Epilepsia最新文献

{"title":"Bilateral centromedian nucleus of thalamus responsive neurostimulation for pediatric-onset drug-resistant epilepsy","authors":"Samuel Ahn,&nbsp;Benjamin Edmonds,&nbsp;Rajsekar R. Rajaraman,&nbsp;Lekha M. Rao,&nbsp;Shaun A. Hussain,&nbsp;Joyce H. Matsumoto,&nbsp;Raman Sankar,&nbsp;Noriko Salamon,&nbsp;Aria Fallah,&nbsp;Hiroki Nariai","doi":"10.1111/epi.18031","DOIUrl":"10.1111/epi.18031","url":null,"abstract":"<p>Neuromodulation therapies offer an efficacious treatment alternative for patients with drug-resistant epilepsy (DRE), particularly those unlikely to benefit from surgical resection. Here we present our retrospective single-center case series of patients with pediatric-onset DRE who underwent responsive neurostimulation (RNS) depth electrode implantation targeting the bilateral centromedian nucleus (CM) of the thalamus between October 2020 and October 2022. Sixteen patients were identified; seizure outcomes, programming parameters, and complications at follow-up were reviewed. The median age at implantation was 13 years (range 3.6–22). Six patients (38%) were younger than 12 years of age at the time of implantation. Ictal electroencephalography (EEG) patterns during patients’ most disabling seizures were reliably detected. Ten patients (62%) achieved 50% or greater reduction in seizure frequency at a median 1.3 years (range 0.6–2.6) of follow-up. Eight patients (50%) experienced sensorimotor side effects, and three patients (19%) had superficial pocket infection, prompting the removal of the RNS device. Side effects of stimulation were experienced mostly in monopolar-cathodal configuration and alleviated with programming change to bipolar configuration or low-frequency stimulation. Closed-loop neurostimulation using RNS targeting bilateral CM is a feasible and useful therapy for patients with pediatric-onset DRE.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11315622/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141285826","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":"Autonomic biosignals, seizure detection, and forecasting.","authors":"Gadi Miron, Mustafa Halimeh, Jesper Jeppesen, Tobias Loddenkemper, Christian Meisel","doi":"10.1111/epi.18034","DOIUrl":"https://doi.org/10.1111/epi.18034","url":null,"abstract":"<p><p>Wearable devices have attracted significant attention in epilepsy research in recent years for their potential to enhance patient care through improved seizure monitoring and forecasting. This narrative review presents a detailed overview of the current clinical state of the art while addressing how devices that assess autonomic nervous system (ANS) function reflect seizures and central nervous system (CNS) state changes. This includes a description of the interactions between the CNS and the ANS, including physiological and epilepsy-related changes affecting their dynamics. We first discuss technical aspects of measuring autonomic biosignals and considerations for using ANS sensors in clinical practice. We then review recent seizure detection and seizure forecasting studies, highlighting their performance and capability for seizure detection and forecasting using devices measuring ANS biomarkers. Finally, we address the field's challenges and provide an outlook for future developments.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":5.6,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260554","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":"Trends in management of patients with new-onset refractory status epilepticus (NORSE) from 2016 to 2023: An interim analysis","authors":"Aurelie Hanin,&nbsp;Anthony D. Jimenez,&nbsp;Margaret Gopaul,&nbsp;Hannah Asbell,&nbsp;Seyhmus Aydemir,&nbsp;Maysaa Merhi Basha,&nbsp;Ayush Batra,&nbsp;Charlotte Damien,&nbsp;Gregory S. Day,&nbsp;Onome Eka,&nbsp;Krista Eschbach,&nbsp;Safoora Fatima,&nbsp;Madeline C. Fields,&nbsp;Brandon Foreman,&nbsp;Elizabeth E. Gerard,&nbsp;Teneille E. Gofton,&nbsp;Hiba A. Haider,&nbsp;Stephen T. Hantus,&nbsp;Sara Hocker,&nbsp;Amy Jongeling,&nbsp;Mariel Kalkach Aparicio,&nbsp;Padmaja Kandula,&nbsp;Peter Kang,&nbsp;Karnig Kazazian,&nbsp;Marissa A. Kellogg,&nbsp;Minjee Kim,&nbsp;Jong Woo Lee,&nbsp;Lara V. Marcuse,&nbsp;Christopher M. McGraw,&nbsp;Wazim Mohamed,&nbsp;Janet Orozco,&nbsp;Cederic M. Pimentel,&nbsp;Vineet Punia,&nbsp;Alexandra M. Ramirez,&nbsp;Claude Steriade,&nbsp;Aaron F. Struck,&nbsp;Olga Taraschenko,&nbsp;Andrew K. Treister,&nbsp;Mark S. Wainwright,&nbsp;Ji Yeoun Yoo,&nbsp;Sahar Zafar,&nbsp;Daniel J. Zhou,&nbsp;Deepti Zutshi,&nbsp;Nicolas Gaspard,&nbsp;Lawrence J. Hirsch","doi":"10.1111/epi.18014","DOIUrl":"10.1111/epi.18014","url":null,"abstract":"<p>In response to the evolving treatment landscape for new-onset refractory status epilepticus (NORSE) and the publication of consensus recommendations in 2022, we conducted a comparative analysis of NORSE management over time. Seventy-seven patients were enrolled by 32 centers, from July 2016 to August 2023, in the NORSE/FIRES biorepository at Yale. Immunotherapy was administered to 88% of patients after a median of 3 days, with 52% receiving second-line immunotherapy after a median of 12 days (anakinra 29%, rituximab 25%, and tocilizumab 19%). There was an increase in the use of second-line immunotherapies (odds ratio [OR] = 1.4, 95% CI = 1.1–1.8) and ketogenic diet (OR = 1.8, 95% CI = 1.3–2.6) over time. Specifically, patients from 2022 to 2023 more frequently received second-line immunotherapy (69% vs 40%; OR = 3.3; 95% CI = 1.3–8.9)—particularly anakinra (50% vs 13%; OR = 6.5; 95% CI = 2.3–21.0), and the ketogenic diet (OR = 6.8; 95% CI = 2.5–20.1)—than those before 2022. Among the 27 patients who received anakinra and/or tocilizumab, earlier administration after status epilepticus onset correlated with a shorter duration of status epilepticus (<i>ρ</i> = .519, <i>p</i> = .005). Our findings indicate an evolution in NORSE management, emphasizing the increasing use of second-line immunotherapies and the ketogenic diet. Future research will clarify the impact of these treatments and their timing on patient outcomes.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260701","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":"Quantitative cellular pathology of the amygdala in temporal lobe epilepsy and correlation with magnetic resonance imaging volumetry, tissue microstructure, and sudden unexpected death in epilepsy risk factors","authors":"Hou Wang Lam,&nbsp;Smriti Patodia,&nbsp;Claudia Zeicu,&nbsp;Yau Mun Lim,&nbsp;Alicja Mrzyglod,&nbsp;Catherine Scott,&nbsp;Joana Oliveira,&nbsp;Jane De Tisi,&nbsp;Antoine Legouhy,&nbsp;Hui Zhang,&nbsp;Matthias Koepp,&nbsp;Beate Diehl,&nbsp;Maria Thom","doi":"10.1111/epi.18033","DOIUrl":"10.1111/epi.18033","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Amygdala enlargement can occur in temporal lobe epilepsy, and increased amygdala volume is also reported in sudden unexpected death in epilepsy (SUDEP). Apnea can be induced by amygdala stimulation, and postconvulsive central apnea (PCCA) and generalized seizures are both known SUDEP risk factors. Neurite orientation dispersion and density imaging (NODDI) has recently provided additional information on altered amygdala microstructure in SUDEP. In a series of 24 surgical temporal lobe epilepsy cases, our aim was to quantify amygdala cellular pathology parameters that could predict enlargement, NODDI changes, and ictal respiratory dysfunction.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using whole slide scanning automated quantitative image analysis methods, parallel evaluation of myelin, axons, dendrites, oligodendroglia, microglia, astroglia, neurons, serotonergic networks, mTOR-pathway activation (pS6) and phosphorylated tau (pTau; AT8, AT100, PHF) in amygdala, periamygdala cortex, and white matter regions of interest were compared with preoperative magnetic resonance imaging data on amygdala size, and in 13 cases with NODDI and evidence of ictal-associated apnea.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We observed significantly higher glial labeling (Iba1, glial fibrillary acidic protein, Olig2) in amygdala regions compared to cortex and a strong positive correlation between Olig2 and Iba1 in the amygdala. Larger amygdala volumes correlated with lower microtubule-associated protein (MAP2), whereas higher NODDI orientation dispersion index correlated with lower Olig2 cell densities. In the three cases with recorded PCCA, higher MAP2 and pS6-235 expression was noted than in those without. pTau did not correlate with SUDEP risk factors, including seizure frequency.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Histological quantitation of amygdala microstructure can shed light on enlargement and diffusion imaging alterations in epilepsy to explore possible mechanisms of amygdala dysfunction, including mTOR pathway activation, that in turn may increase the risk for SUDEP.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18033","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260634","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":"Long-term evaluation of anterior thalamic deep brain stimulation for epilepsy in the European MORE registry","authors":"Elisabeth Kaufmann,&nbsp;Jukka Peltola,&nbsp;Albert J. Colon,&nbsp;Kai Lehtimäki,&nbsp;Milan Majtanik,&nbsp;Jürgen K. Mai,&nbsp;Beata Bóné,&nbsp;Carla Bentes,&nbsp;Volker Coenen,&nbsp;Antonio Gil-Nagel,&nbsp;Antonio J. Goncalves-Ferreira,&nbsp;Philippe Ryvlin,&nbsp;Rod Taylor,&nbsp;Thomas C. Brionne,&nbsp;Frans Gielen,&nbsp;Shannon Song,&nbsp;Paul Boon,&nbsp;the MORE study group","doi":"10.1111/epi.18003","DOIUrl":"10.1111/epi.18003","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Short-term outcomes of deep brain stimulation of the anterior nucleus of the thalamus (ANT-DBS) were reported for people with drug-resistant focal epilepsy (PwE). Because long-term data are still scarce, the Medtronic Registry for Epilepsy (MORE) evaluated clinical routine application of ANT-DBS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>In this multicenter registry, PwE with ANT-DBS were followed up for safety, efficacy, and battery longevity. Follow-up ended after 5 years or upon study closure. Clinical characteristics and stimulation settings were compared between PwE with no benefit, improvers, and responders, that is, PwE with average monthly seizure frequency reduction rates of ≥50%.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Of 170 eligible PwE, 104, 62, and 49 completed the 3-, 4-, and 5-year follow-up, respectively. Most discontinuations (68%) were due to planned study closure as follow-up beyond 2 years was optional. The 5-year follow-up cohort had a median seizure frequency reduction from 16 per month at baseline to 7.9 per month at 5-year follow-up (<i>p</i> &lt; .001), with most-pronounced effects on focal-to-bilateral tonic–clonic seizures (<i>n</i> = 15, 77% reduction, <i>p</i> = .008). At last follow-up (median 3.5 years), 41% (69/170) of PwE were responders. Unifocal epilepsy (<i>p</i> = .035) and a negative history of epilepsy surgery (<i>p</i> = .002) were associated with larger average monthly seizure frequency reductions. Stimulation settings did not differ between response groups. In 179 implanted PwE, DBS-related adverse events (AEs, <i>n</i> = 225) and serious AEs (<i>n</i> = 75) included deterioration in epilepsy or seizure frequency/severity/type (33; 14 serious), memory/cognitive impairment (29; 3 serious), and depression (13; 4 serious). Five deaths occurred (none were ANT-DBS related). Most AEs (76.3%) manifested within the first 2 years after implantation. Activa PC depletion (<i>n</i> = 37) occurred on average after 45 months.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>MORE provides further evidence for the long-term application of ANT-DBS in clinical routine practice. Although clinical benefits increased over time, side effects occurred mainly during the first 2 years. Identified outcome modifiers can help inform PwE selection and management.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260633","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":"Excess prevalence of preexisting chronic conditions in older adults with incident epilepsy","authors":"Siran M. Koroukian,&nbsp;Hannah L. Fein,&nbsp;Long Vu,&nbsp;Wyatt P. Bensken,&nbsp;Nicholas K. Schiltz,&nbsp;Martha Sajatovic,&nbsp;Gena R. Ghearing,&nbsp;David F. Warner","doi":"10.1111/epi.18032","DOIUrl":"10.1111/epi.18032","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Prior studies have examined chronic conditions in older adults with prevalent epilepsy, but rarely among those with incident epilepsy. Identifying the chronic conditions with which older adults present at epilepsy incidence assists with the evaluation of disease burden in this patient population and informs coordinated care development. The aim of this study was to identify preexisting chronic conditions with excess prevalence in older adults with incident epilepsy compared to those without.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Using a random sample of 4 999 999 fee-for-service Medicare beneficiaries aged &gt;65 years, we conducted a retrospective cohort study of epilepsy incidence in 2019. Non-Hispanic Black and Hispanic beneficiaries were oversampled. We identified preexisting chronic conditions from the 2016–2018 Medicare Beneficiary Summary Files and compared chronic condition prevalence between Medicare beneficiaries with and without incident epilepsy in 2019. We characterized variations in preexisting excess chronic condition prevalence by age, sex, and race/ethnicity, adjusting for the racial/ethnic oversampling.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We observed excess prevalence of most preexisting chronic conditions in beneficiaries with incident epilepsy (<i>n</i> = 20 545, weighted <i>n</i> = 19 631). For stroke, for example, the adjusted prevalence rate ratio (APRR) was 4.82 (99% CI:4.60, 5.04), meaning that, compared to those without epilepsy, beneficiaries with incident epilepsy in 2019 had 4.82 times the stroke prevalence. Similarly, beneficiaries with incident epilepsy had a higher prevalence rate for preexisting neurological conditions (APRR = 3.17, 99% CI = 3.08–3.27), substance use disorders (APRR = 3.00, 99% CI = 2.81–3.19), and psychiatric disorders (APRR = 1.98, 99% CI = 1.94–2.01). For most documented chronic conditions, excess prevalence among beneficiaries with incident epilepsy in 2019 was larger for younger age groups compared to older age groups, and for Hispanic beneficiaries compared to both non-Hispanic White and non-Hispanic Black beneficiaries.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Compared to epilepsy-free Medicare beneficiaries, those with incident epilepsy in 2019 had a higher prevalence of most preexisting chronic conditions. Our findings highlight the importance of health promotion and prevention, multidisciplinary care, and elucidating shared pathophysiology to identify opportunities for prevention.</p>\u0000 </section>\u0000 <","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18032","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141260576","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":"WONOEP 2022: Neurotechnology for the diagnosis of epilepsy","authors":"Konrad Wagstyl,&nbsp;Katja Kobow,&nbsp;Pablo M. Casillas-Espinosa,&nbsp;Andrew J. Cole,&nbsp;Diego Jiménez-Jiménez,&nbsp;Hiroki Nariai,&nbsp;Stéphanie Baulac,&nbsp;Terence O'Brien,&nbsp;David C. Henshall,&nbsp;Ozlem Akman,&nbsp;Raman Sankar,&nbsp;Aristea S. Galanopoulou,&nbsp;Stéphane Auvin","doi":"10.1111/epi.18028","DOIUrl":"10.1111/epi.18028","url":null,"abstract":"<p>Epilepsy's myriad causes and clinical presentations ensure that accurate diagnoses and targeted treatments remain a challenge. Advanced neurotechnologies are needed to better characterize individual patients across multiple modalities and analytical techniques. At the XVIth Workshop on Neurobiology of Epilepsy: Early Onset Epilepsies: Neurobiology and Novel Therapeutic Strategies (WONOEP 2022), the session on “advanced tools” highlighted a range of approaches, from molecular phenotyping of genetic epilepsy models and resected tissue samples to imaging-guided localization of epileptogenic tissue for surgical resection of focal malformations. These tools integrate cutting edge research, clinical data acquisition, and advanced computational methods to leverage the rich information contained within increasingly large datasets. A number of common challenges and opportunities emerged, including the need for multidisciplinary collaboration, multimodal integration, potential ethical challenges, and the multistage path to clinical translation. Despite these challenges, advanced epilepsy neurotechnologies offer the potential to improve our understanding of the underlying causes of epilepsy and our capacity to provide patient-specific treatment.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18028","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141199850","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":"Imbalance between hippocampal projection cell and parvalbumin interneuron architecture increases epileptic susceptibility in mouse model of methyl CpG binding protein 2 duplication syndrome","authors":"Junye Ge,&nbsp;Shengjun Xie,&nbsp;Jiamei Duan,&nbsp;Biqing Tian,&nbsp;Pengfei Ren,&nbsp;Erling Hu,&nbsp;Qiyi Huang,&nbsp;Honghui Mao,&nbsp;Yuxin Zou,&nbsp;Qian Chen,&nbsp;Wenting Wang","doi":"10.1111/epi.18027","DOIUrl":"10.1111/epi.18027","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Methyl CpG-binding protein 2 (MECP2) duplication syndrome is a rare X-linked genomic disorder affecting predominantly males, which is usually manifested as epilepsy and autism spectrum disorder (ASD) comorbidity. The transgenic line MeCP2^Tg1 was used for mimicking MECP2 duplication syndrome and showed autism–epilepsy co-occurrence. Previous works suggested that the excitatory/inhibitory (E/I) imbalance is a potential common mechanism for both epilepsy and ASD. The projection neurons and parvalbumin (PV) interneurons account for the majority of E/I balance in the hippocampus. Therefore, we explored how structural changes of projection and PV⁺ neurons occur in the hippocampus of MeCP2^Tg1 mice and whether these morphological changes contribute to epilepsy susceptibility.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We used the interneuron Designer receptors exclusively activated by designer drugs mouse model to inhibit inhibitory neurons in the hippocampus to verify the epilepsy susceptibility of MeCP2^Tg1 (FVB, an inbred strain named as sensitivity to Friend leukemia virus) mice. Electroencephalograms were recorded for the definition of seizure. We performed retro-orbital injection of virus in MeCP2^Tg1 (FVB):CaMKIIα-Cre (C57BL/6) mice or MeCP2^Tg1:PV-Cre (C57BL/6) mice and their littermate controls to specifically label projection and PV⁺ neurons for structural analysis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Epilepsy susceptibility was increased in MeCP2^Tg1 mice. There was a reduced number of PV neurons and reduced dendritic complexity in the hippocampus of MeCP2^Tg1 mice. The dendritic complexity in MeCP2^Tg1 mice was increased compared to wild-type mice, and total dendritic spine density in dentate gyrus of MeCP2^Tg1 mice was also increased. Total dendritic spine density was increased in CA1 of MeCP2^Tg1 mice.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Overexpression of MeCP2 may disrupt crucial signaling pathways, resulting in decreased dendritic complexity of PV interneurons and increased dendritic spine density of projection neurons. This reciprocal modulation of excitatory and inhibitory neuronal structures associated with MeCP2 implies its significance as a potential target in the development of epilepsy and offers a novel perspective on the co-occurrence of autism and epilepsy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141178609","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 “Fenfluramine provides clinically meaningful reduction in frequency of drop seizures in patients with Lennox–Gastaut syndrome: Interim analysis of an open-label extension study”","authors":"","doi":"10.1111/epi.17999","DOIUrl":"10.1111/epi.17999","url":null,"abstract":"<p>Knupp KG, Scheffer IE, Ceulemans B, et al. Fenfluramine provides clinically meaningful reduction in frequency of drop seizures in patients with Lennox–Gastaut syndrome: Interim analysis of an open-label extension study. <i>Epilepsia</i>. 2023;64(1):139–151. doi:10.1111/epi.17431</p><p>In Paragraph 2 on page 145 of Section 3.3 (“Effectiveness”) of the “Results” section, the following sentences contained data describing typical, non-ESC-confirmed drop seizures:</p><p>“In the overall treatment period, median percentage increase in days free of drop seizures was 41.6% compared to pretreatment baseline in the core RCT, with a median absolute change of 1.93 days per 28 days (range = −23.0 to 27.5 days; <i>p</i> &lt; .0001). The longest interval between drop seizures increased from a median of 2.0 days (range = 1–17 days) to a median of 6.0 days (range = 1–365 days).”</p><p>The data ‘41.6%’, ‘1.93 days’, ‘27.5 days’, and ‘6.0 days’ needs to be updated for consistency with the data reported in the rest of the manuscript. The data in this paragraph should be updated to ESC-confirmed drop seizures, as shown below:</p><p>“In the overall treatment period, median percentage increase in days free of drop seizures was 44.6% compared to pretreatment baseline in the core RCT, with a median absolute change of 2.03 days per 28 days (range = −23.0 to 27.8 days; <i>p</i> &lt; .0001). The longest interval between drop seizures increased from a median of 2.0 days (range = 1–17 days) to a median of 7.0 days (range = 1–365 days).”</p><p>The data ‘41.6%’ becomes ‘44.6%’, ‘1.93 days’ becomes ‘2.03 days’, ‘27.5 days’ becomes ‘27.8 days’, and ‘6.0 days’ becomes ‘7.0 days’.</p><p>In Table 2 on page 144 of Section 3.2 of the “Results”, the text “.3 to .5” should be “.3 to &lt;.5”. In the next row, the text “&gt;.5 to .7” should be “≥.5 to .7”.</p><p>In the first paragraph of Section 3.2 of the “Results” on page 144, line 5, the text “.3 - .5 mg/kg/day” should be “.3 - &lt;.5 mg/kg/day”.</p><p>We confirm that these corrections do not change the interpretation of our results. We apologize for these errors.</p>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.17999","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141174232","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":"Cannabidiol-enriched oil for adult patients with drug-resistant epilepsy: Prospective clinical and electrophysiological study","authors":"Sigal Glatt,&nbsp;Sophie Shohat,&nbsp;Mor Yam,&nbsp;Lilach Goldstein,&nbsp;Inbal Maidan,&nbsp;Firas Fahoum","doi":"10.1111/epi.18025","DOIUrl":"10.1111/epi.18025","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Cannabidiol-enriched oil (CBDO) is being used increasingly to improve seizure control in adult patients with drug-resistant epilepsy (DRE), despite the lack of large-scale studies supporting its efficacy in this patient population. We aimed to assess the effects of add-on CBDO on seizure frequency as well as on gait, cognitive, affective, and sleep-quality metrics, and to explore the electrophysiological changes in responder and non-responder DRE patients treated with add-on CBDO.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We prospectively recruited adult DRE patients who were treated with add-on CBDO. Patients were evaluated prior to treatment and following 4 weeks of a maintenance daily dose of ≈260 mg CBD and ≈12 mg Δ9-tetrahydrocannabinol (THC). The outcome measures included seizure response to CBDO (defined as ≥50% decrease in seizures compared to pre-CBDO baseline), gait testing, Montreal Cognitive Assessment (MoCA), Hospital Anxiety and Depression Scale (HADS), and sleep-quality questionnaire assessments. Patients underwent electroencephalography (EEG) recording during rest as well as event-related potentials (ERPs) during visual Go/NoGo task while sitting and while walking.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Nineteen patients were recruited, of which 16 finished pre- and post-CBDO assessments. Seven patients (43.75%) were responders demonstrating an average <i>reduction</i> of 82.4% in seizures, and nine patients (56.25%) were non-responders with an average seizure <i>increase</i> of 30.1%. No differences in demographics and clinical parameters were found between responders and non-responders at baseline. However, responders demonstrated better performance in the dual-task walking post-treatment (<i>p</i> = .015), and correlation between increase in MoCA and seizure reduction (<i>r</i> = .810, <i>p</i> = .027). Post-CBDO P300 amplitude was lower during No/Go-sitting in non-responders (<i>p</i> = .028) and during No/Go-walking in responders (<i>p</i> = .068).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>CBDO treatment can reduce seizures in a subset of patients with DRE, but could aggravate seizure control in a minority of patients; yet we found no specific baseline clinical or electrophysiological characteristics that are associated with response to CBDO. However, changes in ERPs in response to treatment could be a promising direction to better identify patients who could benefit from CBDO treatment.</p>\u0000 </section>\u0000 </div>","PeriodicalId":11768,"journal":{"name":"Epilepsia","volume":null,"pages":null},"PeriodicalIF":6.6,"publicationDate":"2024-05-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/epi.18025","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141160107","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}