1,437 results match your criteria Epilepsy Sudden Unexpected Death in


Forecasting Seizure Clusters from Chronic Ambulatory Electrocorticography.

Epilepsia 2022 Jun 25. Epub 2022 Jun 25.

Texas Institute for Restorative Neurotechnologies, The University of Texas Health Science Center at Houston, Houston, TX, USA.

Seizure clusters are seizures that occur in rapid succession during periods of heightened seizure risk and are associated with substantial morbidity and sudden unexpected death in epilepsy. The objective of this feasibility study is to evaluate the performance of a novel seizure cluster forecasting algorithm. Chronic ambulatory electrocorticography recorded over an average of 38 months in 10 subjects with drug-resistant epilepsies was analyzed pseudoprospectively by dividing data into training (first 85%) and validation periods. Read More

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Changes of oxygen saturation in patients with pure temporal lobe epilepsy.

Seizure 2022 Jun 16;100:30-35. Epub 2022 Jun 16.

Hacettepe University Medicine Faculty, Department of Neurology, Ankara, Turkey. Electronic address:

Purpose: Ictal hypoxemia is accepted as one of the mechanisms underlying sudden unexpected death in epilepsy (SUDEP). Although ictal hypoxemia is more common in generalized seizures, it also occurs in focal seizures with or without generalization. In this study, we aimed to show the relationship between clinical and electroencephalographic findings of seizures in patients with temporal lobe epilepsy (TLE) with periictal oxygen saturation. Read More

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How the characteristics of pediatric neurologists in Latin America influence the communication of sudden unexpected death in epilepsy to patients and caregivers.

Epilepsia Open 2022 Jun 20. Epub 2022 Jun 20.

Faculty of Medicine, Universidad de Chile.

Objective: This study aimed to describe the characteristics of pediatric neurologists (PNs) in Latin America (LA) who attend to children and adolescents with epilepsy and convey to them the risk of sudden unexpected death in epilepsy (SUDEP).

Methods: Personal data as well as details of discussion of SUDEP with families, including relevance of SUDEP disclosure, frequency of such communication, perceived benefits and risks of disclosure, extent of training received on such disclosure, and professional experience with SUDEP were collected through an online survey of PNs from LA. Their personal experience in carrying out this conversation was obtained through responses to an open question, further used to identify the main barriers. Read More

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Cortical neuronal hypertrophy and MTOR pathway activation in CAN regions in SUDEP.

Epilepsia 2022 Jun 18. Epub 2022 Jun 18.

Department of Clinical and Experimental Epilepsy.

Objectives: Dysfunctional connectivity and pre-existing structural abnormalities of central autonomic network regions (CAN) have been shown on MRI in sudden and unexpected death in epilepsy (SUDEP) and may be mechanistically relevant. In a previous post-mortem study we reported increased microglia in CAN regions, including the superior temporal gyrus (STG) in SUDEP. In this current study we investigated mTOR pathway activation and neuronal c-Fos activation in CAN regions in SUDEP compared to control groups. Read More

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Preferences and User Experiences of Wearable Devices in Epilepsy: A Systematic Review and Mixed-Methods Synthesis.

Neurology 2022 Jun 15. Epub 2022 Jun 15.

Department of Neuroscience, Central Clinical School, Monash University, Melbourne, 3004, Victoria, Australia

Background And Objectives: To examine the preferences and user experiences of people with epilepsy and caregivers regarding automated wearable seizure detection devices.

Methods: We performed a mixed-methods systematic review. We searched electronic databases for original peer-reviewed publications between January 1, 2000, and May 26, 2021. Read More

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Abnormal heart rate variability during non-REM sleep and postictal generalized EEG suppression in focal epilepsy.

Clin Neurophysiol 2022 May 27;140:40-44. Epub 2022 May 27.

Department of Epileptology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

Objective: To identify any relationship between abnormal interictal heart rate variability (HRV) during sleep and the occurrence of postictal generalized EEG suppression (PGES), a potential biomarker of sudden unexpected death in epilepsy (SUDEP), in patients with focal epilepsy.

Methods: We retrospectively reviewed 34 consecutive patients with focal epilepsy, who presented with focal to bilateral tonic-clonic seizures (FBTCSs), 19 with PGES (PGES+) and 15 without PGES (PGES-), and 14 patients without epilepsy as controls. HRV spectrum analysis was performed for periods of 10 minutes during wakefulness, non-REM sleep, and REM sleep. Read More

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The Contribution of HCN Channelopathies in Different Epileptic Syndromes, Mechanisms, Modulators, and Potential Treatment Targets: A Systematic Review.

Front Mol Neurosci 2022 19;15:807202. Epub 2022 May 19.

Department of Pediatrics, Xiangya Hospital, Central South University, Changsha, China.

Background: Hyperpolarization-activated cyclic nucleotide-gated (HCN) current reduces dendritic summation, suppresses dendritic calcium spikes, and enables inhibitory GABA-mediated postsynaptic potentials, thereby suppressing epilepsy. However, it is unclear whether increased HCN current can produce epilepsy. We hypothesized that gain-of-function (GOF) and loss-of-function (LOF) variants of HCN channel genes may cause epilepsy. Read More

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Common ictal and interictal perfusion patterns. A window into the epileptogenic network and SUDEP mechanism in Drug Resistant Focal Epilepsy?

Curr Pharm Des 2022 Jun 3. Epub 2022 Jun 3.

Clinical Neurophysiology International Center of Neurologic Restoration Cuba.

Background: Focal epilepsies have been described as network disease. Noninvasive investigative techniques have been used to characterize epileptogenic networks.

Objetive: To describe ictal and interictal cortical and subcortical perfusion patterns using single photon emission computed tomography (SPECT), in patients with drug-resistant epilepsy (DRE). Read More

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Genetic therapeutic advancements for Dravet Syndrome.

Epilepsy Behav 2022 Jul 30;132:108741. Epub 2022 May 30.

UCL Institute for Women's Health, 86-96 Chenies Mews, London WC1E 6HX, UK. Electronic address:

Dravet Syndrome is a genetic epileptic syndrome characterized by severe and intractable seizures associated with cognitive, motor, and behavioral impairments. The disease is also linked with increased mortality mainly due to sudden unexpected death in epilepsy. Over 80% of cases are due to a de novo mutation in one allele of the SCN1A gene, which encodes the α-subunit of the voltage-gated ion channel Na1. Read More

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Sudden Unexpected Death in Epilepsy: A Report of Three Commonly Encountered Anatomic Findings in the Forensic Setting With Recommendations for Best Practices.

Am J Forensic Med Pathol 2022 May 30. Epub 2022 May 30.

From the Department of Pathology, Wake Forest School of Medicine, Winston-Salem, NC.

Abstract: Sudden unexplained death in epilepsy (SUDEP) is the most common cause of death in children and young adults with epilepsy with epileptic patients harboring a 27 times increased risk of death from SUDEP. Structural brain lesions are encountered in up to 50% of autopsy cases. In this case series, we report 3 previously undiagnosed structural causes of SUDEP discovered at autopsy at our institution including schizencephaly, ganglioglioma, and focal cortical dysplasia. Read More

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Sudden unexpected death in epilepsy (SUDEP): Definition, epidemiology, and significance of education.

Epilepsy Behav 2022 Jul 24;132:108742. Epub 2022 May 24.

Epilepsy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran; Jefferson Comprehensive Epilepsy Center, Department of Neurology, Thomas Jefferson University, Philadelphia, PA, USA. Electronic address:

People with epilepsy (PWE) may die suddenly and unexpectedly and without a clear under-lying pathological etiology; this is called SUDEP (sudden unexpected death in epilepsy). The pooled estimated incidence rate for SUDEP is 23 times the incidence rate of sudden death in the general population with the same age. Empowering healthcare professionals, PWE, and their care-givers with the appropriate knowledge about SUDEP is very important to enable efficient preventive measures in PWE. Read More

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Toward automated prediction of sudden unexpected death in epilepsy.

Authors:
Bin Gu Hojjat Adeli

Rev Neurosci 2022 May 27. Epub 2022 May 27.

Department of Neuroscience, Ohio State University, Columbus, OH 43210, USA.

Sudden unexpected death in epilepsy (SUDEP) is a devastating yet overlooked complication of epilepsy. The rare and complex nature of SUDEP makes it challenging to study. No prediction or prevention of SUDEP is currently available in a clinical setting. Read More

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The diverse role of the raphe 5-HTergic systems in epilepsy.

Acta Pharmacol Sin 2022 May 25. Epub 2022 May 25.

Key Laboratory of Neuropharmacology and Translational Medicine of Zhejiang Province, School of Pharmaceutical Sciences, Zhejiang Chinese Medical University, Hangzhou, 310053, China.

The raphe nuclei comprise nearly all of 5-hydroxytryptaminergic (5-HTergic) neurons in the brain and are widely acknowledged to participate in the modulation of neural excitability. "Excitability-inhibition imbalance" results in a variety of brain disorders, including epilepsy. Epilepsy is a common neurological disorder characterized by hypersynchronous epileptic seizures accompanied by many psychological, social, cognitive consequences. Read More

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Disruption of Synaptic Transmission in the Bed Nucleus of the Stria Terminalis Reduces Seizure-Induced Death in DBA/1 Mice and Alters Brainstem E/I Balance.

ASN Neuro 2022 Jan-Dec;14:17590914221103188

Department of Neurology, 12328Vanderbilt University Medical Center, Nashville, TN, USA.

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in refractory epilepsy patients. Accumulating evidence from recent human studies and animal models suggests that seizure-related respiratory arrest may be important for initiating cardiorespiratory arrest and death. Prior evidence suggests that apnea onset can coincide with seizure spread to the amygdala and that stimulation of the amygdala can reliably induce apneas in epilepsy patients, potentially implicating amygdalar regions in seizure-related respiratory arrest and subsequent postictal hypoventilation and cardiorespiratory death. Read More

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Fine mapping and candidate gene analysis of a dravet syndrome modifier locus on mouse chromosome 11.

Mamm Genome 2022 May 23. Epub 2022 May 23.

Department of Pharmacology, Northwestern University Feinberg School of Medicine, 320 E. Superior St., Searle 8-510, Chicago, IL, 60611, USA.

Pathogenic variants in SCN1A result in a spectrum of phenotypes ranging from mild febrile seizures to Dravet syndrome, a severe infant-onset epileptic encephalopathy. Individuals with Dravet syndrome have developmental delays, elevated risk for sudden unexpected death in epilepsy (SUDEP), and have multiple seizure types that are often refractory to treatment. Although most Dravet syndrome variants arise de novo, there are cases where an SCN1A variant was inherited from mildly affected parents, as well as some individuals with de novo loss-of-function or truncation mutations that presented with milder phenotypes. Read More

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Vagus nerve stimulation for conservative therapy-refractive epilepsy and depression.

Laryngorhinootologie 2022 May 23;101(S 01):S114-S143. Epub 2022 May 23.

Klinik für HNO-Heilkunde, Kopf- und Halschirurgie, KRH Klinikum Nordstadt, Hannover.

Numerous studies confirm that the vagus nerve stimulation (VNS) is an efficient, indirect neuromodulatory therapy with electrically induced current for epilepsy that cannot be treated by epilepsy surgery and is therapy-refractory and for drug therapy-refractory depression. VNS is an established, evidence-based and in the long-term cost-effective therapy in an interdisciplinary overall concept.Long-term data on the safety and tolerance of the method are available despite the heterogeneity of the patient populations. Read More

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The contribution of fenfluramine to the treatment of Dravet syndrome in Spain through Multi-Criteria Decision Analysis.

Epilepsy Behav 2022 Jul 16;132:108711. Epub 2022 May 16.

Omakase Consulting S.L., Barcelona, Spain. Electronic address:

Introduction: Dravet Syndrome (DS) is a severe, developmental epileptic encephalopathy (DEE) that begins in infancy and is characterized by pharmaco-resistant epilepsy and neurodevelopmental delay. Despite available antiseizure medications (ASMs), there is a need for new therapeutic options with greater efficacy in reducing seizure frequency and with adequate safety and tolerability profiles. Fenfluramine is a new ASM for the treatment of seizures associated with DS as add-on therapy to other ASMs for patients aged 2 years and older. Read More

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Discussion of the Brazilian neurologists about sudden unexpected death in epilepsy.

Rev Assoc Med Bras (1992) 2022 May;68(5):675-679

Escola Paulista de Medicina/Universidade Federal de São Paulo, Disciplina de Neurologia Experimental - São Paulo (SP), Brazil.

Objective: This study aimed to evaluate the concept of health professionals affiliated with the Brazilian League of Epilepsy on whether or not to inform patients about the risk factors related to the occurrence of sudden unexpected death in epilepsy.

Methods: A descriptive research of inquiry was conducted with direct survey on the Brazilian neurologist's view, regarding medical behavior in the health area to report or not about the risk of sudden unexpected death in epilepsy. Data collection consisted of a structured questionnaire available online. Read More

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Correction to: Ictal neural oscillatory alterations precede sudden unexpected death in epilepsy.

Authors:

Brain Commun 2022 13;4(3):fcac121. Epub 2022 May 13.

[This corrects the article DOI: 10.1093/braincomms/fcac073.]. Read More

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Pathogenicity Assignment of Variants in Genes Associated With Cardiac Channelopathies Evolve Toward Diagnostic Uncertainty.

Circ Genom Precis Med 2022 Jun 11;15(3):e003491. Epub 2022 May 11.

Division of Pediatric Cardiology, Department of Pediatrics (M.B.R., I.M.L., A.P.L.), Duke University School of Medicine, Durham, NC.

Background: Accurately determining variant pathogenicity is critical in the diagnosis of cardiac channelopathies; however, it remains unknown how variant pathogenicity status changes over time. Our aim is to use a comprehensive analysis of ClinVar to understand the mutability of variant evaluation in channelopathy-associated genes to inform clinical decision-making around variant calling.

Methods: We identified 10 genes () strongly associated with cardiac channelopathies, as well as 3 comparison gene sets (disputed long QT syndrome, sudden unexpected death in epilepsy, and all ClinVar). Read More

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Perampanel enhances the cardiovagal tone and heart rate variability (HRV) in patients with drug-resistant temporal lobe epilepsy.

Seizure 2022 Jul 29;99:16-23. Epub 2022 Apr 29.

Department of Geriatrics, Neurosciences & Orthopedics Unit of Neurophysiopathology and Sleep Medicine, IRCCS Policlinico Universitario Agostino Gemelli Catholic University, Rome.

Objective: The temporal lobe plays a central role in the regulation of the "Central Autonomic Network" and cardiovascular functions. The blockade of glutamatergic pathways in the temporal lobe affects cardio-autonomic control. Perampanel (PER) is a non-competitive agonist of the AMPA receptor. Read More

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The recovery position for maintenance of adequate ventilation and the prevention of cardiac arrest: A systematic review.

Resusc Plus 2022 Jun 29;10:100236. Epub 2022 Apr 29.

Department of Emergency Medicine, University of Virginia, United States of America.

Aim: To conduct a systematic review of the use of the recovery position in adults and children with non-traumatic decreased levels of responsiveness changes outcomes in comparison with other positioning strategies.

Methods: We searched Medline (Ovid), Embase, Cochrane Library, CINAHL, medRxiv and Google Scholar from inception to 15 March 2021 for studies involving adults and children in an out-of-hospital, first aid setting who had reduced levels of responsiveness of non-traumatic aetiology but did not require resuscitative interventions. We used the ROBINS-I tool to assess risk of bias and GRADE methodology to determine the certainty of evidence. Read More

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Life-span characterization of epilepsy and comorbidities in Dravet syndrome mice carrying a targeted deletion of exon 1 of the Scn1a gene.

Exp Neurol 2022 Aug 26;354:114090. Epub 2022 Apr 26.

Department of Physiology and Medical Physics, RCSI University of Medicine and Health Sciences, Dublin, Ireland; FutureNeuro SFI Research Centre, RCSI University of Medicine and Health Sciences, Dublin, Ireland. Electronic address:

Objective: Dravet Syndrome (DS) is a catastrophic form of paediatric epilepsy associated with multiple comorbidities mainly caused by mutations in the SCN1A gene. DS progresses in three different phases termed febrile, worsening and stabilization stage. Mice that are haploinsufficient for Scn1a faithfully model each stage of DS, although various aspects have not been fully described, including the temporal appearance and sex differences of the epilepsy and comorbidities. Read More

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TAU ablation in excitatory neurons and postnatal TAU knockdown reduce epilepsy, SUDEP, and autism behaviors in a Dravet syndrome model.

Sci Transl Med 2022 04 27;14(642):eabm5527. Epub 2022 Apr 27.

Gladstone Institute of Neurological Disease, Gladstone Institutes, San Francisco, CA 94158, USA.

Intracellular accumulation of TAU aggregates is a hallmark of several neurodegenerative diseases. However, global genetic reduction of TAU is beneficial also in models of other brain disorders that lack such TAU pathology, suggesting a pathogenic role of nonaggregated TAU. Here, conditional ablation of TAU in excitatory, but not inhibitory, neurons reduced epilepsy, sudden unexpected death in epilepsy, overactivation of the phosphoinositide 3-kinase-AKT-mammalian target of rapamycin pathway, brain overgrowth (megalencephaly), and autism-like behaviors in a mouse model of Dravet syndrome, a severe epileptic encephalopathy of early childhood. Read More

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Ictal neural oscillatory alterations precede sudden unexpected death in epilepsy.

Brain Commun 2022 25;4(2):fcac073. Epub 2022 Mar 25.

Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, NC, USA.

Sudden unexpected death in epilepsy is the most catastrophic outcome of epilepsy. Each year there are as many as 1.65 cases of such death for every 1000 individuals with epilepsy. Read More

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New inroads into the brain circuits and network dynamics behind sudden unexpected death in epilepsy.

Brain Commun 2022 13;4(2):fcac097. Epub 2022 Apr 13.

Simons Initiative for the Developing Brain, Patrick Wild Centre, Centre for Discovery Brain Sciences, University of Edinburgh, Edinburgh EH8 9XD, UK.

This scientific commentary refers to 'Hyperexcitable superior colliculus and fatal brainstem spreading depolarization in a model of sudden unexpected death in epilepsy' by Cain . (https://doi.org/10. Read More

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Hyperexcitable superior colliculus and fatal brainstem spreading depolarization in a model of Sudden Unexpected Death in Epilepsy.

Brain Commun 2022 19;4(2):fcac006. Epub 2022 Jan 19.

Djavad Mowafaghian Centre for Brain Health, University of British Columbia, Vancouver, Canada.

Cardiorespiratory arrest and death in mouse models of sudden unexpected death in epilepsy occur when spreading depolarization is triggered by cortical seizures and then propagates to the brainstem. However, the critical brain regions and the specific changes required to allow spreading depolarization to propagate to the brainstem under the relatively rare circumstances leading to a fatal seizure are unknown. We previously found that following cortical seizure-inducing electrical stimulation, spreading depolarization could occur in both the superior and inferior colliculi in Cacna1a mice, but was never observed in wild-type animals or following non-seizure-inducing stimuli in Cacna1a mice. Read More

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January 2022

Dravet syndrome in children-A population-based study.

Epilepsy Res 2022 05 8;182:106922. Epub 2022 Apr 8.

Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, 405 30 Gothenburg, Sweden; Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Member of the ERN EpiCARE, 413 45 Gothenburg, Sweden.

Objective: The aim was to describe age at diagnosis, cumulative incidence, SCN1A variants, mortality, seizure types and treatments in children with Dravet Syndrome (DS) in Sweden.

Methods: Children diagnosed with DS, born between January 1st 2000 and December 31st 2018 were included in a population-based study. Clinical data, frequency of seizure types and treatments were collected from caregivers and medical records in 42 children. Read More

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