Article
Clinical Neurology
Jeong-Youn Kim, Jeongyoon Shin, Laehyun Kim, Se Hee Kim
Summary: This study aims to investigate the quantitative electroencephalography (EEG) features associated with a high risk of sudden unexpected death in epilepsy (SUDEP) in patients with Dravet syndrome (DS). The study found that patients with high SUDEP-7 scores have different EEG features from those with low SUDEP-7 scores, suggesting that EEG may be used as a biomarker of SUDEP in DS.
FRONTIERS IN NEUROLOGY
(2023)
Article
Clinical Neurology
Qi Zhang, Ana Suller-Marti, Jane Jian Ding, Gansen Deng, Wenqing He, Jorge G. Burneo, Robert R. Hammond, Lee-Cyn Ang
Summary: Patients with epilepsy are at increased risk for premature death, and SUDEP is one of the main causes. This study reviewed epilepsy-related deaths from 2000 to 2018 and identified characteristics associated with SUDEP. The revised coroner act had an impact on the investigation of epilepsy-related deaths. This is the first detailed clinical-pathological study based on a Canadian cohort, reinforcing previous findings and emphasizing the importance of clinicopathological correlation in accurate classification of epilepsy-related deaths.
Article
Clinical Neurology
Julie Bourgeois-Vionnet, Julien Jung, Romain Bouet, Mathilde Leclercq, Helene Catenoix, Laurent Bezin, Philippe Ryvlin, Sylvain Rheims
Summary: The study found that coffee consumption may be a protective factor for seizure-related respiratory dysfunction, with a dose-dependent effect.
Article
Clinical Neurology
Marius Klovgaard, Thomas H. Lynge, Ioannis Tsiropoulos, Peter Uldall, Jytte Banner, Bo G. Winkel, Philippe Ryvlin, Jacob Tfelt-Hansen, Anne Sabers
Summary: This study in Denmark found that in individuals with epilepsy aged 1-49, the most common causes of death were cancer, SUDEP, and pneumonia, with significantly increased risks compared to those without epilepsy. The research also revealed a lower SUDEP risk in children compared to adults, and identified epilepsy as a major risk factor for sudden unexpected deaths in the background population.
Article
Behavioral Sciences
Ali A. Asadi-Pooya, Eugen Trinka, Francesco Brigo, Coraline Hingray, Ioannis Karakis, Simona Lattanzi, Kette D. Valente, Guilca Contreras, Saule T. Turuspekova, Nirmeen Adel Kishk, Ghaieb Aljandeel, Mohsen Farazdaghi, Yamile Calle Lopez, Najib Kissani, Chahnez Triki, Gunter Kraemer, Rainer Surges, Boulenouar Mesraoua, Hsiang-Yu Yu, Anilu Daza-Restrepo, Taoufik Alsaadi, Abdullah Al-Asmi, Mansur A. Kutlubaev, Chrisma Pretorius, Asel Jusupova, Samson G. Khachatryan, Lakshmi Narasimhan Ranganathan, Abdulaziz Ashkanani, Torbjorn Tomson, David Gigineishvili
Summary: This study investigates the opinions and attitudes of neurologists worldwide regarding counseling about sudden unexpected death in epilepsy (SUDEP). The results indicate that many neurologists rarely discuss the risk of SUDEP with patients and their caregivers, suggesting a severe lack of attention to this matter.
EPILEPSY & BEHAVIOR
(2022)
Article
Behavioral Sciences
Boulenouar Mesraoua, Torbjorn Tomson, Martin Brodie, A. Ali Asadi-Pooya
Summary: Proper education about SUDEP for healthcare professionals, PWE, and their caregivers is crucial in order to implement effective preventive measures.
EPILEPSY & BEHAVIOR
(2022)
Article
Veterinary Sciences
Enrice Huenerfauth, Jasmin Nessler, Johannes Erath, Andrea Tipold
Summary: This study retrospectively analyzed the occurrence of probable sudden unexpected death in dogs with epilepsy (pSUDED) using an online questionnaire. The results suggest that pSUDED occurs in a similar rate as human SUDEP and should be considered as a possible complication in epileptic dogs.
FRONTIERS IN VETERINARY SCIENCE
(2021)
Article
Clinical Neurology
Chris Serrand, Sylvain Rheims, Marie Faucanie, Arielle Crespel, Vera Dinkelacker, William Szurhaj, Arnaud Biraben, Fabrice Bartolomei, Nathalie de Grissac, Elizabeth Landre, Marie Denuelle, Laurent Vercueil, Cecile Marchal, Louis Maillard, Philippe Derambure, Sophie Dupont, Vincent Navarro, Thibault Mura, Audrey Jaussent, Valerie Macioce, Philippe Ryvlin, Marie-Christine Picot
Summary: A case-control study was conducted to identify risk factors for sudden unexpected death in epilepsy (SUDEP) and develop a risk score for prevention. The study found that generalized and nocturnal seizures increased the risk of SUDEP, while the ability to alert someone of an oncoming seizure was protective. The developed SUDEP-CARE score showed good discrimination capabilities and will require external validation.
EUROPEAN JOURNAL OF NEUROLOGY
(2023)
Article
Clinical Neurology
Fengping Yan, Fu Zhang, Yanan Yan, Le Zhang, Yuanyuan Chen
Summary: Epilepsy is a common neurological disorder that is associated with increased morbidity and mortality. Sudden unexpected death in epilepsy (SUDEP) is one of the most common causes for epilepsy-related deaths. The present study investigated the postmortem findings of neurological, cardiac, and pulmonary abnormalities in SUDEP cases.
FRONTIERS IN NEUROLOGY
(2023)
Article
Neurosciences
Alexandra N. Petrucci, Katelyn G. Joyal, Jonathan W. Chou, Rui Li, Kimberly M. Vencer, Gordon F. Buchanan
Summary: The research suggests that serotonin and the dorsal raphe nucleus may regulate the duration of post-ictal generalized EEG suppression following seizures.
Article
Clinical Neurology
Sarah E. Buerki, Cordula Haas, Jacqueline Neubauer
Summary: The study analyzed genetic data from SIDS and SUD cases, focusing on epilepsy-related genes, and found pathogenic/likely pathogenic variants in OPA1, RAI1, SCN3A, SCN5A and TSC2. The post-mortem analysis identified potentially disease-causing variants that might have contributed to sudden death events, emphasizing the importance of counseling on risk and preventive measures in genetic epilepsy. Further research and interpretation of identified variants are needed to better understand the pathophysiological mechanisms in SIDS, SUD, and SUDEP cases.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2023)
Review
Chemistry, Multidisciplinary
He-ming Cheng, Chen-shu Gao, Qiu-wen Lou, Zhong Chen, Yi Wang
Summary: This review focuses on the roles of the raphe nuclei and 5-HTergic system in epilepsy and epilepsy-related comorbidities. The research suggests that the raphe nuclei have an important role in epilepsy and interventions targeting the activity of the raphe nuclei can have diverse effects on seizures and comorbidities. However, there are still many unanswered questions regarding the relationship between the 5-HTergic neural circuit and epilepsy.
ACTA PHARMACOLOGICA SINICA
(2022)
Article
Behavioral Sciences
Mark Farrenburg, Aniket Rali, Anna Grodzinsky, Patrick Landazuri
Summary: The survey results indicate a lack of knowledge and experience among cardiologists regarding seizure-related bradyarrhythmias and SUDEP. Understanding of ictal bradyarrhythmias and postictal bradycardia or asystole is limited, highlighting the potential benefit of formal education on these topics for cardiologists.
EPILEPSY & BEHAVIOR
(2021)
Article
Clinical Neurology
Roozbeh Tarighati Rasekhi, Kathryn N. Devlin, Joely A. Mass, Mustafa Donmez, Burcu Asma, Michael R. Sperling, Maromi Nei
Summary: The study aimed to evaluate the validity of the SUDEP-7 inventory and its components for predicting SUDEP risk, and proposed a new three-item SUDEP-3 inventory which outperformed the SUDEP-7 in predicting SUDEP.
Article
Clinical Neurology
Johnson P. Hampson, Nuria Lacuey, M. R. S. Sandhya Rani, Jaison S. Hampson, Kristina A. Simeone, Timothy A. Simeone, Ponnada A. Narayana, Louis Lemieux, Samden D. Lhatoo
Summary: This study found differences in respiratory chemosensing between patients with epilepsy and healthy controls using functional magnetic resonance imaging (fMRI). The findings suggest that fMRI can detect abnormalities in the brainstem of epilepsy patients, which could be potential biomarkers for sudden unexpected death in epilepsy (SUDEP).
FRONTIERS IN NEUROLOGY
(2022)
Correction
Cardiac & Cardiovascular Systems
Marios Margaritis, Francesca Saini, Ania A. Baranowska-Clarke, Sarah Parsons, Aryan Vink, Charley Budgeon, Natalie Allcock, Bart E. Wagner, Nilesh J. Samani, Jan von derThusen, Jan Lukas Robertus, Mary N. Sheppard, David Adlam
CARDIOVASCULAR RESEARCH
(2023)
Article
Biochemistry & Molecular Biology
Susanna T. E. Cooper, Joseph D. Westaby, Zoe H. R. Haines, Giles O. Malone, Mary N. Sheppard, Daniel N. Meijles
Summary: This study compares human and mouse models to investigate the pathological remodeling process and timeline of hypertensive heart disease. The findings suggest that perivascular fibrosis plays a significant role in disease progression in humans, while cardiomyocyte hypertrophy has less impact. In addition, the study reveals that CD45+ inflammatory cell infiltration is an early response event preceding myocardial inflammation and fibrosis.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Education, Special
Kaitlin K. Moran, Mary E. Sheppard
Summary: This study examines the roles and collaboration between medical professionals and EI/ECSE providers in the Early Intervention and Early Childhood Special Education Systems. The findings reveal that both groups are committed to serving families, but are often frustrated by conflicting recommendations for service delivery.
TOPICS IN EARLY CHILDHOOD SPECIAL EDUCATION
(2023)
Article
Peripheral Vascular Disease
Hossam Alslaim, Sreeja Sanampudi, Driss Raissi, Weibo Fu, Amanda Y. Behr, Mohamed Issa, Mary B. Sheppard, Neal L. Weintraub, Michael Winkler
Summary: Developing a comprehensive and generic schema for characterizing the aorta to standardize data collection and improve the accuracy of aortic measurements. The International College of Angiology Aortic Research Schema is proposed as a comprehensive design that can be applied for various clinically relevant purposes.
INTERNATIONAL JOURNAL OF ANGIOLOGY
(2023)
Article
Clinical Neurology
Jerzy P. P. Szaflarski, Orrin Devinsky, Merrick Lopez, Yong D. D. Park, Pilar Pichon Zentil, Anup D. D. Patel, Elizabeth A. A. Thiele, Robert T. T. Wechsler, Daniel Checketts, Farhad Sahebkar
Summary: The CBD expanded access program initiated in 2014 provided additional CBD treatment for patients with treatment-resistant epilepsies. The study showed that CBD treatment was effective in reducing seizure frequency and had an acceptable safety profile for long-term use in patients with treatment-resistant epilepsies.
Article
Cardiac & Cardiovascular Systems
Joseph David Westaby, Emelia Zullo, Luciana Morais Bicalho, Robert Henry Anderson, Mary Noelle Sheppard
Summary: Descriptive morphological studies of the normal heart are lacking. This study provides normal dimensions of the atria, ventricles, valves and sub-epicardial fat, comparing the findings in terms of sex, age and body measurements. The results show significant differences between males and females in the morphologically normal heart, which should be considered in cardiac imaging, risk stratification, diagnosis, and treatment outcomes.
CARDIOVASCULAR PATHOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Karen L. Kelly, Peter T. Lin, Cristina Basso, Melanie Bois, L. Maximilian Buja, Stephen D. Cohle, Giulia d'Amati, Emily Duncanson, John T. Fallon, Dennis Firchau, Gregory Fishbein, Carla Giordano, Charles Leduc, Silvio H. Litovsky, Shannon Mackey-Bojack, Joseph J. Maleszewski, Katarzyna Michaud, Robert F. Padera, Stavroula A. Papadodima, Sarah Parsons, Stanley J. Radio, Stefania Rizzo, Susan J. Roe, Maria Romero, Mary N. Sheppard, James R. Stone, Carmela D. Tan, Gaetano Thiene, Allard C. van der Wal, John P. Veinot
Summary: Sudden cardiac death is an unexpected death caused by a cardiac condition, accounting for approximately 15-20% of all deaths. It commonly occurs in older adults with acquired heart disease but can also happen in young individuals due to genetic transmission. Investigation of these deaths is crucial due to the potential impact on other family members and the lack of consistent guidelines for cardiac examination in these cases.
CARDIOVASCULAR PATHOLOGY
(2023)
Article
Clinical Neurology
Robert J. Flamini, Anne M. Comi, E. Martina Bebin, Michael G. Chez, Gary Clark, Orrin Devinsky, Shaun A. Hussain, Paul D. Lyons, Anup D. Patel, Jillian L. Rosengard, Farhad Sahebkar, Eric Segal, Laurie Seltzer, Jerzy P. Szaflarski, Arie Weinstock
Summary: The CBD Expanded Access Program provided CBD treatment to patients with treatment-resistant epilepsy and showed a significant reduction in seizure frequency. CBD was well tolerated and effective in treating both convulsive and nonconvulsive seizure types. Controlled trials are needed to further confirm these findings.
Editorial Material
Clinical Neurology
Pavel Klein, Gregory L. Krauss, Bernhard J. Steinhoff, Orrin Devinsky, Michael R. Sperling
Summary: Despite the approval of numerous antiseizure medications (ASMs), a significant number of epilepsy patients still experience seizures. Two new ASMs, cenobamate and fenfluramine, have shown improved efficacy in reducing seizures with sustained results. However, these medications are underutilized, likely due to limited knowledge, access restrictions, and insufficient post-launch information about their efficacy and safety. Addressing these issues can improve seizure control and ultimately reduce morbidity and mortality in epilepsy patients.
Letter
Biochemistry & Molecular Biology
Alexandria N. Tartt, Madeline Mariani, Rene Hen, J. John Mann, Maura Boldrini
MOLECULAR PSYCHIATRY
(2023)
Article
Clinical Neurology
Claudia Zeicu, Antoine Legouhy, Catherine A. Scott, Joana F. A. Oliveira, Gavin P. Winston, John S. Duncan, Sjoerd B. Vos, Maria Thom, Samden Lhatoo, Hui Zhang, Ronald M. Harper, Beate Diehl
Summary: This study investigated the volume and microstructure of the amygdala in patients with epilepsy and found that those with focal-to-bilateral tonic-clonic seizures (FBTCS) had increased amygdala volumes and decreased neurite density compared to healthy controls. Furthermore, patients with post-convulsive central apnea (PCCA) had the highest increase in amygdala volume. These structural alterations may be associated with cardiorespiratory patterns mediated by the amygdala after FBTCS.
Article
Clinical Neurology
Joseph Sullivan, Lieven Lagae, J. Helen Cross, Orrin Devinsky, Renzo G. Guerrini, Kelly Knupp, Linda Laux, Marina Nikanorova, Tilman Polster, Dinesh Talwar, Berten Ceulemans, Rima M. Nabbout, Gail S. Farfel, Bradley R. Galer, Arnold Gammaitoni, Michael Lock, Anupam E. Agarwal, Ingrid Scheffer, FAiRE DS Study Grp
Summary: This study examined the safety and effectiveness of fenfluramine in treating convulsive seizures in patients with Dravet syndrome. The results showed that patients treated with fenfluramine had lower seizure frequency and longer interval between seizures compared to the placebo group. Common side effects of fenfluramine were observed, but no evidence of severe cardiac or vascular issues was found.
Review
Clinical Neurology
Laura Gould, Victoria Delavale, Caitlin Plovnick, Thomas Wisniewski, Orrin Devinsky
Summary: Febrile seizures are associated with an increased risk of epilepsy and rare cases of sudden unexplained death. Mortality rates varied in different studies, with some reporting no deaths and others identifying a significant percentage of deaths associated with febrile seizures. Minor hippocampal histopathological anomalies were common in sudden deaths with or without a history of febrile seizures. Most electroencephalography (EEG) studies were normal, and neuroimaging studies suggested increased right hippocampal volumes. Longer-term prospective studies are needed to fully understand the outcomes of simple or brief complex febrile seizures.
Article
Multidisciplinary Sciences
Rodrigo Ordonez Sierra, Lizeth Katherine Pedraza, Livia Barcsai, Andrea Pejin, Qun Li, Gabor Kozak, Yuichi Takeuchi, Anett J. Nagy, Magor L. Lorincz, Orrin Devinsky, Gyoergy Buzsaki, Antal Berenyi
Summary: Dysregulated fear reactions can be caused by maladaptive processing of trauma-related memories. By manipulating hippocampal SWRs and cortical oscillations, fear extinction can be enhanced in male rats. The modified fear memories become resistant to recall and do not spontaneously reemerge. This effect is mediated by D2 receptor signaling-induced synaptic remodeling in the basolateral amygdala. These findings demonstrate the potential of neuromodulation in augmenting extinction learning and provide a new avenue for anxiety disorder treatments.
NATURE COMMUNICATIONS
(2023)
Article
Clinical Neurology
Heather E. Olson, Sam Amin, Nadia Bahi-Buisson, Orrin Devinsky, Eric D. Marsh, Elia Pestana-Knight, Rajsekar R. Rajaraman, Alex A. Aimetti, Eva Rybak, Fanhui Kong, Ian Miller, Joseph Hulihan, Scott Demarest
Summary: This study reports the 2-year safety and clinical outcomes of ganaxolone treatment in patients with CDD. The results show that ganaxolone significantly reduces major motor seizure frequency and maintains its efficacy and safety over the long term.