Pub Date : 2025-01-29DOI: 10.1016/j.ebr.2025.100745
Gashirai K. Mbizvo , Viraj Bharambe , Brython Hywel , Shubhabrata Biswas , Andrew J. Larner
The Alice in Wonderland syndrome comprises visual and/or somaesthetic distortions, most often migrainous in origin. We present a case of Alice in Wonderland syndrome in association with a right posterior/visual cortex infarction with subsequent seizures including focal motor status (epilepsia partialis continua), the latter a hitherto unreported association. These pathophysiological changes were of right hemisphere origin, suggesting Alice in Wonderland syndrome, if present, might have localising value for seizure focus in the right hemisphere.
{"title":"Alice in Wonderland Syndrome: Localising insights from right visual cortex stroke complicated by epilepsia partialis continua","authors":"Gashirai K. Mbizvo , Viraj Bharambe , Brython Hywel , Shubhabrata Biswas , Andrew J. Larner","doi":"10.1016/j.ebr.2025.100745","DOIUrl":"10.1016/j.ebr.2025.100745","url":null,"abstract":"<div><div>The Alice in Wonderland syndrome comprises visual and/or somaesthetic distortions, most often migrainous in origin. We present a case of Alice in Wonderland syndrome in association with a right posterior/visual cortex infarction with subsequent seizures including focal motor status (epilepsia partialis continua), the latter a hitherto unreported association. These pathophysiological changes were of right hemisphere origin, suggesting Alice in Wonderland syndrome, if present, might have localising value for seizure focus in the right hemisphere.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100745"},"PeriodicalIF":1.8,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129968","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-19DOI: 10.1016/j.ebr.2025.100742
Amir Reza Bahadori , Parisa Javadnia , Afshan Davari , Sajad Shafiee , Sara Ranji , Mehrdad Sheikhvatan , Abbas Tafakhori
This study investigates the impact of the anterior nucleus of the thalamus deep brain stimulation (ANT-DBS) on patients with drug-resistant epilepsy (DRE) in Iran, specifically focusing on its effects on seizure metrics, severity and its influence on quality of life over time. A cohort of eight patients with DRE in Iran who underwent ANT-DBS was evaluated. Pre-operative assessments included comprehensive documentation of seizure frequency, duration, severity scores, and the Quality of Life in Epilepsy Inventory (QOLIE-13). Each patient also underwent high-resolution imaging using a 1.5 Tesla MRI, with targeted electrode placement in the anterior thalamic area. Post-operative evaluations measured changes in seizure frequency, severity scores, duration, and quality of life indicators. All subjects presented with DRE, and the mean age of participants was 24.62 years. Post-operative data revealed significantly reduced seizure frequency, duration, and severity scores. Notably, this reduction was more pronounced at the 6-month follow-up than the 3-month assessment, indicating a progressive therapeutic effect. All patients demonstrated a response to ANT-DBS, with two individuals achieving seizure freedom. Additionally, there was a marked improvement in quality of life, particularly in the domains of energy/fatigue and social functioning. ANT-DBS has been established as a promising and safe therapeutic intervention for patients with DRE. In a cohort of DRE patients in Iran, the treatment demonstrated comparable efficacy in decreasing seizure frequency and severity and enhancing self-reported quality of life, consistent with findings reported in the existing literature. The therapeutic benefits of ANT-DBS appear to augment over time.
{"title":"Effect of deep brain stimulation on the severity of seizures and the quality of life in patients with multifocal drug-resistant epilepsy in Iran: A pilot review of local experience","authors":"Amir Reza Bahadori , Parisa Javadnia , Afshan Davari , Sajad Shafiee , Sara Ranji , Mehrdad Sheikhvatan , Abbas Tafakhori","doi":"10.1016/j.ebr.2025.100742","DOIUrl":"10.1016/j.ebr.2025.100742","url":null,"abstract":"<div><div>This study investigates the impact of the anterior nucleus of the thalamus deep brain stimulation (ANT-DBS) on patients with drug-resistant epilepsy (DRE) in Iran, specifically focusing on its effects on seizure metrics, severity and its influence on quality of life over time. A cohort of eight patients with DRE in Iran who underwent ANT-DBS was evaluated. Pre-operative assessments included comprehensive documentation of seizure frequency, duration, severity scores, and the Quality of Life in Epilepsy Inventory (QOLIE-13). Each patient also underwent high-resolution imaging using a 1.5 Tesla MRI, with targeted electrode placement in the anterior thalamic area. Post-operative evaluations measured changes in seizure frequency, severity scores, duration, and quality of life indicators. All subjects presented with DRE, and the mean age of participants was 24.62 years. Post-operative data revealed significantly reduced seizure frequency, duration, and severity scores. Notably, this reduction was more pronounced at the 6-month follow-up than the 3-month assessment, indicating a progressive therapeutic effect. All patients demonstrated a response to ANT-DBS, with two individuals achieving seizure freedom. Additionally, there was a marked improvement in quality of life, particularly in the domains of energy/fatigue and social functioning. ANT-DBS has been established as a promising and safe therapeutic intervention for patients with DRE. In a cohort of DRE patients in Iran, the treatment demonstrated comparable efficacy in decreasing seizure frequency and severity and enhancing self-reported quality of life, consistent with findings reported in the existing literature. The therapeutic benefits of ANT-DBS appear to augment over time.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100742"},"PeriodicalIF":1.8,"publicationDate":"2025-01-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129967","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-01-15DOI: 10.1016/j.ebr.2025.100741
Joan E. Devin , Fergal O’Shaughnessy , Muskan Sardana , Brian J. Cleary , Jennifer C. Donnelly , Nicola Maher
Epilepsy is a common serious neurological disorder, affecting approximately 28 per 10,000 pregnancies internationally each year. There are limited data on the use of newer anti-seizure medicines (ASMs) in pregnancy, despite increasing use. We aimed to describe the use of newer ASMs in women with epilepsy (WWE) attending the Rotunda Hospital, Dublin, in pregnancy, between 2018 and 2023. We conducted a retrospective case series using electronic health record data. All WWE with a medication order for a newer ASM and a completed pregnancy were included. We identified 34 pregnancies exposed to newer ASMs, namely zonisamide (35.2 %), brivaracetam (23.5 %), eslicarbazepine (23.5 %), lacosamide (17.6 %), and perampanel (2.9 %). Newer ASMs were used as monotherapy in 58.8 % cases. Levetiracetam was the most commonly prescribed concomitant ASM in polytherapy regimens (32.4 %). Seizures occurred during pregnancy or the postpartum period in 50.0 % and 14.7 % of pregnancies, respectively. Twenty-eight pregnancies (80 %) resulted in a livebirth, with median gestation and birth weight of 39 weeks’ [IQR 2] and 3100 g [IQR 790]. One neonate exposed to polytherapy including eslicarbazepine was observed to have a minor anomaly at birth, not requiring follow-up. Findings show that in WWE, most pregnancies exposed to newer ASMs resulted in healthy livebirths at term without negative outcomes. A high proportion of polytherapy exposures and high rate of seizures during pregnancy suggests that this may be a cohort at greater risk for caesarean section or other complications. Findings should be interpreted with caution, with additional data needed to examine the impact of individual ASMs on outcomes.
{"title":"The use of newer anti-seizure medicines in women with epilepsy in pregnancy: A case series","authors":"Joan E. Devin , Fergal O’Shaughnessy , Muskan Sardana , Brian J. Cleary , Jennifer C. Donnelly , Nicola Maher","doi":"10.1016/j.ebr.2025.100741","DOIUrl":"10.1016/j.ebr.2025.100741","url":null,"abstract":"<div><div>Epilepsy is a common serious neurological disorder, affecting approximately 28 per 10,000 pregnancies internationally each year. There are limited data on the use of newer anti-seizure medicines (ASMs) in pregnancy, despite increasing use. We aimed to describe the use of newer ASMs in women with epilepsy (WWE) attending the Rotunda Hospital, Dublin, in pregnancy, between 2018 and 2023. We conducted a retrospective case series using electronic health record data. All WWE with a medication order for a newer ASM and a completed pregnancy were included. We identified 34 pregnancies exposed to newer ASMs, namely zonisamide (35.2 %), brivaracetam (23.5 %), eslicarbazepine (23.5 %), lacosamide (17.6 %), and perampanel (2.9 %). Newer ASMs were used as monotherapy in 58.8 % cases. Levetiracetam was the most commonly prescribed concomitant ASM in polytherapy regimens (32.4 %). Seizures occurred during pregnancy or the postpartum period in 50.0 % and 14.7 % of pregnancies, respectively. Twenty-eight pregnancies (80 %) resulted in a livebirth, with median gestation and birth weight of 39 weeks’ [IQR 2] and 3100 g [IQR 790]. One neonate exposed to polytherapy including eslicarbazepine was observed to have a minor anomaly at birth, not requiring follow-up. Findings show that in WWE, most pregnancies exposed to newer ASMs resulted in healthy livebirths at term without negative outcomes. A high proportion of polytherapy exposures and high rate of seizures during pregnancy suggests that this may be a cohort at greater risk for caesarean section or other complications. Findings should be interpreted with caution, with additional data needed to examine the impact of individual ASMs on outcomes.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100741"},"PeriodicalIF":1.8,"publicationDate":"2025-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-28DOI: 10.1016/j.ebr.2024.100739
Luciana Giambarberi , Halley B. Alexander , Heidi Munger Clary
Attention-deficit/hyperactivity disorder (ADHD) and epilepsy have one of the lesser known and yet highly debated relationships in neuropsychiatry. Similar to anxiety and depression, ADHD has a bidirectional relationship with epilepsy, in which individuals with epilepsy are more likely than the general population to have ADHD and vice versa. Most importantly, an untreated psychiatric condition can affect quality of life. Although the management of ADHD in PWE has been debated due to perceived seizure risk related to ADHD medications, a consensus has developed based on early pediatric studies that support the treatment of ADHD. However, the management of adults with ADHD, particularly in PWE, remains relatively unexplored. This critical gap in knowledge will be addressed using an illustrative case study followed by practical tips on the identification and pharmacologic management of ADHD in adults with epilepsy. The management of ADHD in PWE should begin with a thorough history, medication assessment for cognitive risk, and the addition of a brief ADHD screening tool, such as the Adult ADHD Self-Report Scale (ASRS). Treatment with stimulants, such as methylphenidate, and non-stimulants, such as atomoxetine, are effective. Caution, however, should be taken for any patients with history of bipolar disorder, as some ADHD medications may exacerbate symptoms of other psychiatric conditions. Patients can also be referred to psychotherapy, such as cognitive behavior therapy (CBT) for ADHD, in addition to or in lieu of medications, thus further minimizing potential pharmacological risk. Patients who have tried and failed multiple ADHD medications and/or who carry a more complex psychiatric history should be referred to a psychiatrist.
{"title":"ADHD in Adults with Epilepsy: A Guide for Neurologists","authors":"Luciana Giambarberi , Halley B. Alexander , Heidi Munger Clary","doi":"10.1016/j.ebr.2024.100739","DOIUrl":"10.1016/j.ebr.2024.100739","url":null,"abstract":"<div><div>Attention-deficit/hyperactivity disorder (ADHD) and epilepsy have one of the lesser known and yet highly debated relationships in neuropsychiatry. Similar to anxiety and depression, ADHD has a bidirectional relationship with epilepsy, in which individuals with epilepsy are more likely than the general population to have ADHD and vice versa. Most importantly, an untreated psychiatric condition can affect quality of life. Although the management of ADHD in PWE has been debated due to perceived seizure risk related to ADHD medications, a consensus has developed based on early pediatric studies that support the treatment of ADHD. However, the management of adults with ADHD, particularly in PWE, remains relatively unexplored. This critical gap in knowledge will be addressed using an illustrative case study followed by practical tips on the identification and pharmacologic management of ADHD in adults with epilepsy. The management of ADHD in PWE should begin with a thorough history, medication assessment for cognitive risk, and the addition of a brief ADHD screening tool, such as the Adult ADHD Self-Report Scale (ASRS). Treatment with stimulants, such as methylphenidate, and non-stimulants, such as atomoxetine, are effective. Caution, however, should be taken for any patients with history of bipolar disorder, as some ADHD medications may exacerbate symptoms of other psychiatric conditions. Patients can also be referred to psychotherapy, such as cognitive behavior therapy (CBT) for ADHD, in addition to or in lieu of medications, thus further minimizing potential pharmacological risk. Patients who have tried and failed multiple ADHD medications and/or who carry a more complex psychiatric history should be referred to a psychiatrist.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100739"},"PeriodicalIF":1.8,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143129969","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-28DOI: 10.1016/j.ebr.2024.100735
Rudhab Bahabry, Silvienne Sint Jago, Rebecca M. Hauser, Jonathan Harmon, Leah Dinah Sheppard, Bellafaith Oyassan, Farah D. Lubin
The transcriptional mechanisms underlying impaired hippocampal-dependent memory seen in temporal lobe epilepsy (TLE) have been extensively studied in rodent models. While cognitive testing in these models often involves multiple behavioral tasks, the impact of sequential behavioral testing (SBT) on gene transcription changes in epilepsy remains poorly understood. This study utilized the Kainic Acid (KA) TLE rodent model to examine hippocampal gene expression changes influenced by SBT. Our findings indicate reduced anxiety-related behavior, along with impaired spatial and recognition memory and fear memory in epileptic animals. Quantitative PCR (qPCR) analysis revealed an increase in BDNF, dFosB, Tet2, and Tet3 expression in the epilepsy-SBT group compared to control-SBT, while there was a reduction in Npas4 and Egr4 expression. Immunohistochemistry (IHC) showed that in epileptic animals, performing SBT reversed the loss of 5-hydroxymethylcytosine (5-hmC) in the dorsal hippocampus compared to that seen in home-caged (HC) epileptic animals, and this reversal was neuron-driven. These findings highlight the complex interplay between gene transcription and epigenetic regulation during SBT enrichment in the context of epilepsy.
{"title":"Hippocampal gene expression changes associated with sequential behavioral training in a temporal lobe epilepsy rat model","authors":"Rudhab Bahabry, Silvienne Sint Jago, Rebecca M. Hauser, Jonathan Harmon, Leah Dinah Sheppard, Bellafaith Oyassan, Farah D. Lubin","doi":"10.1016/j.ebr.2024.100735","DOIUrl":"10.1016/j.ebr.2024.100735","url":null,"abstract":"<div><div>The transcriptional mechanisms underlying impaired hippocampal-dependent memory seen in temporal lobe epilepsy (TLE) have been extensively studied in rodent models. While cognitive testing in these models often involves multiple behavioral tasks, the impact of sequential behavioral testing (SBT) on gene transcription changes in epilepsy remains poorly understood. This study utilized the Kainic Acid (KA) TLE rodent model to examine hippocampal gene expression changes influenced by SBT. Our findings indicate reduced anxiety-related behavior, along with impaired spatial and recognition memory and fear memory in epileptic animals. Quantitative PCR (qPCR) analysis revealed an increase in <em>BDNF, dFosB, Tet2, and Tet3</em> expression in the epilepsy-SBT group compared to control-SBT, while there was a reduction in <em>Npas4</em> and <em>Egr4 expression</em>. Immunohistochemistry (IHC) showed that in epileptic animals, performing SBT reversed the loss of 5-hydroxymethylcytosine (5-hmC) in the dorsal hippocampus compared to that seen in home-caged (HC) epileptic animals, and this reversal was neuron-driven. These findings highlight the complex interplay between gene transcription and epigenetic regulation during SBT enrichment in the context of epilepsy.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100735"},"PeriodicalIF":1.8,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11786087/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143081545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-26DOI: 10.1016/j.ebr.2024.100740
Chalongchai Phitsanuwong , Stephanie Schimpf , Sho T. Yano
Dynein Cytoplasmic 1 Heavy chain 1 (DYNC1H1)-related disorders are a spectrum of conditions including neurodevelopmental disorders, congenital brain malformations, and neuromuscular diseases. These clinical features may co-occur, with four main disease entities including epilepsy with developmental epileptic encephalopathy such as infantile epileptic spasms syndrome (IESS) and Lennox-Gastaut syndrome (LGS), axonal Charcot-Marie-Tooth disease type 2O, spinal muscular atrophy with lower extremity-predominance (SMALED), and congenital cortical malformations. Epilepsy associated with this disorder often becomes drug-resistant and requires multiple medications and, in some cases, non-pharmacological treatments. To date, there is no specific epilepsy treatment that is particularly effective in this disorder. We report our experience in a case of a 3-year-old girl with a pathogenic variant in DYNC1H1 who presented with a developmental epileptic encephalopathy consistent with IESS and achieved seizure freedom on classic ketogenic diet (KD) after failing Adrenocorticotropic Hormone (ACTH), vigabatrin, and clobazam. The patient remained seizure free for more than 2 years on dietary monotherapy and had reported improvement in alertness, cognitive ability, muscle tone, and a normalized EEG. The ketogenic diet therapy, therefore, has shown to be highly effective in this case with DYNC1H1-related epilepsy.
动力蛋白-细胞质1重链1 （DYNC1H1）相关疾病是一系列疾病，包括神经发育障碍、先天性脑畸形和神经肌肉疾病。这些临床特征可能与四种主要疾病实体同时发生，包括癫痫伴发展性癫痫性脑病，如婴儿癫痫性痉挛综合征（IESS）和lenox - gastaut综合征（LGS），轴索性charco - marie - tooth病20型，脊髓性肌萎缩伴下肢优势（SMALED）和先天性皮质畸形。与这种疾病相关的癫痫往往具有耐药性，需要多种药物治疗，在某些情况下还需要非药物治疗。迄今为止，还没有针对这种疾病特别有效的特定癫痫治疗方法。我们报告了一例3岁女孩DYNC1H1致病变异，她表现为与IESS一致的发育性癫痫性脑病，在促肾上腺皮质激素（ACTH）、维加巴林和氯巴唑无效后，通过经典生酮饮食（KD）实现了癫痫发作自由。患者接受单一饮食治疗2年多无癫痫发作，并报告警觉性、认知能力、肌肉张力和正常脑电图均有改善。因此，生酮饮食疗法在本例dync1h1相关癫痫中显示出高度有效。
{"title":"Favorable response to ketogenic diet therapy in a patient with DYNC1H1-related epilepsy","authors":"Chalongchai Phitsanuwong , Stephanie Schimpf , Sho T. Yano","doi":"10.1016/j.ebr.2024.100740","DOIUrl":"10.1016/j.ebr.2024.100740","url":null,"abstract":"<div><div>Dynein Cytoplasmic 1 Heavy chain 1 (<em>DYNC1H1)</em>-related disorders are a spectrum of conditions including neurodevelopmental disorders, congenital brain malformations, and neuromuscular diseases. These clinical features may co-occur, with four main disease entities including epilepsy with developmental epileptic encephalopathy such as infantile epileptic spasms syndrome (IESS) and Lennox-Gastaut syndrome (LGS), axonal Charcot-Marie-Tooth disease type 2O, spinal muscular atrophy with lower extremity-predominance (SMALED), and congenital cortical malformations. Epilepsy associated with this disorder often becomes drug-resistant and requires multiple medications and, in some cases, non-pharmacological treatments. To date, there is no specific epilepsy treatment that is particularly effective in this disorder. We report our experience in a case of a 3-year-old girl with a pathogenic variant in <em>DYNC1H1</em> who presented with a developmental epileptic encephalopathy consistent with IESS and achieved seizure freedom on classic ketogenic diet (KD) after failing Adrenocorticotropic Hormone (ACTH), vigabatrin, and clobazam. The patient remained seizure free for more than 2 years on dietary monotherapy and had reported improvement in alertness, cognitive ability, muscle tone, and a normalized EEG. The ketogenic diet therapy, therefore, has shown to be highly effective in this case <em>with DYNC1H1-</em>related epilepsy<em>.</em></div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100740"},"PeriodicalIF":1.8,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11743897/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143013429","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-25DOI: 10.1016/j.ebr.2024.100738
Trudy D. Pang , Richard L. Verrier , Steven C. Schachter
Multifactorial lines of evidence in adults point to a critical linkage between chronic epilepsy and elevated risk for cardiovascular disease and premature cardiac death. Diverse pathophysiological processes appear to be involved that include accelerated atherosclerosis, myocardial infarction, abnormal autonomic tone, heart failure, atrial and ventricular arrhythmias, and hyperlipidemia. Seizure-induced surges in catecholamines and hypoxia may be conducive to cardiovascular damage and the Epileptic Heart condition. The current review provides a systematic strategy for clinical management to reduce risk for cardiovascular disease in adult patients with epilepsy. The proposed approach includes adherence to cardiovascular risk guidelines, incorporation of standard monitoring using electrocardiographic and echocardiographic markers, and regular assessment of plasma lipid profiles. Attention is drawn to the arrhythmogenic risks associated with antiseizure medications (ASMs) with sodium channel blocking properties that can disrupt cardiac conduction and repolarization and predispose to ventricular and atrial arrhythmias. Caution is warranted regarding the use of enzyme-inducing ASMs that can increase plasma lipid levels. The ultimate goals of the proposed management recommendations are to mitigate cardiac risk and reduce premature cardiac death in individuals with chronic epilepsy.
{"title":"Management recommendations to reduce cardiac risk in chronic epilepsy","authors":"Trudy D. Pang , Richard L. Verrier , Steven C. Schachter","doi":"10.1016/j.ebr.2024.100738","DOIUrl":"10.1016/j.ebr.2024.100738","url":null,"abstract":"<div><div>Multifactorial lines of evidence in adults point to a critical linkage between chronic epilepsy and elevated risk for cardiovascular disease and premature cardiac death. Diverse pathophysiological processes appear to be involved that include accelerated atherosclerosis, myocardial infarction, abnormal autonomic tone, heart failure, atrial and ventricular arrhythmias, and hyperlipidemia. Seizure-induced surges in catecholamines and hypoxia may be conducive to cardiovascular damage and the Epileptic Heart condition. The current review provides a systematic strategy for clinical management to reduce risk for cardiovascular disease in adult patients with epilepsy. The proposed approach includes adherence to cardiovascular risk guidelines, incorporation of standard monitoring using electrocardiographic and echocardiographic markers, and regular assessment of plasma lipid profiles. Attention is drawn to the arrhythmogenic risks associated with antiseizure medications (ASMs) with sodium channel blocking properties that can disrupt cardiac conduction and repolarization and predispose to ventricular and atrial arrhythmias. Caution is warranted regarding the use of enzyme-inducing ASMs that can increase plasma lipid levels. The ultimate goals of the proposed management recommendations are to mitigate cardiac risk and reduce premature cardiac death in individuals with chronic epilepsy.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100738"},"PeriodicalIF":1.8,"publicationDate":"2024-12-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143130021","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-22DOI: 10.1016/j.ebr.2024.100737
Siu Hing Lo , Hanna Skrobanski , Miranda Harrison , Jamshaed Siddiqui , Sally Bowditch
Tuberous sclerosis complex (TSC)-associated seizures result in a significant burden for caregivers. To quantify time spent and describe activities undertaken by caregivers of individuals with TSC-associated seizures in Sweden, primary caregivers participated in a cross-sectional, non-interventional online survey. Questions comprised patient/caregiver characteristics, care provision, time, and activities associated with generalized seizures or non-seizure-related care. Twenty-three primary caregivers participated; 96 % parents, 100 % female. Median number of caregivers per individual was three. In the last month, median (interquartile range [IQR]) hours for caregiving per week was 52.0 (25.7–100.0; n = 21); median (IQR) hours for non-seizure-related care was 46.7 (20.0–93.3; n = 21) and for generalized seizure-related care was 4.7 (1.7–15.8; n = 12). Beyond the last month, hours/week of generalized seizure-related care varied from 1.9 (0–8.8) to 14.0 (0.5–77.0). Professional/paid carers contributed 99.2 (73.5–127.5) hours/week of care. Non-seizure-related care activities included assisting with routine medical care (n = 22, 96 %) and daily activities (n = 22, 96 %). Activities relating to generalized seizures included assessing the need for (n = 16, 84 %) and giving (n = 17, 89 %) rescue medication, providing physical support (n = 16, 84 %), and clearing the individual’s environment during generalized seizures (n = 13, 68 %). During generalized seizure recovery, activities included taking the individual to bed (n = 18, 95 %), emotional support (n = 14, 74 %), and managing behavioral issues (n = 14, 74 %). In Sweden, despite contributions of paid caregivers, individuals with TSC-associated seizures require substantial time from unpaid primary caregivers, including seizure- and non-seizure-related care. Generalized seizures have a considerable impact on time spent caregiving and the care activities undertaken.
{"title":"Caregiver burden associated with caring for individuals with tuberous sclerosis complex-associated seizures: A descriptive, non-interventional survey in Sweden","authors":"Siu Hing Lo , Hanna Skrobanski , Miranda Harrison , Jamshaed Siddiqui , Sally Bowditch","doi":"10.1016/j.ebr.2024.100737","DOIUrl":"10.1016/j.ebr.2024.100737","url":null,"abstract":"<div><div>Tuberous sclerosis complex (TSC)-associated seizures result in a significant burden for caregivers. To quantify time spent and describe activities undertaken by caregivers of individuals with TSC-associated seizures in Sweden, primary caregivers participated in a cross-sectional, non-interventional online survey. Questions comprised patient/caregiver characteristics, care provision, time, and activities associated with generalized seizures or non-seizure-related care. Twenty-three primary caregivers participated; 96 % parents, 100 % female. Median number of caregivers per individual was three. In the last month, median (interquartile range [IQR]) hours for caregiving per week was 52.0 (25.7–100.0; n = 21); median (IQR) hours for non-seizure-related care was 46.7 (20.0–93.3; n = 21) and for generalized seizure-related care was 4.7 (1.7–15.8; n = 12). Beyond the last month, hours/week of generalized seizure-related care varied from 1.9 (0–8.8) to 14.0 (0.5–77.0). Professional/paid carers contributed 99.2 (73.5–127.5) hours/week of care. Non-seizure-related care activities included assisting with routine medical care (n = 22, 96 %) and daily activities (n = 22, 96 %). Activities relating to generalized seizures included assessing the need for (n = 16, 84 %) and giving (n = 17, 89 %) rescue medication, providing physical support (n = 16, 84 %), and clearing the individual’s environment during generalized seizures (n = 13, 68 %). During generalized seizure recovery, activities included taking the individual to bed (n = 18, 95 %), emotional support (n = 14, 74 %), and managing behavioral issues (n = 14, 74 %). In Sweden, despite contributions of paid caregivers, individuals with TSC-associated seizures require substantial time from unpaid primary caregivers, including seizure- and non-seizure-related care. Generalized seizures have a considerable impact on time spent caregiving and the care activities undertaken.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100737"},"PeriodicalIF":1.8,"publicationDate":"2024-12-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11787012/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143081539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-13DOI: 10.1016/j.ebr.2024.100734
Riëm El Tahry , Maxine Dibué , Arnaud Szmalec , Roshani Patel , Ryan Verner , Massimiliano Boffini , Firas Fahoum , Michal Tzadok
For patients with drug-resistant epilepsy who are not candidates for epilepsy surgery, Vagus nerve stimulation (VNS) is the most widely available neuromodulation option and has been available in several countries for 30 years. Given its broad availability and extended history on the market, many healthcare providers (HCPs) have developed individualized practice habits regarding the titration and dosing of VNS. This study provides novel evidence to describe the extent to which VNS management differs among providers and discusses recent literature that indicates how unique programming approaches may impact patient outcomes. In this work, practice habits regarding the titration and dosing of VNS were explored through a survey of HCPs and an examination of ongoing study data collected as part of the CORE-VNS Study. The global survey revealed significant variability in dosing and titration habits. Providers reported a wide range of initial/maximum target doses and time-to-dose, even if the population averages approximated guidance from professional societies and the manufacturer’s labeling. Variable dosing and titration were reflected in varied perception of how long it takes to realize the clinical benefits of VNS. In the CORE-VNS Study, this reported experience was represented in how different generator models were used, with users of SenTiva (and the Scheduled Programming feature) depicting faster time-to-dose than those using earlier models of VNS. Our results suggest VNS providers would benefit from continued training on the use of VNS and the use of the scheduled programming feature to enhance consistency of VNS management among providers.
{"title":"Practical Considerations for the rapid titration of VNS","authors":"Riëm El Tahry , Maxine Dibué , Arnaud Szmalec , Roshani Patel , Ryan Verner , Massimiliano Boffini , Firas Fahoum , Michal Tzadok","doi":"10.1016/j.ebr.2024.100734","DOIUrl":"10.1016/j.ebr.2024.100734","url":null,"abstract":"<div><div>For patients with drug-resistant epilepsy who are not candidates for epilepsy surgery, Vagus nerve stimulation (VNS) is the most widely available neuromodulation option and has been available in several countries for 30 years. Given its broad availability and extended history on the market, many healthcare providers (HCPs) have developed individualized practice habits regarding the titration and dosing of VNS. This study provides novel evidence to describe the extent to which VNS management differs among providers and discusses recent literature that indicates how unique programming approaches may impact patient outcomes. In this work, practice habits regarding the titration and dosing of VNS were explored through a survey of HCPs and an examination of ongoing study data collected as part of the CORE-VNS Study. The global survey revealed significant variability in dosing and titration habits. Providers reported a wide range of initial/maximum target doses and time-to-dose, even if the population averages approximated guidance from professional societies and the manufacturer’s labeling. Variable dosing and titration were reflected in varied perception of how long it takes to realize the clinical benefits of VNS. In the CORE-VNS Study, this reported experience was represented in how different generator models were used, with users of SenTiva (and the Scheduled Programming feature) depicting faster time-to-dose than those using earlier models of VNS. Our results suggest VNS providers would benefit from continued training on the use of VNS and the use of the scheduled programming feature to enhance consistency of VNS management among providers.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100734"},"PeriodicalIF":1.8,"publicationDate":"2024-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11721848/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142972451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Epilepsy is the most common chronic neurological condition in children. Many barriers exist in early recognition which cause delay in care and impact quality of life. Some of these children require advanced treatments which are underutilized due to lack of education, awareness and referrals. Overall, childhood epilepsy is underdiagnosed and poorly understood by non-expert providers. We investigated awareness and knowledge about epilepsy from primary care providers via the quality of their referrals. We prospectively collected and examined all epilepsy related referrals to the Paediatric Neurology Division at Children’s Hospital in London, Ontario, Canada during a six-month period. We developed a modified “epilepsy focused” scoring tool to evaluate the referrals and scored them as basic or advanced. During the study time frame 175 (82 %) referrals met the inclusion criteria. Out of these, 152 (87 %) were identified as basic and 23 (13 %) were advanced (p < 0.001). Amongst the referrals that scored basic vs advanced: Family Doctors n = 49 with 40 basic (81 %) vs 9 advanced (18.3 %), Paediatric ER physicians n = 37, all 37 were basic (100 %) and Paediatricians n = 41 with 36 (87 %) basic and 5 (12 %) advanced. Our results showed significant lack of critical information in the content of epilepsy referrals coming from non-epileptologist providers, largely from the cohort of paediatric ED doctors. This reveals that knowledge and awareness of epilepsy in children remains scarce. Identifying these barriers can provide insights to develop strategies to facilitate accurate identification and rapid triage for children presenting with new onset epilepsy.
{"title":"Heterogeneous knowledge of childhood seizures and epilepsy care in Canadian healthcare Providers: Identifying the gaps","authors":"Kregel Michelle , Sherry Coulson , Emily Guarasci , Andrade Andrea","doi":"10.1016/j.ebr.2024.100733","DOIUrl":"10.1016/j.ebr.2024.100733","url":null,"abstract":"<div><div>Epilepsy is the most common chronic neurological condition in children. Many barriers exist in early recognition which cause delay in care and impact quality of life. Some of these children require advanced treatments which are underutilized due to lack of education, awareness and referrals. Overall, childhood epilepsy is underdiagnosed and poorly understood by non-expert providers. We investigated awareness and knowledge about epilepsy from primary care providers via the quality of their referrals. We prospectively collected and examined all epilepsy related referrals to the Paediatric Neurology Division at Children’s Hospital in London, Ontario, Canada during a six-month period. We developed a modified “epilepsy focused” scoring tool to evaluate the referrals and scored them as basic or advanced. During the study time frame 175 (82 %) referrals met the inclusion criteria. Out of these, 152 (87 %) were identified as basic and 23 (13 %) were advanced (p < 0.001). Amongst the referrals that scored basic vs advanced: Family Doctors n = 49 with 40 basic (81 %) vs 9 advanced (18.3 %), Paediatric ER physicians n = 37, all 37 were basic (100 %) and Paediatricians n = 41 with 36 (87 %) basic and 5 (12 %) advanced. Our results showed significant lack of critical information in the content of epilepsy referrals coming from non-epileptologist providers, largely from the cohort of paediatric ED doctors. This reveals that knowledge and awareness of epilepsy in children remains scarce. Identifying these barriers can provide insights to develop strategies to facilitate accurate identification and rapid triage for children presenting with new onset epilepsy.</div></div>","PeriodicalId":36558,"journal":{"name":"Epilepsy and Behavior Reports","volume":"29 ","pages":"Article 100733"},"PeriodicalIF":1.8,"publicationDate":"2024-12-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11729034/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142980082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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