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https://www.readbyqxmd.com/read/28091857/lack-of-heart-rate-variability-during-sleep-related-apnea-in-patients-with-temporal-lobe-epilepsy-tle-an-indirect-marker-of-sudep
#1
C S Nayak, S Sinha, M Nagappa, K Thennarasu, A B Taly
PURPOSE: Apneas occurring during sleep may precipitate autonomic instability in epilepsy patients making them susceptible to sudden death (SUDEP). Literature on heart rate variability (HRV) during apnea among patients with temporal lobe epilepsy (TLE) is sparse. The aim of this study was to characterize the HRV during the peri-apneic period in patients with TLE and compare with HRV of matched healthy individuals during the overnight polysomnographic (PSG) recording. Further, the role of carbamazepine (CBZ) in modulating peri-apneic HRV in the above cohort was also assessed...
January 14, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28088049/sudden-unexpected-death-in-epilepsy-sudep-disclosure-in-pediatric-epilepsy-an-italian-survey-on-to-tell-or-not-to-tell
#2
Galli Federica, Vignoli Aglaia, Canevini Maria Paola, Cerioli Gabriele, Vegni Elena
BACKGROUND AND OBJECTIVES: Although there has recently been significant debate regarding the importance of disclosing the risk of SUDEP, professional societies and clinical practice guidelines currently recommend that the risk of SUDEP be disclosed as part of a comprehensive epilepsy education program. Therefore, the primary aim of the present study was to examine whether healthcare providers treating pediatric patients with epilepsy in Italy would disclose the risk of SUDEP to the parents of children with epilepsy...
January 11, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28087824/neuropathology-of-sudep-role-of-inflammation-blood-brain-barrier-impairment-and-hypoxia
#3
Zuzanna Michalak, Dima Obari, Matthew Ellis, Maria Thom, Sanjay M Sisodiya
OBJECTIVE: To seek a neuropathologic signature of sudden unexpected death in epilepsy (SUDEP) in a postmortem cohort by use of immunohistochemistry for specific markers of inflammation, gliosis, acute neuronal injury due to hypoxia, and blood-brain barrier (BBB) disruption, enabling the generation of hypotheses about potential mechanisms of death in SUDEP. METHODS: Using immunohistochemistry, we investigated the expression of 6 markers (CD163, human leukocyte antigen-antigen D related, glial fibrillary acid protein, hypoxia-inducible factor-1α [HIF-1α], immunoglobulin G, and albumin) in the hippocampus, amygdala, and medulla in 58 postmortem cases: 28 SUDEP (definite and probable), 12 epilepsy controls, and 18 nonepileptic sudden death controls...
January 13, 2017: Neurology
https://www.readbyqxmd.com/read/28070172/summary-of-the-2016-partners-against-mortality-in-epilepsy-pame-conference
#4
(no author information available yet)
Authors: Kevin D Graber, MD, Jeffrey Buchhalter, MD, PhD, Elson So, MD, Rainer Surges, MD, Detlev Boison, PhD, Franck Kalume, PhD, Cyndi Wright, Brian Gehlbach, MD, Jeff Noebels, MD, PhD, Vicky Whittemore, PhD, Elizabeth J. Donner, MD, MSc, Tom Stanton, MPP, Henry Smithson, MD, Jane Hanna, Masud Seyal, MD, PhD, Philippe Ryvlin, MD, PhD The third biannual Partners Against Mortality in Epilepsy (PAME) conference was held in Alexandria, VA from June 23-26, 2016. This was an intimate meeting of clinical and basic scientists, clinicians, people affected by Sudden Unexpected Death in Epilepsy Patients (SUDEP) in a loved one, people living with epilepsy and patient advocate organizations...
November 2016: Epilepsy Currents
https://www.readbyqxmd.com/read/28043059/determining-factors-of-electrocardiographic-abnormalities-in-patients-with-epilepsy-a-case-control-study
#5
Jorge Murilo Barbosa de Sousa, Guilherme Loureiro Fialho, Peter Wolf, Roger Walz, Katia Lin
Sudden unexpected death in epilepsy (SUDEP) is a major cause of mortality in young patients with epilepsy (PWE). Although its mechanisms are still poorly understood, they may include cardiorespiratory dysfunction. Standard 12-lead electrocardiograms (ECGs) were obtained from 62 consecutive patients (aged 18-66y) with a definite diagnosis of epilepsy, without seizures at the day of ECG, and 57 healthy controls matched for sex, age and body mass index (BMI). All ECGs were evaluated by a blinded board-certified cardiologist...
December 20, 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27988968/premature-mortality-of-epilepsy-in-low-and-middle-income-countries-a-systematic-review-from-the-mortality-task-force-of-the-international-league-against-epilepsy
#6
REVIEW
Francis Levira, David J Thurman, Josemir W Sander, W Allen Hauser, Dale C Hesdorffer, Honorati Masanja, Peter Odermatt, Giancarlo Logroscino, Charles R Newton
To determine the magnitude of risk factors and causes of premature mortality associated with epilepsy in low- and middle-income countries (LMICs). We conducted a systematic search of the literature reporting mortality and epilepsy in the World Bank-defined LMICs. We assessed the quality of the studies based on representativeness; ascertainment of cases, diagnosis, and mortality; and extracted data on standardized mortality ratios (SMRs) and mortality rates in people with epilepsy. We examined risk factors and causes of death...
January 2017: Epilepsia
https://www.readbyqxmd.com/read/27935023/causes-of-mortality-in-individuals-with-tuberous-sclerosis-complex
#7
Sam Amin, Andrew Lux, Nuala Calder, Matthew Laugharne, John Osborne, Finbar O'callaghan
AIM: The causes of death in patients with tuberous sclerosis complex (TSC) have rarely been studied, with only one published account, which was reported from the Mayo Clinic in 1991. We aimed to investigate mortality in a large cohort of patients with TSC from one of two national referral clinics in the UK. METHOD: We identified 284 patients who attended Bath TSC clinic between 1981 and 2015, and ascertained causes of death by reviewing medical records, death certificates, and postmortem reports...
December 9, 2016: Developmental Medicine and Child Neurology
https://www.readbyqxmd.com/read/27888514/the-burden-of-premature-mortality-of-epilepsy-in-high-income-countries-a-systematic-review-from-the-mortality-task-force-of-the-international-league-against-epilepsy
#8
REVIEW
David J Thurman, Giancarlo Logroscino, Ettore Beghi, W Allen Hauser, Dale C Hesdorffer, Charles R Newton, Fulvio Alexandre Scorza, Josemir W Sander, Torbjörn Tomson
Since previous reviews of epidemiologic studies of premature mortality among people with epilepsy were completed several years ago, a large body of new evidence about this subject has been published. We aim to update prior reviews of mortality in epilepsy and to reevaluate and quantify the risks, potential risk factors, and causes of these deaths. We systematically searched the Medline and Embase databases to identify published reports describing mortality risks in cohorts and populations of people with epilepsy...
January 2017: Epilepsia
https://www.readbyqxmd.com/read/27886630/the-role-of-antiepileptic-drugs-in-sudden-unexpected-death-in-epilepsy
#9
REVIEW
Dag Aurlien, Leif Gjerstad, Erik Taubøll
Sudden unexpected death in epilepsy (SUDEP) primarily affects young adults and is the leading cause of death related directly to seizures. High frequency of generalized tonic-clonic seizures is the most important risk factor, and effective seizure protection is probably the most important measure to prevent these tragic deaths. For several years a potential role of antiepileptic drugs (AEDs) has been discussed, but at present there is wide agreement that choice of AED therapy does not influence the risk. However, although it is well known that the efficacy and safety profiles of AEDs may differ significantly when used in the treatment of genetic epilepsy compared to symptomatic or cryptogenic epilepsy, this has generally been overlooked in epidemiologic studies of possible relationships between AEDs and SUDEP...
December 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27869560/syncope-seizure-or-both
#10
Paraskevi Koutrolou-Sotiropoulou, Abhijeet Singh, Mason Leeman-Markowski, Eric J Rashba
Ictal asystole (IA) is a rare phenomenon in patients with seizures with an incidence of 0.27-0.4% and has been proposed as a possible mechanism of sudden unexpected death in epilepsy patients (SUDEP). We present a case of a 53-year-old woman who initially presented with episodes of expressive aphasia and was treated with antiepileptic drugs (AEDs). While on therapy she experienced episodes of syncope. During her hospitalization for tapering of AEDs and 24-hour EEG monitoring, the patient developed a seizure followed by sinus bradycardia and an 18-second sinus pause, resulting in loss of consciousness and slowing of cerebral activity...
March 2016: Acute Cardiac Care
https://www.readbyqxmd.com/read/27864903/long-term-monitoring-of-cardiorespiratory-patterns-in-drug-resistant-epilepsy
#11
Daniel M Goldenholz, Amanda Kuhn, Alison Austermuehle, Martin Bachler, Christopher Mayer, Siegfried Wassertheurer, Sara K Inati, William H Theodore
OBJECTIVE: Sudden unexplained death in epilepsy (SUDEP) during inpatient electroencephalography (EEG) monitoring has been a rare but potentially preventable event, with associated cardiopulmonary markers. To date, no systematic evaluation of alarm settings for a continuous pulse oximeter (SpO2 ) has been performed. In addition, evaluation of the interrelationship between the ictal and interictal states for cardiopulmonary measures has not been reported. METHODS: Patients with epilepsy were monitored using video-EEG, SpO2 , and electrocardiography (ECG)...
January 2017: Epilepsia
https://www.readbyqxmd.com/read/27858372/severe-respiratory-acidosis-in-status-epilepticus-as-a-possible-etiology-of-sudden-death-in-lesch-nyhan-disease-a-case-report-and-review-of-the-literature
#12
Alison Christy, William Nyhan, Jenny Wilson
INTRODUCTION: Lesch-Nyhan disease (LND) is an X-linked disorder of purine metabolism, associated with self-mutilation, dystonia, and chorea. Seizures are uncommon in LND. Patients with LND are at risk for sudden and unexpected death. The etiology of this is unknown, but appears to occur from a respiratory process. We propose that respiratory failure secondary to subclinical seizure may lead to sudden death in these patients. CASE: We report a case of an 11-year-old boy with LND who had two episodes of nocturnal gasping...
November 18, 2016: JIMD Reports
https://www.readbyqxmd.com/read/27857627/a-clue-to-seizure-induced-sudep-risk-loss-of-brainstem-serotonergic-control-of-cardiorespiratory-function
#13
Heidi L Grabenstatter
No abstract text is available yet for this article.
November 2016: Epilepsy Currents
https://www.readbyqxmd.com/read/27817982/from-genotype-to-phenotype-in-dravet-disease
#14
Svetlana Gataullina, Olivier Dulac
Dravet syndrome combines clonic generalized, focal or unilateral seizures, beginning within the first year of life, often triggered by hyperthermia whatever its cause, including pertussis vaccination. Long-lasting febrile seizures are frequent in infancy and repeat status epilepticus (SE) has negative prognostic value. Massive myoclonus, rare absences, complex partial seizures and generalized spikes may appear several years later. Myoclonic status may occur in childhood, but acute encephalopathy with febrile SE followed by ischemic lesions and psychomotor impairment, the most severe condition, occurs mainly within the first five years of life...
October 21, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27810515/mortality-in-dravet-syndrome
#15
Monica S Cooper, Anne Mcintosh, Douglas E Crompton, Jacinta M McMahon, Amy Schneider, Kevin Farrell, Vijeya Ganesan, Deepak Gill, Sara Kivity, Tally Lerman-Sagie, Ailsa McLellan, James Pelekanos, Venkateswaran Ramesh, Lynette Sadleir, Elaine Wirrell, Ingrid E Scheffer
We measured the mortality rate and the rate of Sudden Unexpected Death in Epilepsy (SUDEP) in Dravet Syndrome (DS). We studied a cohort of 100 consecutively recruited, unrelated patients with DS; 87 had SCN1A mutations. Living cases had a median follow-up of 17 years. Seventeen patients died, at a median age of seven years (inter-quartile range 3-11 years) with causes of death: 10 SUDEP, four status epilepticus, two drowning and one asphyxia. The SUDEP classification included three Definite, one Definite Plus and six Probable...
December 2016: Epilepsy Research
https://www.readbyqxmd.com/read/27809868/efficacy-of-naloxone-in-reducing-postictal-central-respiratory-dysfunction-in-patients-with-epilepsy-study-protocol-for-a-double-blind-randomized-placebo-controlled-trial
#16
Sylvain Rheims, Luc Valton, Véronique Michel, Louis Maillard, Vincent Navarro, Philippe Convers, Fabrice Bartolomei, Arnaud Biraben, Arielle Crespel, Philippe Derambure, Bertrand de Toffol, Edouard Hirsch, Philippe Kahane, Martine Lemesle Martin, Didier Tourniaire, Sébastien Boulogne, Catherine Mercier, Pascal Roy, Philippe Ryvlin
BACKGROUND: Generalized tonic-clonic seizures (GTCSs) are the main risk factor for sudden unexpected death in epilepsy (SUDEP). Experimental and clinical data strongly suggest that the majority of SUDEP results from a postictal respiratory dysfunction progressing to terminal apnea. Postictal apnea could partly derive from a seizure-induced massive release of endogenous opioids. The main objective of this study is to evaluate the efficacy of an opioid antagonist, naloxone, administered in the immediate aftermath of a GTCS, in reducing the severity of the postictal central respiratory dysfunction...
November 3, 2016: Trials
https://www.readbyqxmd.com/read/27799864/irregular-respiratory-rhythm-a-physiological-biomarker-of-sudep-risk-in-patients-with-nocturnal-seizures
#17
Heidi L Grabenstatter
No abstract text is available yet for this article.
September 2016: Epilepsy Currents
https://www.readbyqxmd.com/read/27791149/cardiac-arrhythmia-in-a-mouse-model-of-sodium-channel-scn8a-epileptic-encephalopathy
#18
Chad R Frasier, Jacy L Wagnon, Yangyang Oliver Bao, Luke G McVeigh, Luis F Lopez-Santiago, Miriam H Meisler, Lori L Isom
: Patients with early infantile epileptic encephalopathy (EIEE) are at increased risk for sudden unexpected death in epilepsy (SUDEP). De novo mutations of the sodium channel gene SCN8A, encoding the sodium channel Nav1.6, result in EIEE13 (OMIM 614558), which has a 10% risk of SUDEP. Here, we investigated the cardiac phenotype of a mouse model expressing the gain of function EIEE13 patient mutation p.Asn1768Asp in Scn8a (Nav1.6-N1768D). We tested Scn8a(N1768D/+) mice for alterations in cardiac excitability...
October 26, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27773556/sudden-death-in-epilepsy-insights-from-the-last-25-years
#19
Lliwen A Jones, Rhys H Thomas
Sudden unexpected death in epilepsy (SUDEP) is the leading cause of mortality in patients with refractory epilepsy, and as such has been a major research focus over the last 25 years. The earliest SUDEP research papers were published in Seizure, as have scores of SUDEP papers since. In this review we discuss the efforts to try and describe the pathophysiological basis of SUDEP, the drive to discover the clinical risk factors that increase the likelihood of SUDEP, and the motivation to increase awareness of SUDEP...
October 14, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27768696/fine-mapping-of-a-dravet-syndrome-modifier-locus-on-mouse-chromosome-5-and-candidate-gene-analysis-by-rna-seq
#20
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
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