1,118 results match your criteria Sudden Unexpected Death in Epilepsy


Seizure and Sudden Unexpected Death in Epilepsy (SUDEP) characteristics in an urban UK intellectual disability service.

Seizure 2020 May 11;80:18-23. Epub 2020 May 11.

University of Exeter Medical School, Exeter, United Kingdom; Cornwall Partnership NHS Foundation Trust, United Kingdom. Electronic address:

Purpose: This study identifies epilepsy-related characteristics and SUDEP risk factors in people with epilepsy (PWE) attending an urban community ID service in the UK where managing epilepsy is not part of the service remit, to understand the care provided to this vulnerable population.

Methods: An electronic database search in a north London community ID service (catchment population approx. 290,000) identified relevant ID/epilepsy characteristics in PWE to compare those with mild ID to moderate-profound ID. Read More

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http://dx.doi.org/10.1016/j.seizure.2020.05.004DOI Listing

A proposed guideline for vagus nerve stimulator handling in palliative care and after death.

Epilepsia 2020 May 28. Epub 2020 May 28.

Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University Health Centre, Montreal, QC, Canada.

Vagus nerve stimulation (VNS) is often used for patients with drug-resistant epilepsy. Although this intervention may improve seizure control and mood, a number of factors must be considered when patients with VNS near end of life. We reviewed relevant literature to create a proposed guideline for management of patients with VNS in palliative care and after death. Read More

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http://dx.doi.org/10.1111/epi.16553DOI Listing

Post-ictal accelerometer silence as a marker of post-ictal immobility.

Epilepsia 2020 May 27. Epub 2020 May 27.

Division of Neuroscience, Institute of Psychiatry, Psychology & Neuroscience, King's College London, London, UK.

Objective: Movement-based wearable sensors are used for detection of convulsive seizures. The identification of the absence of motion following a seizure, known as post-ictal immobility (PI), may represent a potential additional application of wearables. PI has been associated with potentially life-threatening complications and with sudden unexpected death in epilepsy (SUDEP). Read More

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http://dx.doi.org/10.1111/epi.16552DOI Listing

The role of sodium channels in sudden unexpected death in pediatrics.

Mol Genet Genomic Med 2020 May 25:e1309. Epub 2020 May 25.

Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.

Background: Sudden Unexpected Death in Pediatrics (SUDP) is a tragic event, likely caused by the complex interaction of multiple factors. The presence of hippocampal abnormalities in many children with SUDP suggests that epilepsy-related mechanisms may contribute to death, similar to Sudden Unexplained Death in Epilepsy. Because of known associations between the genes SCN1A and SCN5A and sudden death, and shared mechanisms and patterns of expression in genes encoding many voltage-gated sodium channels (VGSCs), we hypothesized that individuals dying from SUDP have pathogenic variants across the entire family of cardiac arrhythmia- and epilepsy-associated VGSC genes. Read More

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http://dx.doi.org/10.1002/mgg3.1309DOI Listing

Periictal electroclinical characteristics of postictal generalized electroencephalographic suppression after generalized convulsive seizures.

Medicine (Baltimore) 2020 May;99(20):e19940

Department of Neurology, West China Hospital of Sichuan University.

The aim of this study was to investigate the demographic, clinical, and electrophysiological characteristics of postictal generalized electroencephalography (EEG) suppression (PGES), thereby facilitating the recognition of PGES and providing clues regarding its risk factors, pathophysiology, and relationship with sudden unexpected death in epilepsy patients (SUDEP).We retrospectively reviewed 237 generalized convulsive seizures (GCSs) in 126 patients during long-term video-EEG (VEEG) recordings. The associations of PGES and prolonged PGES (duration >20 seconds) with person- and seizure-specific variables were evaluated independently using SPSS software. Read More

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http://dx.doi.org/10.1097/MD.0000000000019940DOI Listing

Peri-ictal heart rate variability parameters as surrogate markers of seizure severity.

Epilepsia 2020 May 21. Epub 2020 May 21.

Department of Clinical Neurophysiology, Danish Epilepsy Centre, Dianalund, Denmark.

This study aims at defining objective parameters reflecting the severity of peri-ictal autonomic changes and their relation to post-ictal generalized electroencephalography (EEG) suppression (PGES), with the view that such changes could be detected by wearable seizure detection systems and prove useful to assess the risk of sudden unexpected death in epilepsy (SUDEP). To this purpose, we assessed peri-ictal changes in heart rate variability (HRV) and correlated them with seizure duration, intensity of electromyography-based ictal muscle activity, and presence and duration of post-ictal generalized EEG suppression (PGES). We evaluated 75 motor seizures from 40 patients, including 61 generalized tonic-clonic seizures (GTCS) and 14 other major motor seizure types. Read More

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http://dx.doi.org/10.1111/epi.16491DOI Listing

Genetic/idiopathic generalized epilepsies: Not so good as that!

Rev Neurol (Paris) 2020 May 15. Epub 2020 May 15.

Epilepsy Unit, Hôpital Gui-de-Chauliac, 80, avenue Fliche, 34295 Montpellier cedex 05, France; Research Unit (URCMA : Unité de Recherche sur les Comportements et Mouvements Anormaux), Inserm, U661, 34000 Montpellier, France.

Genetic or idiopathic generalized epilepsies (IGEs) account for 15-20% of all epilepsies. These syndromes have always been considered as good prognosis forms of epilepsy over time; however, for some patients, there is a need to maintain antiseizure drugs (ASD) for a long-time. Drug resistance is not uncommon (7-15%). Read More

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http://dx.doi.org/10.1016/j.neurol.2020.03.018DOI Listing

All-cause mortality and SUDEP in a surgical epilepsy population.

Epilepsy Behav 2020 May 10;108:107093. Epub 2020 May 10.

Department of Neurology, Columbia University Medical Center, New York, NY, United States of America. Electronic address:

Epilepsy surgery is considered to reduce the risk of epilepsy-related mortality, including sudden unexpected death in epilepsy (SUDEP), though data from existing surgical series are conflicting. We retrospectively examined all-cause mortality and SUDEP in a population of 590 epilepsy surgery patients and a comparison group of 122 patients with pharmacoresistant focal epilepsy who did not undergo surgery, treated at Columbia University Medical Center between 1977 and 2014. There were 34 deaths in the surgery group, including 14 cases of SUDEP. Read More

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http://dx.doi.org/10.1016/j.yebeh.2020.107093DOI Listing

Alcohol and sudden unexpected death in epilepsy: do not pop the cork.

Clinics (Sao Paulo) 2020 8;75:e1770. Epub 2020 May 8.

Disciplina de Neurociencia, Escola Paulista de Medicina, Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo, SP, BR.

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http://dx.doi.org/10.6061/clinics/2020/e1770DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7196729PMC

Ictal quantitative surface electromyography correlates with postictal EEG suppression.

Neurology 2020 May 12. Epub 2020 May 12.

From the Department of Clinical Neurophysiology (A.A.A., P.W., S.B.), Danish Epilepsy Centre, Dianalund, Denmark; Department of Clinical Neurosciences (A.A.A.), "Carol Davila" University of Medicine and Pharmacy, Bucharest, Romania; FORCE Technology (I.C.), Hørsholm, Denmark; Brain Sentinel (D.P.C., L.E.W., S.R.V.), San Antonio, TX; Department of Clinical Medicine (P.W.), Neurological Service, Federal University of Santa Catarina, Florianópolis, SC, Brazil; Center for SUDEP Research (S.L.), National Institute of Neurological Disorders and Stroke, Bethesda, MD; Department of Neurology (S.L.), University of Texas Health Sciences Center at Houston; Department of Clinical Neurosciences (P.R.), CHUV, Lausanne, Switzerland; Department of Clinical Neurophysiology (S.B.), Aarhus University Hospital; and Department of Clinical Medicine (S.B.), Aarhus University, Denmark.

Objective: To test the hypothesis that neurophysiologic biomarkers of muscle activation during convulsive seizures reveal seizure severity and to determine whether automatically computed surface EMG parameters during seizures can predict postictal generalized EEG suppression (PGES), indicating increased risk for sudden unexpected death in epilepsy. Wearable EMG devices have been clinically validated for automated detection of generalized tonic-clonic seizures. Our goal was to use quantitative EMG measurements for seizure characterization and risk assessment. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009492DOI Listing

Seizure-related apneas have an inconsistent linkage to amygdala seizure spread.

Epilepsia 2020 May 11. Epub 2020 May 11.

Department of Neurology, University of California, Davis, California.

Objective: Sudden unexpected death in epilepsy (SUDEP) is a frequent cause of death in epilepsy. Respiratory dysfunction is implicated as a critical factor in SUDEP pathophysiology. Human studies have shown that electrical stimulation of the amygdala resulted in apnea, indicating that the amygdala has a role in respiration control. Read More

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http://dx.doi.org/10.1111/epi.16518DOI Listing

Sudden death in epilepsy and ectopic neurohypophysis in Joubert syndrome 23 diagnosed using SNVs/indels and structural variants pipelines on WGS data: a case report.

BMC Med Genet 2020 May 7;21(1):96. Epub 2020 May 7.

Department of Medical Genetics, Oslo University Hospital and University of Oslo, Oslo, Norway.

Background: Joubert syndrome (JBTS) is a genetically heterogeneous group of neurodevelopmental syndromes caused by primary cilia dysfunction. Usually the neurological presentation starts with abnormal neonatal breathing followed by muscular hypotonia, psychomotor delay, and cerebellar ataxia. Cerebral MRI shows mid- and hindbrain anomalies including the molar tooth sign. Read More

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http://dx.doi.org/10.1186/s12881-020-01024-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7204034PMC

Automated video-based detection of nocturnal motor seizures in children.

Epilepsia 2020 May 7. Epub 2020 May 7.

Stichting Epilepsie Instellingen Nederland, Heemstede, the Netherlands.

Seizure detection devices can improve epilepsy care, but wearables are not always tolerated. We previously demonstrated good performance of a real-time video-based algorithm for detection of nocturnal convulsive seizures in adults with learning disabilities. The algorithm calculates the relative frequency content based on the group velocity reconstruction from video-sequence optical flow. Read More

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http://dx.doi.org/10.1111/epi.16504DOI Listing

Wearable devices for seizure detection: Is it time to translate into our clinical practice?

Authors:
S Rheims

Rev Neurol (Paris) 2020 Apr 28. Epub 2020 Apr 28.

Department of functional neurology and epileptology, hospices civils de Lyon, university of Lyon, Lyon, France; Inserm U1028/CNRS UMR 5292, Lyon's neuroscience research center, Lyon, France; Epilepsy institute, Lyon, France. Electronic address:

With the exponential development of mobile health technologies over the past ten years, there has been a growing interest in the potential applications in the field of epilepsy, and specifically for seizure detection. Better detection of seizures is probably one of the best ways to improve patient safety. Overall, we are observing an exponential increase in the number of non-EEG based seizure detection systems and a progressive homogenization of their evaluation procedures. Read More

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http://dx.doi.org/10.1016/j.neurol.2019.12.012DOI Listing

Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches.

Int J Mol Sci 2020 Apr 22;21(8). Epub 2020 Apr 22.

Department of Pharmacy-Drug Sciences, University of Bari "Aldo Moro", 70125 Bari, Italy.

Kv1.1 belongs to the subfamily of voltage-gated potassium channels and acts as a critical regulator of neuronal excitability in the central and peripheral nervous systems. is the only gene that has been associated with episodic ataxia type 1 (EA1), an autosomal dominant disorder characterized by ataxia and myokymia and for which different and variable phenotypes have now been reported. Read More

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http://dx.doi.org/10.3390/ijms21082935DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7215777PMC

Socioeconomic disparities in SUDEP in the US.

Neurology 2020 Apr 23. Epub 2020 Apr 23.

From the Department of Neurology (E.C., M.K., O.D., D.F.), NYU School of Medicine; Department of Epidemiology (D.C.H.), Columbia University Medical Center, New York, NY; San Diego County Medical Examiner's Office (M.B.), CA; Maryland Office of the Chief Medical Examiner (L.L., D.R.F.), Baltimore; New York City Office of Chief Medical Examiner (J.K.G.), NY; and Department of Paediatrics (E.J.D.), Division of Neurology, The Hospital for Sick Children, Toronto, Ontario, Canada.

Objective: To determine the impact of socioeconomic status (SES) on sudden unexpected death in epilepsy (SUDEP) rates.

Methods: We queried all decedents presented for medico-legal investigation at 3 medical examiner (ME) offices across the country (New York City, Maryland, San Diego County) in 2009 to 2010 and 2014 to 2015. We identified all decedents for whom epilepsy/seizure was listed as cause/contributor to death or comorbid condition on the death certificate. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009463DOI Listing

Generalized tonic-clonic seizures with post-ictal atrial fibrillation.

Epilepsy Behav Rep 2020 30;13:100343. Epub 2019 Oct 30.

Department of Neurology, University of Louisville, 500 South Jackson Street, Louisville, KY 40202, USA.

Convulsive seizures are known to cause severe cardiopulmonary changes and increased autonomic activity. Limited reports describe peri-ictal cardiac arrhythmias such as atrial fibrillation (AF) with generalized tonic-clonic seizures (GTCS). We present a unique case of a healthy 23-year-old male patient with new onset prolonged AF in the setting of new onset seizures, occurring on three independent occasions. Read More

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http://dx.doi.org/10.1016/j.ebr.2019.100343DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7170332PMC
October 2019

Prevention of sudden unexpected death in epilepsy: current status and future perspectives.

Expert Rev Neurother 2020 May 26;20(5):497-508. Epub 2020 Apr 26.

Department of Epileptology, University Hospital Bonn, Bonn, Germany.

: Sudden unexpected death in epilepsy (SUDEP) affects about 1 in 1000 people with epilepsy, and even more in medically refractory epilepsy. As most people are between 20 and 40 years when dying suddenly, SUDEP leads to a considerable loss of potential life years. The most important risk factors are nocturnal and tonic-clonic seizures, underscoring that supervision and effective seizure control are key elements for SUDEP prevention. Read More

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http://dx.doi.org/10.1080/14737175.2020.1754195DOI Listing

Adenosine kinase and adenosine receptors A R and A R in temporal lobe epilepsy and hippocampal sclerosis and association with risk factors for SUDEP.

Epilepsia 2020 Apr 3;61(4):787-797. Epub 2020 Apr 3.

Langone Comprehensive Epilepsy Center, New York University, New York, New York.

Objective: The "adenosine hypothesis of SUDEP" (sudden unexpected death in epilepsy) predicts that a seizure-induced adenosine surge combined with impaired metabolic clearance can foster lethal apnea or cardiac arrest. Changes in adenosine receptor density and adenosine kinase (ADK) occur in surgical epilepsy patients. Our aim was to correlate the distribution of ADK and adenosine A and A receptors (A R and A R) in surgical tissue from patients with temporal lobe epilepsy and hippocampal sclerosis (TLE/HS) with SUDEP risk factors. Read More

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http://dx.doi.org/10.1111/epi.16487DOI Listing

Treatments for the prevention of Sudden Unexpected Death in Epilepsy (SUDEP).

Cochrane Database Syst Rev 2020 04 2;4:CD011792. Epub 2020 Apr 2.

University of Liverpool, Department of Biostatistics, Block F, Waterhouse Building, 1-5 Brownlow Hill, Liverpool, UK, L69 3GL.

Background: This is an updated version of the original Cochrane Review, published in 2016, Issue 7. Sudden Unexpected Death in Epilepsy (SUDEP) is defined as sudden, unexpected, witnessed or unwitnessed, non-traumatic or non-drowning death of people with epilepsy, with or without evidence of a seizure, excluding documented status epilepticus and in whom postmortem examination does not reveal a structural or toxicological cause for death. SUDEP has a reported incidence of 1 to 2 per 1000 patient-years and represents the most common epilepsy-related cause of death. Read More

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http://dx.doi.org/10.1002/14651858.CD011792.pub3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7115126PMC

Genetic generalized and focal epilepsy prevalence in the North American SUDEP Registry.

Neurology 2020 Apr 26;94(16):e1757-e1763. Epub 2020 Mar 26.

From the Comprehensive Epilepsy Center (C.V., D.F., O.D.), New York University School of Medicine, New York; and Division of Neurology (E.D.), The Hospital for Sick Children, University of Toronto, Canada.

Objective: To assess relative rates and clinical features of patients with genetic generalized epilepsy (GGE), focal epilepsy (FE), and developmental encephalopathic epilepsy (DEE) in the North American SUDEP Registry (NASR).

Methods: We identified all adjudicated definite, definite plus, and probable sudden unexpected death in epilepsy (SUDEP) cases (n = 262) and determined epilepsy type (GGE, FE, or DEE) from medical record review including history, imaging and EEG results, genetics, and next-of-kin interviews.

Results: Of the 262 SUDEP cases, 41 occurred in GGE, 95 in FE, 24 in DEE, and 102 were unclassifiable. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009295DOI Listing

Cardiac arrhythmias in Dravet syndrome: an observational multicenter study.

Ann Clin Transl Neurol 2020 Apr 24;7(4):462-473. Epub 2020 Mar 24.

Stichting Epilepsie Instellingen Nederland - SEIN, Achterweg 5, 2103 SW Heemstede, Dokter Denekampweg 20, 8025 BV, Zwolle, The Netherlands.

Objectives: We ascertained the prevalence of ictal arrhythmias to explain the high rate of sudden unexpected death in epilepsy (SUDEP) in Dravet syndrome (DS).

Methods: We selected cases with clinical DS, ≥6 years, SCN1A mutation, and ≥1 seizure/week. Home-based ECG recordings were performed for 20 days continuously. Read More

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http://dx.doi.org/10.1002/acn3.51017DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7187713PMC

Sudden death in a patient with long QT syndrome presenting with an epileptic phenotype.

Ann Noninvasive Electrocardiol 2020 Mar 21:e12753. Epub 2020 Mar 21.

Department of Electrophysiology, Rhode Island Hospital, Brown University, Providence, RI, USA.

Patients with epilepsy suffer from a higher mortality rate than the general population, a portion of which is not due to epilepsy itself or comorbid conditions. Sudden unexpected death in epilepsy (SUDEP) is a common but poorly understood cause of death in patients with intractable epilepsy and often afflicts younger patients. The pathophysiology of SUDEP is poorly defined but does not appear to be related to prolonged seizure activity or resultant injury. Read More

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http://dx.doi.org/10.1111/anec.12753DOI Listing

KCNT1-related epilepsy: An international multicenter cohort of 27 pediatric cases.

Epilepsia 2020 Apr 13;61(4):679-692. Epub 2020 Mar 13.

Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.

Objective: Through international collaboration, we evaluated the phenotypic aspects of a multiethnic cohort of KCNT1-related epilepsy and explored genotype-phenotype correlations associated with frequently encountered variants.

Methods: A cross-sectional analysis of children harboring pathogenic or likely pathogenic KCNT1 variants was completed. Children with one of the two more common recurrent KCNT1 variants were compared with the rest of the cohort for the presence of particular characteristics. Read More

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http://dx.doi.org/10.1111/epi.16480DOI Listing
April 2020
4.571 Impact Factor

Dravet Syndrome: A Review of Current Management.

Pediatr Neurol 2020 Jun 31;107:28-40. Epub 2020 Jan 31.

Division of Pediatric Neurology, Department of Pediatrics & Neurology, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, Tennessee.

Dravet syndrome is a debilitating epileptic encephalopathy of childhood with few treatment options available in the United States before 2018. In the modern era, new genetic testing options will allow diagnosis closer to disease onset. Three new medicines-stiripentol, cannabidiol, and fenfluramine-have documented efficacy and safety as adjunctive therapies for treating pharmacoresistant Dravet syndrome. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2020.01.005DOI Listing

A human amygdala site that inhibits respiration and elicits apnea in pediatric epilepsy.

JCI Insight 2020 Mar 26;5(6). Epub 2020 Mar 26.

Department of Neurosurgery.

BACKGROUNDSeizure-induced inhibition of respiration plays a critical role in sudden unexpected death in epilepsy (SUDEP). However, the mechanisms underlying seizure-induced central apnea in pediatric epilepsy are unknown.METHODSWe studied 8 pediatric patients with intractable epilepsy undergoing intracranial electroencephalography. Read More

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http://dx.doi.org/10.1172/jci.insight.134852DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213805PMC

Incorporating Ethically Relevant Empirical Data From Systematic Review of Reasons: A Case Study of .

AJOB Empir Bioeth 2020 Apr-Jun;11(2):91-103. Epub 2020 Mar 11.

Harvard Medical School, Boston, MA, USA.

In this report we use a case study of risk of (SUDEP) to illustrate the contribution of systematic literature reviews of disease-specific ethical issues (DSEI). In particular, we show how ethically-relevant empirical data from such reviews can be used in the examination of the reasons for and against a particular normative approach to our DSEI. That is, we have attempted to offer a normative recommendation in response to the question of whether or not the risk of SUDEP should be disclosed to all patients. Read More

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http://dx.doi.org/10.1080/23294515.2020.1737981DOI Listing

Mortality in infantile spasms: A hospital-based study.

Epilepsia 2020 Apr 5;61(4):702-713. Epub 2020 Mar 5.

Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, Massachusetts.

Objective: To determine risk factors and causes for mortality during childhood in patients with infantile spasms (IS). We describe the overall goals of care for those who died.

Methods: This is a retrospective chart review of IS patients born between 2000 and 2011. Read More

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http://dx.doi.org/10.1111/epi.16468DOI Listing

Detection of Postictal Generalized Electroencephalogram Suppression: Random Forest Approach.

JMIR Med Inform 2020 Feb 14;8(2):e17061. Epub 2020 Feb 14.

School of Biomedical Informatics, University of Texas Health Science Center, Houston, TX, United States.

Background: Sudden unexpected death in epilepsy (SUDEP) is second only to stroke in neurological events resulting in years of potential life lost. Postictal generalized electroencephalogram (EEG) suppression (PGES) is a period of suppressed brain activity often occurring after generalized tonic-clonic seizure, a most significant risk factor for SUDEP. Therefore, PGES has been considered as a potential biomarker for SUDEP risk. Read More

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http://dx.doi.org/10.2196/17061DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7055778PMC
February 2020

Assessment of cardiac structure and function in a murine model of temporal lobe epilepsy.

Epilepsy Res 2020 Mar 24;161:106300. Epub 2020 Feb 24.

Dep. of Neurology, Oslo University Hospital, Rikshospitalet, Oslo, Norway. Electronic address:

Sudden unexpected death in epilepsy (SUDEP) is a significant cause of premature seizure-related death. An association between SUDEP and cardiac remodeling has been suggested. However, whether SUDEP is a direct consequence of acute or recurrent seizures is unsettled. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2020.106300DOI Listing

Epilepsy in older people.

Lancet 2020 02;395(10225):735-748

National Institute for Health Research, Biomedical Research Centre, University College London Hospitals, UCL Queen Square Institute of Neurology, London, UK; Chalfont Centre for Epilepsy, Chalfont St Peter, UK; Stichting Epilepsie Instellingen Nederland (SEIN), Heemstede, Netherlands.

Globally, as populations age there will be challenges and opportunities to deliver optimal health care to senior citizens. Epilepsy, a condition characterised by spontaneous recurrent seizures, is common in older adults (aged >65 years) and yet has received comparatively little attention in this age group. In this Review, we evaluate the underlying causes of epilepsy in older people, explore difficulties in establishing a diagnosis of epilepsy in this population, discuss appropriate antiseizure medications, and evaluate potential surgical treatment options. Read More

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http://dx.doi.org/10.1016/S0140-6736(19)33064-8DOI Listing
February 2020
45.217 Impact Factor

The Epileptic Heart: Concept and clinical evidence.

Epilepsy Behav 2020 Apr 25;105:106946. Epub 2020 Feb 25.

Harvard Medical School, Beth Israel Deaconess Medical Center, Division of Cardiovascular Medicine and Department of Neurology, Boston, MA United States of America.

Sudden unexpected death in epilepsy (SUDEP) is generally considered to result from a seizure, typically convulsive and usually but not always occurring during sleep, followed by a sequence of events in the postictal period starting with respiratory distress and progressing to eventual cardiac asystole and death. Yet, recent community-based studies indicate a 3-fold greater incidence of sudden cardiac death in patients with chronic epilepsy than in the general population, and that in 66% of cases, the cardiac arrest occurred during routine daily activity and without a temporal relationship with a typical seizure. To distinguish a primarily cardiac cause of death in patients with epilepsy from the above description of SUDEP, we propose the concept of the "Epileptic Heart" as "a heart and coronary vasculature damaged by chronic epilepsy as a result of repeated surges in catecholamines and hypoxemia leading to electrical and mechanical dysfunction. Read More

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http://dx.doi.org/10.1016/j.yebeh.2020.106946DOI Listing

Prone, lateral, or supine positioning at seizure onset determines the postictal body position: A multicenter video-EEG monitoring cohort study.

Seizure 2020 Feb 21;76:173-178. Epub 2020 Feb 21.

Epilepsy Center Frankfurt Rhine-Main, Department of Neurology, Goethe-University Frankfurt, Frankfurt am Main, Germany; LOEWE Center for Personalized and Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany; Epilepsy Center Hessen and Department of Neurology, Philipps-University Marburg, Marburg (Lahn), Germany. Electronic address:

Purpose: Most patients who die from sudden unexpected death in epilepsy (SUDEP) are found in the prone position. We evaluated whether changes in body position occur during generalized convulsive seizures (GCSs).

Method: GCSs in patients undergoing video-EEG-monitoring between 2007 and 2017 at epilepsy centers in Frankfurt and Marburg were analyzed in relation to changes in body position. Read More

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http://dx.doi.org/10.1016/j.seizure.2020.02.008DOI Listing
February 2020

Review on post-mortem diagnosis in suspected SUDEP: Currently still a difficult task for Forensic Pathologists.

J Forensic Leg Med 2020 Feb 5;70:101920. Epub 2020 Feb 5.

Department of Legal and Forensic Medicine, University of Genova, Via De' Toni 12, 16132, Genova, Italy. Electronic address:

Sudden and unexpected death in epilepsy (SUDEP) represents the predominant cause of premature deaths in young adults with epilepsy and is more common with patients with poorly controlled and generalized convulsive seizures. It is reported that there are 1,16 cases for every 1000 subjects affected with epilepsy. This review takes stock of the current problems and issues in the autopsy of cases of sudden death with epileptic people. Read More

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http://dx.doi.org/10.1016/j.jflm.2020.101920DOI Listing
February 2020

Changing Landscape of Dravet Syndrome Management: An Overview.

Authors:
Debopam Samanta

Neuropediatrics 2020 04 20;51(2):135-145. Epub 2020 Feb 20.

Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States.

Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy, is a severe developmental and epileptic encephalopathy caused by loss-of-function mutations in one copy of (haploinsufficiency), located on chromosome 2q24, with decreased function of Nav1.1 sodium channels in GABAergic inhibitory interneurons. Pharmacoresistant seizures in DS start in the infancy in the form of hemiclonic febrile status epilepticus. Read More

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http://dx.doi.org/10.1055/s-0040-1701694DOI Listing

Metabolism-based drug discovery in zebrafish: An emerging strategy to uncover new anti-seizure therapies.

Neuropharmacology 2020 May 4;167:107988. Epub 2020 Feb 4.

Department of Medical Genetics, Cumming School of Medicine, University of Calgary, Canada; Alberta Children's Hospital Research Institute, University of Calgary, Canada. Electronic address:

As one of the most common neurological disorders, epilepsy can occur throughout the lifespan and from a multiplicity of causes, including genetic mutations, inflammation, neurotrauma, or brain malformations. Although pharmacological agents are the mainstay of treatment for seizure control, an unyielding 30-40% of patients remain refractory to these medications and continue to experience spontaneous recurrent seizures with attendant life-long cognitive, behavioural, and mental health issues, as well as an increased risk for sudden unexpected death. Despite over eight decades of antiseizure drug (ASD) discovery and the approval of dozens of new medications, the percentage of this refractory population remains virtually unchanged, suggesting that drugs with new and unexpected mechanisms of action are needed. Read More

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http://dx.doi.org/10.1016/j.neuropharm.2020.107988DOI Listing

Progressive cardiorespiratory dysfunction in Kv1.1 knockout mice may provide temporal biomarkers of pending sudden unexpected death in epilepsy (SUDEP): The contribution of orexin.

Epilepsia 2020 Mar 6;61(3):572-588. Epub 2020 Feb 6.

Department of Pharmacology, Creighton University School of Medicine, Omaha, NE, USA.

Objective: Immediately preceding sudden unexpected death in epilepsy (SUDEP), patients experienced a final generalized tonic-clonic seizure (GTCS), rapid ventilation, apnea, bradycardia, terminal apnea, and asystole. Whether a progressive pathophysiology develops and increases risk of SUDEP remains unknown. Here, we determined (a) heart rate, respiratory rate, and blood oxygen saturation (SaO ) in low-risk and high-risk knockout (KO) mice; and (b) whether blocking receptors for orexin, a cardiorespiratory neuromodulator, influences cardiorespiratory function mice or longevity in high-risk KO mice. Read More

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http://dx.doi.org/10.1111/epi.16434DOI Listing

SUDEP among young adults in the San Diego County Medical Examiner Office.

Epilepsia 2020 Mar 6;61(3):e17-e22. Epub 2020 Feb 6.

Department of Neurology, Comprehensive Epilepsy Center, NYU Langone Health, New York, NY, USA.

Excess mortality due to epilepsy is greatest among young adults. However, the relative proportions of sudden unexpected death in epilepsy (SUDEP) and other epilepsy-related causes of death are not well defined. We prospectively adjudicated cause of death in all 18- to 45-year-olds with a history of seizure/epilepsy who underwent medicolegal investigation in San Diego County between 2014 and 2017. Read More

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http://dx.doi.org/10.1111/epi.16443DOI Listing

Audiogenic seizure as a model of sudden death in epilepsy: A comparative study between four inbred mouse strains from early life to adulthood.

Epilepsia 2020 Feb 24;61(2):342-349. Epub 2020 Jan 24.

Univ Rennes, CHU Rennes, Inserm, LTSI (Laboratoire de Traitement du Signal et de l'Image), UMR-1099, F-35000, Rennes, France.

Objective: Mouse models of sudden unexpected death in epileptic patients (SUDEP) using audiogenic seizures (AGS) are valuable because death can occur following a sound-induced seizure in the absence of any pharmacologic or electric component. However, only a few strains of mice are AGS prone, and the vast majority of studies involve DBA/2 or DBA/1 inbred strains. With the goal of characterizing the variation of AGS susceptibility with age, and of offering a larger panel of mice available for AGS studies, we performed a comparative study of the variability in AGS responses. Read More

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http://dx.doi.org/10.1111/epi.16432DOI Listing
February 2020

Neuron-specific Kv1.1 deficiency is sufficient to cause epilepsy, premature death, and cardiorespiratory dysregulation.

Neurobiol Dis 2020 04 21;137:104759. Epub 2020 Jan 21.

Department of Cellular Biology and Anatomy, Louisiana State University Health Sciences Center Shreveport, LA 71103, United States of America; Department of Biological Sciences, Southern Methodist University, Dallas, TX 75275, United States of America. Electronic address:

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of epilepsy-related mortality, but the precise cellular substrates involved remain elusive. Epilepsy-associated ion channel genes with co-expression in brain and heart have been proposed as SUDEP candidate genes since they provide a singular unifying link between seizures and lethal cardiac arrhythmias. Here, we generated a conditional knockout (cKO) mouse with neuron-specific deletion of Kcna1, a SUDEP-associated gene with brain-heart co-expression, to test whether seizure-evoked cardiac arrhythmias and SUDEP require the absence of Kv1. Read More

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http://dx.doi.org/10.1016/j.nbd.2020.104759DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7050436PMC
April 2020
5.078 Impact Factor

Changes in autonomic nervous function and influencing factors in a rat insular cortex electrical kindling model.

Neurosci Lett 2020 Mar 21;721:134782. Epub 2020 Jan 21.

Department of Neurology and Neuroscience Center, First Hospital of Jilin University, Changchun 130021, China. Electronic address:

In mammals, the insular cortex plays an important role in autonomic regulation. In patients with insular epilepsy, seizures are always accompanied by autonomic changes. Accordingly, we aimed to establish an electrical kindling model in autonomic-mediating areas of the insular cortex, and to conduct a long-term observation of epileptic genesis in these animals until sudden unexpected death. Read More

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http://dx.doi.org/10.1016/j.neulet.2020.134782DOI Listing

Epilepsy Benchmarks Area IV: Limit or Prevent Adverse Consequence of Seizures and Their Treatment Across the Life Span.

Epilepsy Curr 2020 Jan-Feb;20(1_suppl):31S-39S. Epub 2020 Jan 23.

Epilepsy Genetics Program, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Epilepsy represents a complex spectrum disorder, with patients sharing seizures as a common symptom and manifesting a broad array of additional clinical phenotypes. To understand this disorder and treat individuals who live with epilepsy, it is important not only to identify pathogenic mechanisms underlying epilepsy but also to understand their relationships with other health-related factors. Benchmarks Area IV focuses on the impact of seizures and their treatment on quality of life, development, cognitive function, and other aspects and comorbidities that often affect individuals with epilepsy. Read More

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http://dx.doi.org/10.1177/1535759719895277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7031803PMC
January 2020

Neuropeptide depletion in the amygdala in sudden unexpected death in epilepsy: A postmortem study.

Epilepsia 2020 Feb 20;61(2):310-318. Epub 2020 Jan 20.

Department of Clinical and Experimental Epilepsy, University College London Queen Square Institute of Neurology, London, UK.

Objective: Sudden unexpected death in epilepsy (SUDEP) is typically unwitnessed but can be preceded by seizures in the period prior to death. Peri-ictal respiratory dysfunction is a likely mechanism for some SUDEP, and central apnea has been shown following amygdala stimulation. The amygdala is enriched in neuropeptides that modulate neuronal activity and can be transiently depleted following seizures. Read More

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http://dx.doi.org/10.1111/epi.16425DOI Listing
February 2020

Sudden unexpected death in epilepsy (SUDEP) in New Zealand; a retrospective review.

N Z Med J 2020 01 17;133(1508):65-71. Epub 2020 Jan 17.

Neurologist, Neurology Department, Auckland City Hospital, Auckland.

Aim: Sudden unexpected death in epilepsy (SUDEP) is well recognised and widely reported but remains poorly understood. SUDEP in young adults is 27 times more common than sudden death in control populations. The incidence of SUDEP in New Zealand is not known but up to 40 people with epilepsy may die from SUDEP every year. Read More

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January 2020

Phenotypic and genetic spectrum of SCN8A-related disorders, treatment options, and outcomes.

Epilepsia 2019 12;60 Suppl 3:S77-S85

Danish Epilepsy Center, Dianalund, Denmark.

Pathogenic variants in SCN8A have originally been described in patients with developmental and epileptic encephalopathy (DEE). However, recent studies have shown that SCN8A variants can be associated with a broader phenotypic spectrum, including the following: (1) Patients with early onset, severe DEE, developing severe cognitive and motor regression, pyramidal/extrapyramidal signs, and cortical blindness. Severe SCN8A-DEE is characterized by intractable seizures beginning in the first months of life. Read More

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http://dx.doi.org/10.1111/epi.16319DOI Listing
December 2019

Excitation and Inhibition Balance Underlying Epileptiform Activity.

IEEE Trans Biomed Eng 2020 Jan 1. Epub 2020 Jan 1.

Objective: The phenomenon of postictal generalized EEG suppression state (PGES) - a period with suppressed activity following seizure termination and has been found to be associated with sudden unexpected death in epilepsy - remains poorly understood. This work aims to examine the how the balance of excitation and inhibition (E/I balance) affect the dynamics of seizure and PGES.

Methods: A network of 1000 Izhikevich model neurons was developed and only the strengths of synaptic connections were adjusted to recreate the dynamics ob- served in recordings of seizure and PGES from human patients. Read More

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http://dx.doi.org/10.1109/TBME.2019.2963430DOI Listing
January 2020

Editorial: Sudden Unexpected Death in Epilepsy: Bio-markers, Mechanisms, Risk Identification and Prevention.

Front Neurol 2019 4;10:1277. Epub 2019 Dec 4.

Department of Neurology, David Geffen-UCLA School of Medicine, Sylmar, CA, United States.

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http://dx.doi.org/10.3389/fneur.2019.01277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6904271PMC
December 2019

Serotonin and sudden unexpected death in epilepsy.

Exp Neurol 2020 Mar 19;325:113145. Epub 2019 Dec 19.

Interdisciplinary Graduate Program in Neuroscience, University of Iowa, Iowa City, IA 52242, United States of America; Department of Neurology, University of Iowa, Iowa City, IA 52242, United States of America; Iowa Neuroscience Institute, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, United States of America. Electronic address:

Epilepsy is a highly prevalent disease characterized by recurrent, spontaneous seizures. Approximately one-third of epilepsy patients will not achieve seizure freedom with medical management and become refractory to conventional treatments. These patients are at greatest risk for sudden unexpected death in epilepsy (SUDEP). Read More

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http://dx.doi.org/10.1016/j.expneurol.2019.113145DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7029792PMC