Arts, Katrien Jansen, Magdalena Krygier, Robert Smigiel, Stephane Auvin, Kern Olofson, Cathrine Elisabeth Gjerulfsen, Berten Ceulemans, Rikke S Møller, Allan Bayat, Sarah Weckhuysen","doi":"10.1111/epi.18310","DOIUrl":"https://doi.org/10.1111/epi.18310","url":null,"abstract":"<p><strong>Objective: </strong>Heterozygous gain-of-function (GOF) variants in KCNQ2 and KCNQ3, encoding the voltage-gated potassium channel subunits Kv7.2 and Kv7.3, lead to neurodevelopmental disorders for which no established treatments are available. Amitriptyline, an antidepressant, blocks Kv7.2/Kv7.3 and has previously been reported to be effective in a single individual with a KCNQ2 GOF variant. We designed a retrospective, single-arm, multicenter study to investigate the effects of amitriptyline in a real-world setting.</p><p><strong>Methods: </strong>We used a 7-point Likert scale to measure seizure frequency, clinical examination, motor function, alertness, skill acquisition, communication, mood, behavior, self-care, sleep, tiredness, and electroencephalogram at baseline, after a minimum of 6 weeks of intervention, and, if applicable, after discontinuation. Adverse events were assessed in all participants, and the effectiveness of the treatment was evaluated in 11 individuals who received a minimum dosage of .5 mg/kg/day for at least 6 weeks. Data were collected from October 2023 to August 2024.</p><p><strong>Results: </strong>Thirteen individuals, eight with a pathogenic KCNQ2 GOF variant and five with a pathogenic KCNQ3 GOF variant, were included. Nine were female, and the median age at start of amitriptyline was 7.1 years (range = 1.5-20 years). Eleven individuals received a minimum dosage of .5 mg/kg/day for at least 6 weeks. The median dosage of amitriptyline administered was 1 mg/kg/day, with a median treatment duration of 29 weeks. Although amitriptyline was ineffective in two individuals (18%), eight (72%) demonstrated at least minimal improvement in two or more domains, with improvements in alertness and communication being the most frequently reported. In those with reported improvements, amitriptyline was discontinued in four individuals, but continued improvements were seen, to the same or greater extent compared to treatment. The remaining five individuals are on continued treatment because of perceived benefits.</p><p><strong>Significance: </strong>Overall, the effect of amitriptyline remains unclear, and formal n-of-1 trials are needed to investigate the precise effects of amitriptyline in KCNQ GOF-related neurodevelopmental disorders.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440338","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}

{"title":"Supervised machine learning compared to large language models for identifying functional seizures from medical records.","authors":"Wesley T Kerr, Katherine N McFarlane, Gabriela Figueiredo Pucci, Danielle R Carns, Alex Israel, Lianne Vighetti, Page B Pennell, John M Stern, Zongqi Xia, Yanshan Wang","doi":"10.1111/epi.18272","DOIUrl":"https://doi.org/10.1111/epi.18272","url":null,"abstract":"<p><strong>Objective: </strong>The Functional Seizures Likelihood Score (FSLS) is a supervised machine learning-based diagnostic score that was developed to differentiate functional seizures (FS) from epileptic seizures (ES). In contrast to this targeted approach, large language models (LLMs) can identify patterns in data for which they were not specifically trained. To evaluate the relative benefits of each approach, we compared the diagnostic performance of the FSLS to two LLMs: ChatGPT and GPT-4.</p><p><strong>Methods: </strong>In total, 114 anonymized cases were constructed based on patients with documented FS, ES, mixed ES and FS, or physiologic seizure-like events (PSLEs). Text-based data were presented in three sequential prompts to the LLMs, showing the history of present illness (HPI), electroencephalography (EEG) results, and neuroimaging results. We compared the accuracy (number of correct predictions/number of cases) and area under the receiver-operating characteristic (ROC) curves (AUCs) of the LLMs to the FSLS using mixed-effects logistic regression.</p><p><strong>Results: </strong>The accuracy of FSLS was 74% (95% confidence interval [CI] 65%-82%) and the AUC was 85% (95% CI 77%-92%). GPT-4 was superior to both the FSLS and ChatGPT (p <.001), with an accuracy of 85% (95% CI 77%-91%) and AUC of 87% (95% CI 79%-95%). Cohen's kappa between the FSLS and GPT-4 was 40% (fair). The LLMs provided different predictions on different days when the same note was provided for 33% of patients, and the LLM's self-rated certainty was moderately correlated with this observed variability (Spearman's rho²: 30% [fair, ChatGPT] and 63% [substantial, GPT-4]).</p><p><strong>Significance: </strong>Both GPT-4 and the FSLS identified a substantial subset of patients with FS based on clinical history. The fair agreement in predictions highlights that the LLMs identified patients differently from the structured score. The inconsistency of the LLMs' predictions across days and incomplete insight into their own consistency was concerning. This comparison highlights both benefits and cautions about how machine learning and artificial intelligence could identify patients with FS in clinical practice.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440359","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}

{"title":"Portable ultra-low-field magnetic resonance imaging enables postictal seizure imaging.","authors":"Tobias Bauer, Hemmen Sabir, Tobias Baumgartner, Attila Rácz, Jan Pukropski, Mostafa Badr, Simon Olbrich, Annalena Lange, Justus Bisten, Anne Groteklaes, Nils C Lehnen, Fernando Cendes, Alexander Radbruch, Rainer Surges, Theodor Rüber","doi":"10.1111/epi.18273","DOIUrl":"https://doi.org/10.1111/epi.18273","url":null,"abstract":"<p><p>The detection of transient peri-ictal magnetic resonance imaging (MRI) abnormalities has been variable after epileptic seizures. The most common reason for this variability is that abnormalities may disappear if the interval between seizure and scan acquisition is prolonged using conventional high-field systems. Here, we deployed a portable ultra-low-field MRI system in the presurgical evaluation at the bedside of individuals with epilepsy. We hypothesized that this novel technology enables rapid postictal scans and reliably shows focal peri-ictal MRI abnormalities in the seizure onset zone. A .064-T Swoop Portable MR Imaging System was used. Postictally, an axial diffusion-weighted sequence was acquired. The interictal MRI consisted of the diffusion-weighted and three-dimensional T1-weighted sequences. Postictal-interictal difference maps of diffusion-weighted volumes were calculated. Three individuals were included. Two individuals with focal aware seizures scanned 29 s and 19 min after the seizure, respectively, showed focal restrictions in diffusivity in the seizure onset zone, and a third individual scanned 5 h 45 min after a focal to bilateral tonic-clonic seizure showed global restrictions of diffusivity. Portable ultra-low-field MRI opens a new line of inquiry with the aim to establish postictal seizure imaging as part of the presurgical evaluation of people with epilepsy.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440357","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}

{"title":"Cognitive and brain health in juvenile myoclonic epilepsy: Role of social determinants of health.","authors":"Aaron F Struck, Camille Garcia-Ramos, Vivek Prabhakaran, Veena Nair, Nagesh Adluru, Anusha Adluru, Dace Almane, Jana E Jones, Bruce P Hermann","doi":"10.1111/epi.18296","DOIUrl":"10.1111/epi.18296","url":null,"abstract":"<p><strong>Objective: </strong>Juvenile myoclonic epilepsy (JME) is a prevalent genetic generalized epilepsy with linked abnormalities in cognition, behavior, and brain structure. Well recognized is the potential for advancing understanding of the epigenetic contributions to the neurobehavioral complications of JME, but to date there has been no examination of the role of socioeconomic disadvantage in regard to the cognitive and brain health of JME, which is the focus of this investigation.</p><p><strong>Methods: </strong>Seventy-seven patients with JME and 44 unrelated controls underwent neuropsychological assessment, structural neuroimaging, and clinical interview to delineate epilepsy history and aspects of family status. The Area Deprivation Index characterized the presence and degree of neighborhood disadvantage, which was examined in relation to cognitive factor scores underlying a comprehensive neuropsychological test battery, academic metrics, integrity of brain structure, and family characteristics.</p><p><strong>Results: </strong>JME participants resided in neighborhoods associated with significantly more socioeconomic disadvantage, which was associated with significantly poorer performance across all three cognitive factor scores and reading fluency. JME was associated with significant reduction of total subcortical gray matter (GM) but not total cortical gray or white matter volumes. Among controls, participants residing in more advantaged areas exhibited increased volumes of total subcortical GM and diverse subcortical structures as well as areas of increased cortical thickness and volume in frontal/prefrontal regions, findings that were compromised or not evident in JME, raising the possibility of disease-related attenuation of socioeconomic advantage.</p><p><strong>Significance: </strong>Socioeconomic disadvantage in JME is associated with adverse effects on cognitive and academic status, whereas socioeconomic advantage in controls is associated with increased brain volumes and thickness, markers of brain health that were largely attenuated or absent in JME. The associations detected here argue for the need to better integrate the social determinants of health with genetic and epigenetic factors in advancing understanding of cognitive and brain health in JME.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143440342","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}

{"title":"Epilepsia – February 2025 announcements","authors":"","doi":"10.1111/epi.18290","DOIUrl":"https://doi.org/10.1111/epi.18290","url":null,"abstract":"&lt;p&gt;\u0000 \u0000 &lt;b&gt;ASEPA-ANZAN Pre-Congress EEG Teaching Course&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;18–19 February 2025&lt;/p&gt;&lt;p&gt;New Delhi, India&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;15th Asian &amp; Oceanian Epilepsy Congress&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;20–23 February 2025&lt;/p&gt;&lt;p&gt;New Delhi, India&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;18th Latin American Summer School on Epilepsy&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;7–15 March 2025&lt;/p&gt;&lt;p&gt;São Paulo, Brazil&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;1st Pediatrics Commission Symposium&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;21–24 March 2025&lt;/p&gt;&lt;p&gt;São Paulo, Brazil&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;7th ILAE School on EEG in the First Year of Life&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;26–27 April 2025&lt;/p&gt;&lt;p&gt;Sanya City, Hainan, China&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;ILAE School on Neuroimaging 2025&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;15–17 May 2025&lt;/p&gt;&lt;p&gt;Potsdam, Germany&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;5th African Epilepsy Congress&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;16–18 May 2025&lt;/p&gt;&lt;p&gt;Lusaka, Zambia&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;15th ILAE School for Neuropathology and Neuroimaging in Epilepsy&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;31 July - 3 August 2025&lt;/p&gt;&lt;p&gt;Campinas, São Paulo, Brazil&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;XVIII Workshop on Neurobiology of Epilepsy (WONOEP 2025)&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;25–29 August 2025&lt;/p&gt;&lt;p&gt;Portugal&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;36th International Epilepsy Congress&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;30 August - 3 September 2025&lt;/p&gt;&lt;p&gt;Lisbon, Portugal&lt;/p&gt;&lt;p&gt;\u0000 &lt;b&gt;2026&lt;/b&gt;\u0000 &lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;16th European Epilepsy Congress&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;5–9 September 2026&lt;/p&gt;&lt;p&gt;Athens, Greece&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;ILAE Eastern Mediterranean &amp; Africa webinaire&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;11 February 2025&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;Epilepsy and Autism&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;27 February 2025&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;Non-convulsive status epilepticus&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;28 February 2025&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;ILAE e-Forum: Diagnosis and classification of Developmental and Epileptic Encephalopathies (DEE)&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;22 April 2025&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;3rd International Online Course on Pathogenesis of Epilepsy&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;27 February - 29 May 2025&lt;/p&gt;&lt;p&gt;Online&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;World Neuroscience and Psychiatry Conference 2025 (WNPC25)&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;8–9 March 2025&lt;/p&gt;&lt;p&gt;Bangkok, Thailand&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;19th World Congress on Controversies in Neurology&lt;/b&gt;\u0000 \u0000 &lt;/p&gt;&lt;p&gt;20–22 March 2025&lt;/p&gt;&lt;p&gt;Prague, Czech Republic&lt;/p&gt;&lt;p&gt;\u0000 \u0000 &lt;b&gt;13th Trinational Conference o","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":"66 2","pages":"618-619"},"PeriodicalIF":6.6,"publicationDate":"2025-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18290","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143423852","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":"Evaluating the effectiveness of medical therapy in infantile epileptic spasms syndrome due to surgically-remediable lesions.","authors":"Avantika Singh, Aristides Hadjinicolaou, Christina Briscoe-Abath, Stephanie Donatelli, Catherine Salussolia, Nishtha Gupta, Alexandra Santana Almansa, Bo Zhang, Scellig Stone, Mark Libenson, Debopam Samanta, Jeffrey Bolton, Chellamani Harini","doi":"10.1111/epi.18291","DOIUrl":"https://doi.org/10.1111/epi.18291","url":null,"abstract":"<p><strong>Objective: </strong>This single-center retrospective study examined the response to initial standard therapy in children with infantile epileptic spasms syndrome (IESS) associated with surgically-remediable lesion and evaluated the risk factors for drug resistance. We assessed whether the failure of the first standard therapy for surgically-remediable IESS predicted eventual drug resistance.</p><p><strong>Methods: </strong>New-onset IESS with surgically-remediable lesions was included. Regression analysis was performed to identify risk factors for drug resistance. Kaplan-Meier survival analysis stratified by the response to first standard therapy was conducted to explore if earlier recognition of drug-resistant epilepsy (DRE) was possible.</p><p><strong>Results: </strong>We identified 61 patients (57% female) with IESS and surgically-remediable lesion (median follow-up of 52 months). First standard treatment started at a median of 15 days after IESS onset resulted in favorable initial response in 31%. Response rate to second standard therapy among those who failed first treatment was 53%, with an overall response rate to sequential standard therapy of 63.8%. Relapses (epileptic spasms/focal seizures) were frequent (59%). At last follow-up, 41% (n = 25) remained drug responsive. The cumulative proportion of survival free of drug resistance was 57% at 2 months, dropping to 38% at 36 months after IESS diagnosis. The odds for DRE increased with extensive magnetic resonance imaging (MRI) abnormality (odds ratio [OR] = 38.5) and congenital-structural abnormality (OR = 23.3) and decreased with older age at IESS onset (OR = 0.68). Kaplan-Meier survival curve differed significantly between responders and non-responders to first standard therapy (p = .02). In the drug-resistant group (n = 36), 34 underwent surgery with Engel class I outcome in 76.5%.</p><p><strong>Significance: </strong>Although two-thirds of surgically-remediable IESS exhibited an initial response to medical therapy, relapses were frequent. The majority progressed to DRE during follow-up, particularly those with younger age at IESS onset, congenital-structural etiology, or extensive MRI abnormalities. Patients at risk for DRE can be recognized early by the lack of response to first standard therapy (OR 4.15). Our findings can help reduce delays for surgical referral in patients with surgically-remediable IESS.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143406407","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}

{"title":"Clinical effectiveness, feasibility, acceptability, and usability of mobile health applications for epilepsy: A systematic review.","authors":"Evelyn Gotlieb, Shahab Marzoughi, Churl-Su Kwon, Michael Harmon, Maren Kimura, Ashley Truesdale, Chloe Sweetnam, Céline Soudant, Margaret H Downes, Neil A Busis, Benjamin R Kummer, Nathalie Jetté","doi":"10.1111/epi.18288","DOIUrl":"https://doi.org/10.1111/epi.18288","url":null,"abstract":"<p><p>Mobile applications are widely used in epilepsy, although their impact on clinical effectiveness (CE) and their feasibility, acceptability, and usability (FAU) remain unclear. We conducted a systematic review investigating CE and FAU of epilepsy mobile applications using MEDLINE and Embase from database inception to June 21, 2024. We followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses reporting standards. The protocol was registered on PROSPERO (CRD42019134848). In duplicate, we determined study quality using the Newcastle-Ottawa Quality Assessment Scale (NOQAS) and the Joanna Briggs Critical Appraisal Checklist (to determine eligibility for inclusion), risk of bias using the Cochrane Risk of Bias tool, and usability study quality using the 15-point Silva scale. We identified 8953 studies, of which 20 were included. Twelve (60.0%) addressed CE, nine (45.0%) acceptability, five (25.0%) usability, and eight (40.0%) feasibility. Five (25.0%) evaluated CE and FAU. Studies comprised prospective cohort (n = 9, 45.0%), pilot (n = 3, 15.0%), randomized controlled trial (n = 7, 35.0%), and pre/post (n = 1, 5.0%) designs. Most apps were used for self-management or to enhance education or communication between patients and providers. Cohort studies demonstrated fair quality (median NOQAS score = 5, interquartile range [IQR] = 5.0-5.8), whereas of seven randomized controlled trials, four (57.1%) had some concern for bias. Usability studies demonstrated high quality (median Silva score = 10, IQR = 10-11). Apps were predominantly intended for patient use (n = 9, 75.0%). Symptom reporting and medication management were the most common app targets in both CE and FAU studies (n = 8, 66.7%; n = 9, 69.2%), although FAU studies more frequently used monitoring or tracking (n = 10, 76.9%) and reminder setting (n = 10, 76.9%) than CE apps (n = 7, 58.3%). Investigations of application use most commonly studied CE and patient-facing apps. Additional high-quality evidence is necessary to evaluate the CE and FAU of app use in epilepsy to work toward the standardization of FAU metrics and development of implementation guidelines.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":" ","pages":""},"PeriodicalIF":6.6,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143406401","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}