Epilepsia Open最新文献

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Refining management strategies for Lennox–Gastaut syndrome: Updated algorithms and practical approaches 改进lenox - gastaut综合征的管理策略:更新的算法和实用方法。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-19 DOI: 10.1002/epi4.13075
Stéphane Auvin, Alexis Arzimanoglou, Mercè Falip, Pasquale Striano, J. Helen Cross
{"title":"Refining management strategies for Lennox–Gastaut syndrome: Updated algorithms and practical approaches","authors":"Stéphane Auvin, Alexis Arzimanoglou, Mercè Falip, Pasquale Striano, J. Helen Cross","doi":"10.1002/epi4.13075","DOIUrl":"10.1002/epi4.13075","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>Lennox–Gastaut syndrome (LGS) is a severe developmental and epileptic encephalopathy (DEE) characterized by multiple types of drug-resistant seizures (which must include tonic seizures) with classical onset before 8 years (although some cases with later onset have also been described), abnormal electroencephalographic features, and cognitive and behavioral impairments. Management and treatment of LGS are challenging, due to associated comorbidities and the treatment resistance of seizures. A panel of five epileptologists reconvened to provide updated guidance and treatment algorithms for LGS, incorporating recent advancements in antiseizure medications (ASMs) and understanding of DEEs. The resulting consensus document is based on current evidence from clinical trials and clinical practice and the panel's expert opinion, focusing on new ASMs with novel mechanisms of action, such as highly purified cannabidiol and fenfluramine. For a patient presenting with newly diagnosed LGS or suspected LGS, the recommended first-line treatment continues to be valproate. If this is ineffective as monotherapy, adjunctive therapy with, firstly, lamotrigine and secondly, rufinamide, is recommended. If seizure control remains suboptimal, subsequent adjunctive ASM treatment options include (alphabetically) cannabidiol, clobazam, felbamate, fenfluramine, and topiramate, although evidence for these is more limited. Whenever possible, no more than two ASMs should be used together. Nonpharmacological treatment approaches should be used in conjunction with ASM therapy and include ketogenic diet therapies, vagus nerve stimulation, and corpus callosotomy. Patients with LGS that has evolved from another type of epilepsy who are not already being treated with valproate should be transitioned to valproate and then managed using the same algorithm as for newly diagnosed LGS. Older patients with established LGS should be reviewed at least annually by a suitably experienced neurologist. The revised guidance aims to improve seizure control and quality of life for patients with LGS through personalized, evidence-based treatment strategies while addressing the challenges of accurate diagnosis and management in a rapidly evolving therapeutic landscape.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>Lennox–Gastaut syndrome (LGS) is a severe type of epilepsy that usually starts in childhood but continues into adulthood. It is characterized by a variety of different types of seizures (abnormal electrical activity in the brain), which are difficult to treat and often cause people with the condition to fall and injure themselves. Most people with LGS have learning difficulties and need a lot of support, often in residential care. The authors are experts in treating people with LGS and thi","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"85-106"},"PeriodicalIF":2.8,"publicationDate":"2024-12-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13075","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863796","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Gut-microbiota-brain Axis and post-traumatic epilepsy. 肠道-微生物群-脑轴与创伤后癫痫。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-17 DOI: 10.1002/epi4.13113
Andrey Mazarati
{"title":"Gut-microbiota-brain Axis and post-traumatic epilepsy.","authors":"Andrey Mazarati","doi":"10.1002/epi4.13113","DOIUrl":"https://doi.org/10.1002/epi4.13113","url":null,"abstract":"<p><p>There has been growing evidence that perturbations in gut-microbiota-brain axis (GMBA) are involved in mechanisms of chronic sequelae of traumatic brain injury (TBI). This review discusses the connection between GMBA and post-traumatic epilepsy (PTE), the latter being a common outcome of TBI. The focus is on two aspects of post-TBI GMBA dysfunction that are relevant to epilepsy. First are impairments in intestinal permeability with subsequent translocation of gut bacteria into the bloodstream. Specifically, endotoxemia following TBI may have a serendipitous protective effect against PTE through lipopolysaccharide conditioning, which may be leveraged for the development of therapeutic interventions. Second are changes in microbial composition (i.e., dysbiosis). Here, the GMBA-PTE connection is explored from predictive biomarker perspective, whereby the risk of PTE can be stratified based on specific microbial profiles. Finally, microbiota transplantation is discussed both as a tool to examine the role of gut microbiota in PTE and as a prelude to novel approaches for PTE therapy and prevention.</p>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":" ","pages":""},"PeriodicalIF":2.8,"publicationDate":"2024-12-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142834726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Incidence and Predictors of Later Epilepsy in Neonates with Encephalopathy: The Impact of Electrographic Seizures 脑病新生儿晚期癫痫的发病率和预测因素:电图癫痫发作的影响。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-16 DOI: 10.1002/epi4.13089
Carol M. Stephens, Jacopo Proietti, Sean R. Mathieson, Vicki Livingstone, Brian McNamara, Niamh McSweeney, Olivia O'Mahony, Brian H. Walsh, Deirdre M. Murray, Geraldine B. Boylan
{"title":"Incidence and Predictors of Later Epilepsy in Neonates with Encephalopathy: The Impact of Electrographic Seizures","authors":"Carol M. Stephens,&nbsp;Jacopo Proietti,&nbsp;Sean R. Mathieson,&nbsp;Vicki Livingstone,&nbsp;Brian McNamara,&nbsp;Niamh McSweeney,&nbsp;Olivia O'Mahony,&nbsp;Brian H. Walsh,&nbsp;Deirdre M. Murray,&nbsp;Geraldine B. Boylan","doi":"10.1002/epi4.13089","DOIUrl":"10.1002/epi4.13089","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To determine the incidence of later epilepsy in full-term infants with neonatal encephalopathy (NE) who undergo continuous electroencephalography (cEEG) monitoring in the neonatal period and to identify potential predictors of later epilepsy both in infants with and without electrographic neonatal seizures (ENS).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>This was a retrospective observational study performed at Cork University Maternity Hospital, Cork, Ireland, between 2003 and 2019. All term infants with NE had a minimum of 2 h of cEEG monitoring in the neonatal period. ENS were identified via cEEG monitoring. Pediatric medical charts were reviewed to determine if epilepsy developed after the neonatal period and to determine potential predictors of epilepsy in infants both with and without ENS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Two hundred and eighty infants were included. The overall incidence rate of epilepsy was 17.55 per 1000 person-years (95% CI: 10.91 to 28.23). In infants with ENS (<i>n</i> = 82), the incidence rate was 39.27 per 1000 person-years (95% CI: 22.30 to 69.16). In infants without ENS (<i>n</i> = 198), the incidence rate was 7.54 per 1000 person-years (95% CI: 3.14 to 18.12). The incidence rate was significantly higher in the ENS group compared to the non-ENS group (<i>p</i>-value = 0.002). Several potential predictors for the development of later epilepsy were identified including infants delivered vaginally, low Apgar scores at 1 and 5 min, severe HIE diagnosis, presence of ENS, a severely abnormal EEG background and an abnormal brain MRI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>Following NE, term infants are at risk of epilepsy with a significantly higher incidence rate in infants who experience ENS compared to those who did not. Close follow-up is required in both groups well into the childhood period.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>This study aimed to determine the occurrence of epilepsy in children who were monitored for seizures in the newborn period. The occurrence of epilepsy was higher in infants who experienced seizures in the newborn period compared to those who did not. Several potential predictors of later epilepsy were identified in both groups of infants (those with and without seizures in the newborn period). Both groups of infants require close follow-up in childhood.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"155-167"},"PeriodicalIF":2.8,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13089","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142827950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful management of refractory epilepsy in creatine transporter deficiency with cannabidiol and clobazam: A case report 用大麻二酚和氯巴赞成功治疗肌酸转运体缺乏症难治性癫痫:病例报告。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-16 DOI: 10.1002/epi4.13116
Maria Borrell-Pichot, Carmen Fons, Susana Boronat, Alba Sierra-Marcos
{"title":"Successful management of refractory epilepsy in creatine transporter deficiency with cannabidiol and clobazam: A case report","authors":"Maria Borrell-Pichot,&nbsp;Carmen Fons,&nbsp;Susana Boronat,&nbsp;Alba Sierra-Marcos","doi":"10.1002/epi4.13116","DOIUrl":"10.1002/epi4.13116","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>Creatine transporter deficiency (CRTR-D) is a rare X-linked inherited disease belonging to the group of cerebral creatine deficiency disorders. Major clinical features include developmental delay and epilepsy. To date, fewer than 200 individuals with CRTR-D have been reported. As a result, there is little evidence for effective treatment. Available therapies are creatine precursors, with a mild effect on disease progression. Concerning epilepsy, standard management is recommended and no specific anti-seizure medication (ASM) has been shown to be effective in refractory cases. We report the case of a 28-year-old male patient with CRTR-D and childhood-onset refractory epilepsy. He had an average of 10–20 focal motor seizures with impaired consciousness per month. He had tried several ASMs without significant improvement. Treatment with cannabidiol (CBD) and clobazam (CLB) in combination was added. The patient became seizure-free from the first week, and up to 1 year of follow-up. Behavioral improvement was also noted by his caregivers. No adverse effects were reported. Very few cases of CRTR-D with refractory epilepsy have been reported. This calls for more extensive research and suggests a possible role for CBD in cerebral creatine metabolism and transport and valuable option for future studies.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>Creatine transporter deficiency (CRTR-D) is a rare genetic disorder causing mental, behavioral, and movement problems. More than half of patients also have seizures, but because there are fewer than 200 known cases, it is difficult to know the best treatment options. We present a 28-year-old man with CRTR-D who had severe developmental delays and frequent seizures since childhood, despite trying many medications. After starting cannabidiol and clobazam, he has been seizure-free for a year. Sharing this success might help other people with CRTR-D benefit from similar treatments.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"342-347"},"PeriodicalIF":2.8,"publicationDate":"2024-12-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13116","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142827963","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
MRI findings in patients with psychogenic non-epileptic seizures: Prevalence, distribution, and classification of the findings. A single tertiary epilepsy center experience 心因性非癫痫性发作患者的MRI表现:患病率、分布和分类。单一三级癫痫中心经验。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-12 DOI: 10.1002/epi4.13114
Aleix Jareno-Badenas, Mario Matute-González, Luis Pintor, Estefanía Conde-Blanco, Mar Carreño, Xavier Setoain, Camilo Pineda, Fernando Diego Choque-Chávez, Tomás Fernández, Núria Bargalló, Sofía González-Ortiz
{"title":"MRI findings in patients with psychogenic non-epileptic seizures: Prevalence, distribution, and classification of the findings. A single tertiary epilepsy center experience","authors":"Aleix Jareno-Badenas,&nbsp;Mario Matute-González,&nbsp;Luis Pintor,&nbsp;Estefanía Conde-Blanco,&nbsp;Mar Carreño,&nbsp;Xavier Setoain,&nbsp;Camilo Pineda,&nbsp;Fernando Diego Choque-Chávez,&nbsp;Tomás Fernández,&nbsp;Núria Bargalló,&nbsp;Sofía González-Ortiz","doi":"10.1002/epi4.13114","DOIUrl":"10.1002/epi4.13114","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Psychogenic non-epileptic seizures (PNES) mimic epileptic seizures without electroencephalographic correlation. Although classified as psychiatric disorders, their neurobiological or structural basis remains unclear. This study aimed to assess the prevalence and characteristics of MRI abnormalities in patients with PNES and those with comorbid epilepsy, compared to the general population, to enhance radiological evaluation and management.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Method</h3>\u0000 \u0000 <p>We retrospectively identified patients with a definitive diagnosis of PNES, evaluated in the refractory epilepsy unit of our tertiary epilepsy center. Patients were classified into two groups according to their comorbidity with epilepsy (PNES and PNES+). The MRI findings were evaluated and classified by two radiologists, who reported the category of the findings, laterality, and location. The two groups were compared using the chi-square test, as well as the frequencies of findings in the general population extracted from the literature.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Forty-six patients fulfilled the inclusion criteria. Thirty females and 16 males. MRI findings were present in 25/35 (71.4%) patients in the PNES group and 9/11 (81.8%) In the PNES + group, showing statistically significant differences in the frequency of findings with the general population (8.4–28.1%).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Significance</h3>\u0000 \u0000 <p>MRI anomalies are common in PNES patients and even more prevalent in complex cases referred to epilepsy units, underscoring the necessity of correlating MRI findings with clinical-electrical patterns.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>In this article, we observed a higher frequency of cerebral magnetic resonance findings in patients with psychogenic non-epileptic seizures than in the general population. We also observed a higher frequency of this pathology among women, as well as right cerebral hemisphere affections. The exposed findings suggest a potential structural basis of this pathology. This hypothesis requires confirmation with larger studies.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"269-276"},"PeriodicalIF":2.8,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13114","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142812600","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Epilepsia Open—December 2024 announcements 癫痫病开放- 2024年12月公告
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-11 DOI: 10.1002/epi4.13109
{"title":"Epilepsia Open—December 2024 announcements","authors":"","doi":"10.1002/epi4.13109","DOIUrl":"https://doi.org/10.1002/epi4.13109","url":null,"abstract":"","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"9 6","pages":"2553-2555"},"PeriodicalIF":2.8,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13109","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142860907","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Exploring ketogenic diet resistance in glucose transporter type 1 deficiency syndrome: A comprehensive review and critical appraisal 探索1型葡萄糖转运蛋白缺乏综合征的生酮饮食抵抗:全面回顾和批判性评价。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-06 DOI: 10.1002/epi4.13110
Raffaele Falsaperla, Vincenzo Sortino, Gerhard Josef Kluger, Thomas Herberhold, Andrea Rüegger, Pasquale Striano, Martino Ruggieri, Joerg Klepper, Georgia Ramantani
{"title":"Exploring ketogenic diet resistance in glucose transporter type 1 deficiency syndrome: A comprehensive review and critical appraisal","authors":"Raffaele Falsaperla,&nbsp;Vincenzo Sortino,&nbsp;Gerhard Josef Kluger,&nbsp;Thomas Herberhold,&nbsp;Andrea Rüegger,&nbsp;Pasquale Striano,&nbsp;Martino Ruggieri,&nbsp;Joerg Klepper,&nbsp;Georgia Ramantani","doi":"10.1002/epi4.13110","DOIUrl":"10.1002/epi4.13110","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>Glucose transporter type 1 deficiency syndrome (GLUT1DS) commonly presents with early-onset epilepsy that often resists conventional pharmacological treatment. Ketogenic diet therapy (KDT) is the preferred approach to address the underlying metabolic anomaly. However, a subset of GLUT1DS patients presents resistance to KDT, with the causes remaining elusive. This comprehensive literature review aims to explore the characteristics of KDT failure in GLUT1DS and identify risk factors within this population. Our goal is to improve counseling and prognostication for these patients. So, we conducted a comprehensive literature review on PubMed, focusing on studies documenting pediatric GLUT1DS patients with drug-resistant epilepsy unresponsive to KDT. We identified five cases of KDT failure in female GLUT1DS patients, aged 10 days to 13 years at diagnosis. Predominant seizure types were absence seizures, with a few cases of clonic, tonic, or myoclonic seizures. EEG consistently revealed 2–3.5 Hz generalized spike-and-wave discharges. Genetic investigations revealed point mutations and deletions in two cases each. Despite an in-depth search, no specific features were found to reliably distinguish KDT non-responders from responders, underscoring the need for further research. In cases of KDT ineffectiveness for seizure control in GLUT1DS patients, exploring alternative therapeutic strategies becomes imperative to managing symptoms while maintaining quality of life. Large-scale multicenter studies, facilitated through international collaborations like the European Network for Therapy in Rare Epilepsies (NETRE), hold promise in elucidating the complexities of this patient population and developing personalized therapeutic approaches.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>Glucose transporter type 1 deficiency syndrome often causes difficult-to-treat epilepsy. The ketogenic diet works for many patients, but some do not respond. This review investigated cases of diet failure but could not identify common features among poor responders. Further research is needed to understand these cases and explore alternative treatments.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"31-39"},"PeriodicalIF":2.8,"publicationDate":"2024-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13110","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142784771","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Antiseizure medication prescribing in people with Dravet syndrome: An analysis of real-time administrative data Dravet综合征患者抗癫痫药物处方:实时管理数据分析。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-05 DOI: 10.1002/epi4.13105
Kevin Y. Xu, Binx Yezhe Lin, M. Scott Perry, Fábio A. Nascimento
{"title":"Antiseizure medication prescribing in people with Dravet syndrome: An analysis of real-time administrative data","authors":"Kevin Y. Xu,&nbsp;Binx Yezhe Lin,&nbsp;M. Scott Perry,&nbsp;Fábio A. Nascimento","doi":"10.1002/epi4.13105","DOIUrl":"10.1002/epi4.13105","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <p>Since 2018, three new antiseizure medications (ASMs) received FDA approval for Dravet syndrome (DS) in the U.S: cannabidiol, stiripentol, and fenfluramine. Yet, the uptake of these ASMs in routine clinical practice is unknown. We use new ICD-10 codes for DS (implemented in 2020) to estimate ASM receipt in patients with DS. We analyzed the TriNetX Network, a real-time electronic health record-based dataset linked to prescription data encompassing all 50 states of the U.S. After identifying patients with health care encounters for DS in 2021 and 2022 (via ICD-10 codes), we examined ASM prescribing in the year following a DS claim: 2022 and 2023, respectively. We retrieved 387 and 451 patients receiving claims for DS in 2021 and 2022, respectively. Clobazam, diazepam, valproate, midazolam, clonazepam, levetiracetam, and cannabidiol were the most common ASMs used (29%–44%). Stiripentol and fenfluramine prescribing was limited (7%–16%); these two ASMs, considered second-line therapies in DS, were prescribed less often than ASMs considered third-line or beyond. Cannabidiol, stiripentol, and fenfluramine prescribing rates remained nearly identical in the 2021 and 2022 cohorts. Our data suggests that stiripentol, fenfluramine, and, to an extent, cannabidiol may be underused in a large, diverse, primarily U.S.-based population of patients with DS.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Plain Language Summary</h3>\u0000 \u0000 <p>In an analysis of routinely-collected health care claims in the U.S., we found that the uptake of new antiseizure medications for Dravet Syndrome (i.e., stiripentol, fenfluramine, and cannabidiol) has been limited since 2022. Even though stiripentol and fenfluramine are considered second-line treatments for Dravet syndrome, we found they were prescribed less frequently than medicines considered third-line or beyond. These findings raise concern for underutilization of new antiseizure medications for Dravet syndrome in the United States.</p>\u0000 </section>\u0000 </div>","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"336-341"},"PeriodicalIF":2.8,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13105","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142784768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Preventing epileptogenesis by interaction between inositol isomers and proteins 肌醇异构体与蛋白质相互作用预防癫痫发生。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-04 DOI: 10.1002/epi4.13061
Lia Tsverava, Luka Kharkhelauri, Vincenzo Lagani, Giorgi Gamkrelidze, Veriko Bokuchava, Tamar Kiguradze, Merab Kokaia, Revaz Solomonia
{"title":"Preventing epileptogenesis by interaction between inositol isomers and proteins","authors":"Lia Tsverava,&nbsp;Luka Kharkhelauri,&nbsp;Vincenzo Lagani,&nbsp;Giorgi Gamkrelidze,&nbsp;Veriko Bokuchava,&nbsp;Tamar Kiguradze,&nbsp;Merab Kokaia,&nbsp;Revaz Solomonia","doi":"10.1002/epi4.13061","DOIUrl":"10.1002/epi4.13061","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Objective&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Inositols play significant roles in biological systems. Myo-inositol (MI), the most prevalent isomer, functions as an osmolyte and mediates cell signal transduction. Other notable isomers include Scyllo-inositol (SCI) and D-Chiro-inositol (DCHI). Our previous investigations have highlighted MI's potential antiepileptogenic effects, although its exact mechanisms of action during epileptogenesis remain unclear. A critical, unexplored area is how inositols interact with proteins. Additionally, the antiepileptogenic capabilities of SCI and DCHI have yet to be determined. This study seeks to address these gaps.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Inositol interacting proteins were identified by cellular thermal shift assay. Status epilepticus (SE) in rats was induced using kainic acid (KA), followed by a 28-day treatment with either MI, SCI, DCHI, or saline. The duration and frequencies of behavioral spontaneous recurrent seizures (SRS) were scored for 8 weeks by 24 h video monitoring system. The effects of inositol treatment on spatial learning and memory deficits associated with epileptogenesis were evaluated by Morris water maze test. The changes in protein amounts were studied by Western immunoblotting.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;We identified several proteins that interact with inositols, noting both commonalities and isomer-specific associations. For the first time, we demonstrated that the treatment with SCI and DCHI, alongside MI, significantly reduces the frequency and duration of behavioral SRS in a KA-induced post-status epilepsy model in rats. This reduction persisted for 4 weeks post-treatment. Moreover, all three inositol isomers mitigated spatial learning and memory deficits associated with epileptogenesis. Alterations in the inositol interacting proteins: alpha synuclein and 14-3-3 theta were further examined 8 weeks post-SE in the hippocampus and neocortex of rats.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Significance&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Myo-inositol, SCI and DCHI interact with a number of proteins involved in different biological pathways. All studied inositol isomers express long-term beneficial effects on KA-induced SRS and the associated comorbidities. Inositols can be successfully used in the future for translational research.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Plain Language Summary&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Epilepsy is a common neurological disorder characterized by spontaneous recurrent seizures and a range","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"120-133"},"PeriodicalIF":2.8,"publicationDate":"2024-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13061","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142779266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The appearance of scalp high-frequency oscillations is associated with poor seizure control in pediatric epilepsy patients 小儿癫痫患者出现头皮高频振荡与癫痫发作控制不良有关。
IF 2.8 3区 医学
Epilepsia Open Pub Date : 2024-12-03 DOI: 10.1002/epi4.13032
Keisuke Maeda, Nami Hosoda, Himari Tsuboi, Honoka Naito, Chiaki Kudo, Junichi Fukumoto, Shiho Fujita, Naohiro Ichino, Keisuke Osakabe, Keiko Sugimoto, Shunta Yamaguchi, Naoko Ishihara
{"title":"The appearance of scalp high-frequency oscillations is associated with poor seizure control in pediatric epilepsy patients","authors":"Keisuke Maeda,&nbsp;Nami Hosoda,&nbsp;Himari Tsuboi,&nbsp;Honoka Naito,&nbsp;Chiaki Kudo,&nbsp;Junichi Fukumoto,&nbsp;Shiho Fujita,&nbsp;Naohiro Ichino,&nbsp;Keisuke Osakabe,&nbsp;Keiko Sugimoto,&nbsp;Shunta Yamaguchi,&nbsp;Naoko Ishihara","doi":"10.1002/epi4.13032","DOIUrl":"10.1002/epi4.13032","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Objective&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Epilepsy treatment with anti-seizure medications (ASMs) is based on careful assessment of the balance between the likelihood of further seizures and the risk of side effects of treatment. However, there is currently no established biomarker to ascertain seizure control status with ASMs. High-frequency oscillations (HFOs), transient bursts of EEG activity with frequencies beyond 80 Hz, are a new and promising noninvasive epilepsy biomarker. We compared the risk of scalp HFO appearance between pediatric patients with good and poor seizure control by treatment with ASMs.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;A total of 72 epilepsy patients (aged 0–18 years, 39 males) with good and poor seizure control with ASMs participated in this study. We applied a validated automated detector to determine HFO and spike. We calculated the odds ratios (ORs) for scalp HFO and spike appearance according to seizure control status by multiple logistic regression analysis.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;Scalp HFO was seen more commonly and with a significantly higher detection rate in patients with poor seizure control as compared with patients with good seizure control for both ripple and fast ripple. These significant associations were found for both focal and generalized epilepsy. The ORs for scalp HFO appearance adjusted for confounding factors were significantly higher in patients with poor seizure control compared to those with good seizure control (ripple: OR [95% CI] = 11.91 [2.21–64.30], &lt;i&gt;p&lt;/i&gt; = 0.004; fast ripple: 4.98 [1.03–24.09], &lt;i&gt;p&lt;/i&gt; = 0.046). There were no significant associations between spike appearance and seizure control status.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Significance&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;We found an increased risk of scalp HFO appearance in patients with poor seizure control. The results of this study support that scalp HFO is associated with patients having frequent seizures after treatment in both ripple and fast ripple.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Plain Language Summary&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;This study analyzed scalp high-frequency oscillations and spikes in pediatric patients with various types of epilepsy who were being treated using ASMs. The results showed that an increased risk of scalp HFO appearance was observed in patients with poor seizure control compared to those with good seizure control. These findings were observed in both the ripple (80–250 Hz) and fast ripple (250–500 Hz) bands. The sca","PeriodicalId":12038,"journal":{"name":"Epilepsia Open","volume":"10 1","pages":"107-119"},"PeriodicalIF":2.8,"publicationDate":"2024-12-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/epi4.13032","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142767493","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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