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Nicole A Hawkins, Nicole J Zachwieja, Alison R Miller, Lyndsey L Anderson, Jennifer A Kearney
A substantial number of mutations have been identified in voltage-gated sodium channel genes that result in various forms of human epilepsy. SCN1A mutations result in a spectrum of severity ranging from mild febrile seizures to Dravet syndrome, an infant-onset epileptic encephalopathy. Dravet syndrome patients experience multiple seizures types that are often refractory to treatment, developmental delays, and elevated risk for SUDEP. The same sodium channel mutation can produce epilepsy phenotypes of varying clinical severity...
October 2016: PLoS Genetics
Roisin Bartlam, Rajiv Mohanraj
BACKGROUND: Seizures can lead to cardiac arrhythmias by a number of mechanisms including activation/inhibition of cortical autonomic centers, increase in vagal tone through activation of brainstem reflex centers, and respiratory failure. Ictal asystole (IA) is a potential mechanism underlying sudden unexpected death in epilepsy (SUDEP). We analyzed the clinical features of 5 patients who developed IA requiring pacemaker implantation. METHODS: Patients with ictal arrhythmias were identified from the video-telemetry and ambulatory EEG database at Greater Manchester Neurosciences Centre, as well as an independent epilepsy residential care facility...
October 13, 2016: Epilepsy & Behavior: E&B
Rajesh RamachandranNair, Susan M Jack
PURPOSE: The purpose of this descriptive qualitative study was to understand the range of adult patients' views on whether and how to discuss the issue of SUDEP with patients with epilepsy and to clarify the optimal timing and formulation of this information. METHOD: The principles of fundamental qualitative description informed all design decisions. Twenty-three patients aged 18-65years participated in the study. Nineteen participants completed a single one-on-one telephone interview, whereas four participants shared their experiences in a focus group...
October 12, 2016: Epilepsy & Behavior: E&B
Carl L Faingold, Marcus Randall, Chang Zeng, Shifang Peng, Xiaoyan Long, Hua-Jun Feng
Drugs that enhance the action of serotonin (5-hydroxytrypamine, 5-HT), including several selective serotonin reuptake inhibitors (SSRIs), reduce susceptibility to seizure-induced respiratory arrest (S-IRA) that leads to death in the DBA/1 mouse model of sudden unexpected death in epilepsy (SUDEP). However, it is not clear if specific 5-HT receptors are important in the action of these drugs and whether the brain is the major site of action of these agents in this SUDEP model. The current study examined the actions of agents that affect the 5-HT3 receptor subtype on S-IRA and whether intracerebroventricular (ICV) microinjection of an SSRI would reduce S-IRA susceptibility in DBA/1 mice...
October 12, 2016: Epilepsy & Behavior: E&B
Sharon Shmuely, Sanjay M Sisodiya, W Boudewijn Gunning, Josemir W Sander, Roland D Thijs
INTRODUCTION: Premature mortality is a major issue in Dravet syndrome (DS). To improve understanding of DS premature mortality, we conducted a comprehensive literature search with a particular emphasis on SUDEP. METHODS: We searched PubMed, Embase, Web of Science, Cochrane, CENTRAL, CINAHL, PsycINFO, Academic Search Premier, and ScienceDirect on the following terms: "Dravet syndrome", "severe myoclonic epilepsy", "SMEI", "mortality", "survivors", "prognosis", and "death"...
October 9, 2016: Epilepsy & Behavior: E&B
Srinivasa P Kommajosyula, Marcus E Randall, Srinivasan Tupal, Carl L Faingold
Patients with epilepsy are at risk of sudden unexpected death in epilepsy (SUDEP). The most common series of events in witnessed cases of SUDEP is a generalized convulsive seizure followed by terminal apnea. Risk factors for SUDEP include prolonged postictal depression (PID), as well as alcohol abuse. The present study examined these issues in a genetic epilepsy model that exhibits generalized convulsive audiogenic seizures (AGSz) but rarely exhibits seizure-induced death, the genetically epilepsy-prone rats (GEPR-9s)...
October 7, 2016: Epilepsy & Behavior: E&B
S Keddie, H Angus-Leppan, T Parker, S Toescu, A Nash, O Adewunmi, Rsn Liu
OBJECTIVE: To examine patient knowledge about sudden unexpected death in epilepsy (SUDEP) compared to other risks in epilepsy. To explore patients' experiences surrounding SUDEP disclosure and opinions on how information should be delivered. DESIGN: A cross-sectional questionnaire. SETTING: Royal Free Hospital, London outpatient epilepsy clinics. PARTICIPANTS: New and follow-up patients attending epilepsy clinics at a London teaching hospital over six months...
September 2016: JRSM Open
Gordon F Buchanan, George B Richerson
No abstract text is available yet for this article.
October 2016: Nature Reviews. Neurology
Diego F Tovar Quiroga, Jeffrey W Britton, Elaine C Wirrell
PURPOSE: Seizure detection devices (SDD) may reduce the potential for seizure-related injury, SUDEP or status epilepticus. We performed a survey of persons with epilepsy (PWE) and caregivers to assess their perspectives regarding the features and priorities that should be considered in the design of these devices. METHODS: PWE/caregiver completed a survey which assessed the worry of undetected seizures, the impact of this concern on diurnal functioning/sleep and the level of interest in using SDD...
October 2016: Seizure: the Journal of the British Epilepsy Association
Eija Gaily, Markus Lommi, Risto Lapatto, Anna-Elina Lehesjoki
OBJECTIVE: Population-based studies on infantile epilepsy syndromes are scarce. Our aim was to provide syndrome-specific data on the incidence and outcome of epilepsy in a population-based cohort of infants with epilepsy onset in the first year. METHODS: Included were all infants born in 1997 through 2006 whose epileptic seizures started before 12 months of age and who were residents of the Helsinki University Hospital district at the time of seizure onset. Patients were ascertained from hospital statistics, and all patient charts were reviewed...
October 2016: Epilepsia
Orrin Devinsky, Dale C Hesdorffer, David J Thurman, Samden Lhatoo, George Richerson
Sudden unexpected death in epilepsy (SUDEP) can affect individuals of any age, but is most common in younger adults (aged 20-45 years). Generalised tonic-clonic seizures are the greatest risk factor for SUDEP; most often, SUDEP occurs after this type of seizure in bed during sleep hours and the person is found in a prone position. SUDEP excludes other forms of seizure-related sudden death that might be mechanistically related (eg, death after single febrile, unprovoked seizures, or status epilepticus). Typically, postictal apnoea and bradycardia progress to asystole and death...
September 2016: Lancet Neurology
Anouk Van de Vel, Kris Cuppens, Bert Bonroy, Milica Milosevic, Katrien Jansen, Sabine Van Huffel, Bart Vanrumste, Patrick Cras, Lieven Lagae, Berten Ceulemans
PURPOSE: Detection of, and alarming for epileptic seizures is increasingly demanded and researched. Our previous review article provided an overview of non-invasive, non-EEG (electro-encephalography) body signals that can be measured, along with corresponding methods, state of the art research, and commercially available systems. Three years later, many more studies and devices have emerged. Moreover, the boom of smart phones and tablets created a new market for seizure detection applications...
October 2016: Seizure: the Journal of the British Epilepsy Association
Kevin G Hampel, Amirhossein Jahanbekam, Christian E Elger, Rainer Surges
OBJECTIVE: Cardiorespiratory function alterations are commonly observed with epileptic seizures and may lead to syncope and sudden unexpected death in epilepsy (SUDEP). Although most previous research has focused on controlling heart rate (HR) and respiration, little is known about seizure-related regulation of systemic blood pressure (BP). Herein, we have investigated whether the periictal modulation of systemic BP and HR depends on seizure characteristics. METHODS: Systemic arterial BP, HR, and peripheral capillary oxygen saturation (SPO2 ) were continuously and noninvasively monitored using the ccNexfin device in those epilepsy patients undergoing video-electroencephalography (EEG) telemetry...
October 2016: Epilepsia
Krishnan Ravindran, Kim L Powell, Marian Todaro, Terence J O'Brien
Alterations in cardiac electrophysiology are an established consequence of long-standing drug resistant epilepsy. Patients with chronic epilepsy display abnormalities in both sinoatrial node pacemaker current as well as ventricular repolarizing current that places them at a greater risk of developing life-threatening cardiac arrhythmias. The development of cardiac arrhythmias secondary to drug resistant epilepsy is believed to be a key mechanism underlying the phenomenon of Sudden Unexpected Death in EPilepsy (SUDEP)...
August 11, 2016: Epilepsy Research
Rebecca L Holt, Eric Arehart, Arsen Hunanyan, Nina A Fainberg, Mohamad A Mikati
Several factors, such as epilepsy syndrome, poor compliance, and increased seizure frequency increase the risks of sudden unexpected death in epilepsy (SUDEP). Animal models have revealed that the mechanisms of SUDEP involve initially a primary event, often a seizure of sufficient type and severity, that occurs in a brain, which is vulnerable to SUDEP due to either genetic or antecedent factors. This primary event initiates a cascade of secondary events starting, as some models indicate, with cortical spreading depolarization that propagates to the brainstem where it results in autonomic dysfunction...
May 2016: Seminars in Pediatric Neurology
Gordon F Buchanan, George B Richerson
No abstract text is available yet for this article.
September 2016: Nature Reviews. Neurology
Dirk-Matthias AltenmĂŒller, Andreas Schulze-Bonhage, Christian E Elger, Rainer Surges
OBJECTIVES: Postictal generalized EEG suppression (PGES) frequently occurs after generalized convulsive seizures (GCS) and may be involved in the pathophysiology of sudden unexpected death in epilepsy (SUDEP). It is usually determined using conventional scalp EEG which is likely to miss cerebral activity in deeper brain structures. Here, we examined intracranial EEG activity after GCS to unravel the pattern and extent of local brain activity during apparent PGES on scalp EEG (s-PGES)...
September 2016: Epilepsy & Behavior: E&B
Barbara L Kroner, Mansour Fahimi, William D Gaillard, Anne Kenyon, David J Thurman
Self-reported epilepsy may be influenced by culture, knowledge, and beliefs. We screened 6420 residents of the District of Columbia (DC) for epilepsy to investigate whether socio-demographics were associated with whether they reported their diagnosis as epilepsy or as seizure disorder. Lifetime and active prevalence rates were 0.54% and 0.21%, respectively for 'epilepsy' and 1.30% and 0.70%, respectively for 'seizure disorder'. Seizure disorder was reported significantly more often than epilepsy among blacks, females, respondents≄50years, those with lower level education, respondents who lived alone and in low income neighborhoods, and those who resided in DC for at least five years...
September 2016: Epilepsy & Behavior: E&B
Sharon Shmuely, Rainer Surges, Josemir W Sander, Roland D Thijs
BACKGROUND: Most victims of sudden unexpected death in epilepsy (SUDEP) are found prone with signs suggestive of an unwitnessed convulsive seizure (CS). Prone sleeping has been proposed as a risk factor for SUDEP. Little is known, however, about the change of body position during the course of CSs. METHODS: We retrospectively reviewed video-EEG data and assessed body positions during the course of CSs, until there was a physical interaction by nursing staff with the subject...
September 2016: Epilepsy & Behavior: E&B
Fulvio A Scorza, Esper A Cavalheiro, Jaderson Costa da Costa
Sudden unexpected death in epilepsy (SUDEP) is the most common cause of death in people with intractable epilepsy. Probably, optimization of seizure control will prevent some of these deaths. Briefly, we integrated in this paper some data about the epidemiology, risk factors, etiology, and preventative measures in the management of SUDEP.
July 2016: Arquivos de Neuro-psiquiatria
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