1,255 results match your criteria Sudden Unexpected Death in Epilepsy

Probable Sudden Unexpected Death in Dogs With Epilepsy (pSUDED).

Front Vet Sci 2021 27;8:600307. Epub 2021 Apr 27.

Department of Small Animal Medicine and Surgery, University of Veterinary Medicine, Hannover, Germany.

Sudden unexpected death in human epileptic patients (SUDEP) is defined as death related to recurrent unprovoked seizures, death occurring unexpectedly, and suddenly in a patient with reasonable state of health, without an obvious medical cause of death, trauma, asphyxia, or intractable status epilepticus, and in post mortem examination no obvious reason for death can be found. "Probable SUDEP" (pSUDEP) is defined as SUDEP not confirmed pathologically. The adapted abbreviation for dogs is used in the following: "pSUDED" (probable sudden unexpected death in dogs with epilepsy). Read More

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Early onset epilepsy and sudden unexpected death in epilepsy with cardiac arrhythmia in mice carrying the early infantile epileptic encephalopathy 47 gain-of-function FHF1(FGF12) missense mutation.

Epilepsia 2021 May 13. Epub 2021 May 13.

Department of Biological Sciences, Hunter College of City University of New York, New York, New York, USA.

Objective: Fibroblast growth factor homologous factors (FHFs) are brain and cardiac sodium channel-binding proteins that modulate channel density and inactivation gating. A recurrent de novo gain-of-function missense mutation in the FHF1(FGF12) gene (p.Arg52His) is associated with early infantile epileptic encephalopathy 47 (EIEE47; Online Mendelian Inheritance in Man database 617166). Read More

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Regional microglial populations in central autonomic brain regions in SUDEP.

Epilepsia 2021 May 4. Epub 2021 May 4.

Departments of Clinical and Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, UK.

Objective: Sudden unexpected death in epilepsy (SUDEP) may arise as a result of autonomic dysfunction during a seizure. The central autonomic networks (CANs) modulate brainstem cardiorespiratory regulation. Recent magnetic resonance imaging (MRI) studies in SUDEP have shown cortical and subcortical volume changes and altered connectivity between CAN regions, but the pathological correlate is unknown. Read More

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Towards Mutation-Specific Precision Medicine in Atypical Clinical Phenotypes of Inherited Arrhythmia Syndromes.

Int J Mol Sci 2021 Apr 10;22(8). Epub 2021 Apr 10.

Department of Cardiovascular Medicine, Gunma University Graduate School of Medicine, Maebashi 371-8511, Gunma, Japan.

Most causal genes for inherited arrhythmia syndromes (IASs) encode cardiac ion channel-related proteins. Genotype-phenotype studies and functional analyses of mutant genes, using heterologous expression systems and animal models, have revealed the pathophysiology of IASs and enabled, in part, the establishment of causal gene-specific precision medicine. Additionally, the utilization of induced pluripotent stem cell (iPSC) technology have provided further insights into the pathophysiology of IASs and novel promising therapeutic strategies, especially in long QT syndrome. Read More

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Sudden Unexpected Death in Epilepsy: A PersonaliZed Prediction Tool.

Neurology 2021 Apr 28. Epub 2021 Apr 28.

Ashwani Jha, NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK; Cheongeun Oh,Division of Biostatistics, Department of Population Health, New York University Langone Health, New York, NY, USA;Dale Hesdorffer,Department of Epidemiology, Columbia University Medical Center, New York, NY, USA; Beate Diehl, NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK; Sasha Devore, Comprehensive Epilepsy Center, New York University Langone Medical Center, New York, NY, USA; Martin Brodie,Epilepsy Unit, University of Glasgow, Glasgow, Scotland; Torbjörn Tomson,Department of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden; Josemir W. Sander,NIHR University College London Hospitals Biomedical Research Centre, UCL Queen Square Institute of Neurology, London, WC1N 3BG, UK & Chalfont Centre for Epilepsy, Chalfont St Peter, SL9 0RJ, UK & Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, Netherlands; Thaddeus S. Walczak,MINCEP Comprehensive Epilepsy Center, University of Minnesota, Minneapolis, MN, USA;Orrin Devinsky,Comprehensive Epilepsy Center, New York University Langone Medical Center, New York, NY, USA.

Objective: To develop and validate a tool for individualised prediction of Sudden Unexpected Death in Epilepsy (SUDEP) risk, we re-analysed data from one cohort and three case-control studies undertaken 1980-2005.

Methods: We entered 1273 epilepsy cases (287 SUDEP, 986 controls) and 22 clinical predictor variables into a Bayesian logistic regression model.

Results: Cross-validated individualized model predictions were superior to baseline models developed from only average population risk or from generalised tonic-clonic seizure frequency (pairwise difference in leave-one-subject-out expected log posterior density = 35. Read More

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Proteomics and Transcriptomics of the Hippocampus and Cortex in SUDEP and High-Risk SUDEP Patients.

Neurology 2021 Apr 28. Epub 2021 Apr 28.

Comprehensive Epilepsy Center, NYU School of Medicine, New York, NY, USA.

Objective: To identify the molecular signaling pathways underlying sudden unexpected death in epilepsy (SUDEP) and high-risk SUDEP compared to epilepsy control patients.

Methods: For proteomics analyses, we evaluated the hippocampus and frontal cortex from microdissected post-mortem brain tissue of 12 SUDEP and 14 non-SUDEP epilepsy patients. For transcriptomics analyses, we evaluated hippocampus and temporal cortex surgical brain tissue from mesial temporal lobe epilepsy (MTLE) patients: 6 low-risk and 8 high-risk SUDEP as determined by a short (< 50 seconds) or prolonged (≥ 50 seconds) postictal generalized EEG suppression (PGES) that may indicate severely depressed brain activity impairing respiration, arousal, and protective reflexes. Read More

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Kv1.1 subunits localize to cardiorespiratory brain networks in mice where their absence induces astrogliosis and microgliosis.

Mol Cell Neurosci 2021 Apr 24;113:103615. Epub 2021 Apr 24.

Department of Cellular Biology & Anatomy, Louisiana State University Health Sciences Center, Shreveport, LA, USA; Department of Biological Sciences, Southern Methodist University, Dallas, TX, USA. Electronic address:

Cardiorespiratory collapse following a seizure is a suspected cause of sudden unexpected death in epilepsy (SUDEP), the leading cause of epilepsy-related mortality. In the commonly used Kcna1 gene knockout (Kcna1) mouse model of SUDEP, cardiorespiratory profiling reveals an array of aberrant breathing patterns that could contribute to risk of seizure-related mortality. However, the brain structures mediating these respiratory abnormalities remain unknown. Read More

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Mutations Are Associated With Benign Epilepsy of Childhood With Centrotemporal Spikes With or Without Arrhythmia.

Front Neurosci 2021 7;15:629610. Epub 2021 Apr 7.

Institute of Neuroscience and Department of Neurology of the Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou, China.

encodes ryanodine receptor 2 protein (RYR-2) that is mainly located on endoplasmic reticulum membrane and regulates intracellular calcium concentration. The RYR-2 protein is ubiquitously distributed and highly expressed in the heart and brain. Previous studies have identified the mutations in the etiology of arrhythmogenic right ventricular dysplasia 2 and catecholaminergic polymorphic ventricular tachycardia. Read More

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Origin of post-ictal and post-anesthesia adverse effects and possibly of SUDEP.

Med Hypotheses 2021 Apr 9;151:110591. Epub 2021 Apr 9.

Physics Dept., Sami Shamoon College of Engineering, Beer-Sheva, Israel.

The origin of post-ictal malfunctions is debatable. We want to propose a novel idea of a cause of these adverse results occurring following epileptic seizures and anesthesia. Previously we have put forward the idea that epileptic seizures termination is caused by the function of the glymphatic system in the brain. Read More

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Multi-system Monitoring for Identification of Seizures, Arrhythmias and Apnea in Conscious Restrained Rabbits.

J Vis Exp 2021 03 27(169). Epub 2021 Mar 27.

Department of Pharmacology, Upstate Medical University;

Patients with ion channelopathies are at a high risk of developing seizures and fatal cardiac arrhythmias. There is a higher prevalence of heart disease and arrhythmias in people with epilepsy (i.e. Read More

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Vagus nerve stimulation does not alter brainstem nuclei morphology in patients with refractory epilepsy.

Epilepsy Behav 2021 May 7;118:107940. Epub 2021 Apr 7.

Department of Clinical Neurological Sciences, London Health Sciences Centre, Western University, London, Ontario, Canada; Department of Pathology and Lab Medicine, London Health Sciences Centre, Western University, London, Ontario, Canada. Electronic address:

Objective: To describe morphological characteristics of the brainstem nuclei in response to chronic vagus nerve stimulation (VNS) in patients with refractory epilepsy.

Background: VNS is a treatment option for individuals with medically refractory epilepsy. While treatment with VNS may achieve up to 50% seizure reduction and is protective against sudden unexpected death in epilepsy (SUDEP), its mechanism of action is not fully understood. Read More

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Cardiac Autonomic Dysfunction and Risk of Sudden Unexpected Death in Epilepsy.

Neurology 2021 Apr 9. Epub 2021 Apr 9.

: INSERM CIC-IT 1403, Lille University Medical Center, Lille, France.

Objective: We aimed to test whether patients who died from sudden unexpected death in epilepsy (SUDEP) had an abnormal cardiac autonomic response to sympathetic stimulation by hyperventilation.

Methods: We conducted a retrospective, observational, case-control study of a group of patients who died from SUDEP and controls who were matched with the patients for epilepsy type, drug resistance, sex, age at electroencephalogram recording, age at onset of epilepsy, and duration of epilepsy. We analyzed the heart rate (HR) and heart rate variability (HRV) at rest, during and after hyperventilation performed during the patient's last electroencephalogram recording before SUDEP. Read More

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The SANTÉ study at 10 years of follow-up: Effectiveness, safety, and sudden unexpected death in epilepsy.

Epilepsia 2021 Apr 8. Epub 2021 Apr 8.

Department of Neurology, Stanford University, Stanford, California, USA.

Objective: We evaluated the efficacy and safety of deep brain anterior thalamus stimulation after 7 and 10 years, and report the incidence of sudden unexpected death in epilepsy (SUDEP) and overall mortality in adults in the Stimulation of the Anterior Nucleus of the Thalamus for Epilepsy (SANTÉ) study.

Methods: After the 3-month blinded and 9-month unblinded phases, subjects continued to be assessed during long-term follow-up (LTFU) and later a continued therapy access phase (CAP), to further characterize adverse events and the incidence of SUDEP. Stimulus parameter and medication changes were allowed. Read More

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SCN8A-related developmental and epileptic encephalopathy with ictal asystole requiring cardiac pacemaker implantation.

Brain Dev 2021 Apr 4. Epub 2021 Apr 4.

Department of Pediatrics and Neonatology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Introduction: SCN8A-related epilepsy has various phenotypes. In particular, patients with developmental and epileptic encephalopathy (DEE) are resistant to antiepileptic drugs and may present with autonomic symptoms, such as marked bradycardia and apnea during seizures, and thus have an increased risk of sudden death. Herein, we report a case of very severe SCN8A-related epilepsy necessitating cardiac pacemaker implantation because of repetitive ictal asystole. Read More

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Risk estimation of SUDEP during COVID-19 pandemic era in a tertiary referral center.

Epilepsy Res 2021 Jul 24;173:106625. Epub 2021 Mar 24.

Department of Neurology, Kasr Al-Ainy Faculty of Medicine, Cairo University, Cairo, Egypt.

Objective: No data exist regarding the impact of the lockdown due to the COVID-19 pandemic on the risk factors of sudden unexpected death in epilepsy (SUDEP). This study aimed to stratify risk factors of SUDEP in relation to COVID-19 lockdown, among patients with epilepsy (PWE) in Cairo University epilepsy unit (CUEU). Therefore, we can detect risk factors and mitigate such factors in the second wave of the virus. Read More

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Immunoreactivity of Muscarinic Acetylcholine M2 and Serotonin 5-HT2B Receptors, Norepinephrine Transporter and Kir Channels in a Model of Epilepsy.

Life (Basel) 2021 Mar 26;11(4). Epub 2021 Mar 26.

Comprehensive Epilepsy Center, Department of Neurology, NYU Langone School of Medicine, New York, NY 10010, USA.

Epilepsy is characterized by an imbalance in neurotransmitter activity; an increased excitatory to an inhibitory activity. Acetylcholine (ACh), serotonin, and norepinephrine (NE) may modulate neural activity via several mechanisms, mainly through its receptors/transporter activity and alterations in the extracellular potassium (K) concentration via K ion channels. Seizures may disrupt the regulation of inwardly rectifying K (Kir) channels and alter the receptor/transporter activity. Read More

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Differential Methylation in the Regulatory Region in Sudden Unexplained Death and Sudden Unexpected Death in Epilepsy.

Int J Mol Sci 2021 Mar 10;22(6). Epub 2021 Mar 10.

Section of Forensic Genetics, Department of Forensic Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, DK-1353 Copenhagen, Denmark.

Sudden cardiac death (SCD) is a diagnostic challenge in forensic medicine. In a relatively large proportion of the SCDs, the deaths remain unexplained after autopsy. This challenge is likely caused by unknown disease mechanisms. Read More

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Proceedings of the Sleep and Epilepsy Workshop: Section 3 Mortality: Sleep, Night, and SUDEP.

Epilepsy Curr 2021 Mar 31:15357597211004556. Epub 2021 Mar 31.

Department of Neurology, 1861Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in patients with refractory epilepsy. Likely pathophysiological mechanisms include seizure-induced cardiac and respiratory dysregulation. A frequently identified feature in SUDEP cases is that they occur at night. Read More

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Sleep and Epilepsy: a Focused Review of Pathophysiology, Clinical Syndromes, Co-morbidities, and Therapy.

Neurotherapeutics 2021 01 30;18(1):170-180. Epub 2021 Mar 30.

Division of Epilepsy and Sleep, Columbia University, New York, NY, USA.

A healthy brain requires balancing of waking and sleeping states. The normal changes in waking and sleeping states result in neurophysiological conditions that either increase or decrease the tendency of seizures and interictal discharges to occur. This article reviews the manifold and complex relationships between sleep and epilepsy and discusses treatment of the sleep-related epilepsies. Read More

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January 2021

Characterization of the Insular Role in Cardiac Function through Intracranial Electrical Stimulation of the Human Insula.

Ann Neurol 2021 Mar 30. Epub 2021 Mar 30.

Epilepsy Monitoring Unit, Department of Neurology, Member of ERN EpiCARE, Hospital del Mar, Barcelona, Spain.

Objective: The link between brain function and cardiovascular dynamics is an important issue yet to be elucidated completely. The insula is a neocortical brain area that is thought to have a cardiac chronotropic regulatory function, but its role in cardiac contractility is unknown. We aimed to analyze the variability in heart rate and cardiac contractility after functional activation of different insular regions through direct electrical stimulation (E-stim) in humans. Read More

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Ictal and Interictal Cardiac Manifestations in Epilepsy. A Review of Their Relation With an Altered Central Control of Autonomic Functions and With the Risk of SUDEP.

Front Neurol 2021 12;12:642645. Epub 2021 Mar 12.

Lyon Neuroscience Research Center, INSERM U 1028, CNRS UMR, Lyon, France.

There is a complex interrelation between epilepsy and cardiac pathology, with both acute and long-term effects of seizures on the regulation of the cardiac rhythm and on the heart functioning. A specific issue is the potential relation between these cardiac manifestations and the risk of Sudden and Unexpected Death in Epilepsy (SUDEP), with unclear respective role of centrally-control ictal changes, long-term epilepsy-related dysregulation of the neurovegetative control and direct effects on the heart function. In the present review, we detailed available data about ictal cardiac changes, along with interictal cardiac manifestations associated with long-term functional and structural alterations of the heart. Read More

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Adrenergic Mechanisms of Audiogenic Seizure-Induced Death in a Mouse Model of Encephalopathy.

Front Neurosci 2021 4;15:581048. Epub 2021 Mar 4.

Department of Anesthesiology, University of Virginia Health System, Charlottesville, VA, United States.

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death amongst patients whose seizures are not adequately controlled by current therapies. Patients with encephalopathy have an elevated risk for SUDEP. While transgenic mouse models have provided insight into the molecular mechanisms of encephalopathy etiology, our understanding of seizure-induced death has been hampered by the inability to reliably trigger both seizures and seizure-induced death in these mice. Read More

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SUDEP - more attention to the heart? A narrative review on molecular autopsy in epilepsy.

Seizure 2021 Apr 15;87:103-106. Epub 2021 Mar 15.

Medical Sciences Post-graduate Program, Federal University of Santa Catarina, (UFSC), Florianópolis, SC, Brazil; Neurology Division, Federal University of Santa Catarina, (UFSC), Florianópolis, SC, Brazil; Center for Applied Neurosciences (CeNAp), Federal University of Santa Catarina, (UFSC), Florianópolis, SC, Brazil. Electronic address:

Sudden unexpected death in epilepsy (SUDEP) has been identified as one of the most prevalent causes of mortality in epilepsy, and SUDEP has consequently become an important topic of research. The causes appear multifactorial, including epilepsy-induced cardiac arrest. Current understanding of autopsy negative sudden unexplained death (SUD) in general population and its relation to sudden arrhythmic death syndrome (SADS) could shed some light in SUDEP. Read More

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Personalized EEG Feature Selection for Low-Complexity Seizure Monitoring.

Int J Neural Syst 2021 Mar 22:2150018. Epub 2021 Mar 22.

Department of Neurology and Neurotherapeutic, The University of Texas Southwestern Medical Center, Dallas 75230, USA.

Approximately, one third of patients with epilepsy are refractory to medical therapy and thus can be at high risk of injuries and sudden unexpected death. A low-complexity electroencephalography (EEG)-based seizure monitoring algorithm is critically important for daily use, especially for wearable monitoring platforms. This paper presents a personalized EEG feature selection approach, which is the key to achieve a reliable seizure monitoring with a low computational cost. Read More

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Risk of SUDEP during infancy.

Epilepsy Behav 2021 Mar 16:107896. Epub 2021 Mar 16.

Dipartimento di Scienze Biomediche e Neuromotorie, Università di Bologna, Bologna, Italy; IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.

Risk of sudden unexpected death in epilepsy (SUDEP) in children is influenced by different factors such as etiology, seizure type and frequency, treatment, and environment. A greater severity of epilepsy, in terms of seizure frequency, seizures type, especially with nocturnal generalized tonic-clonic seizures (GTCS), and resistance to anti-seizure medication are predisposing factors to SUDEP. Potential mechanisms of SUDEP might involve respiratory, cardiovascular, and central autonomic dysfunctions, either combined or in isolation. Read More

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A systematic review of adults with Dravet syndrome.

Seizure 2021 Apr 22;87:39-45. Epub 2021 Feb 22.

Institute of Medical Science, Faculty of Medicine, University of Toronto, Toronto, Canada; Adult Epilepsy Genetics Program, Division of Neurology, Krembil Research Institute, Toronto Western Hospital, Toronto, Canada; Division of Neurology, Department of Medicine, University of Toronto, ON, Canada; Krembil Neurosciences Institute, University Health Network, Toronto, ON, Canada. Electronic address:

Dravet Syndrome (DS) is a rare and severe infantile-onset epileptic encephalopathy. DS research focuses mainly on children. We did a systematic review, completed on January 18, 2021, examining the number of clinical DS studies. Read More

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Serotonergic therapy in epilepsy.

Curr Opin Neurol 2021 Apr;34(2):206-212

Department of Molecular and Cellular Biochemistry, University of Kentucky, Lexington, KY USA.

Purpose Of Review: The serotonergic system is implicated in multiple aspects of epilepsy, including seizure susceptibility, sudden unexpected death in epilepsy (SUDEP), and comorbid depression. Despite the complexity of serotonin's effects on various neuronal networks, ongoing research provides considerable insight into the role of serotonin in human epilepsy. This review explores the potential roles of serotonergic therapies to improve clinical outcomes in epilepsy. Read More

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Interictal autonomic dysfunction.

Curr Opin Neurol 2021 Apr;34(2):197-205

Department of Neuroscience, Central Clinical School, Monash University.

Purpose Of Review: Epilepsy is associated with autonomic dysfunction. Here, we provide an up-to-date review on measures of interictal autonomic function, focusing on heart rate variability (HRV), baroreflex sensitivity (BRS) and electrodermal activity (EDA).

Recent Findings: Resting HRV, BRS and EDA are altered in patients with epilepsy compared with healthy controls. Read More

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Seizure Clusters, Seizure Severity Markers, and SUDEP Risk.

Front Neurol 2021 12;12:643916. Epub 2021 Feb 12.

National Institute of Neurological Disorders and Stroke Center for Sudden Unexpected Death in Epilepsy Research (CSR), McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX, United States.

Seizure clusters may be related to Sudden Unexpected Death in Epilepsy (SUDEP). Two or more generalized convulsive seizures (GCS) were captured during video electroencephalography in 7/11 (64%) patients with monitored SUDEP in the MORTEMUS study. It follows that seizure clusters may be associated with epilepsy severity and possibly with SUDEP risk. Read More

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February 2021