sudden unexplained death in epilepsy

Thus, proning may be the consequence of forced ictal body version rather than sleeping prone (64). Prolongation of postictal generalized electroencephalographic suppression is a marker of seizure severity and has been suggested as a premortem risk identifier and was noted in all cases of monitored sudden unexplained death in epilepsy in the MORTEMUS study (94). The https:// ensures that you are connecting to the -, Devinsky O, Bundock E, Hesdorffer D, Donner E, Moseley B, Cihan E, Hussain F, Friedman D. Resolving ambiguities in SUDEP classification. Clinical pharmacology: drugs as a benefit and/or risk in sudden unexpected death in epilepsy? Hippocampal morphometry in sudden and unexpected death in epilepsy. Incidence, recurrence, and risk factors for peri-ictal central apnea and sudden unexpected death in epilepsy. However, postmortem examinations of sudden unexplained death in epilepsy patients often show abnormalities that could be potential contributors to death or could be the cause or consequence of seizures and epilepsy or other comorbidities either related or unrelated to epilepsy (77). In the February 2012 issue of the Journal, Epilepsia, there are two studies that address the risk factors of sudden unexpected death in epilepsy (SUDEP). Significant postictal hypotension: expanding the spectrum of seizure-induced autonomic dysregulation. Dear Readers, Contributors, Editorial Board, Editorial staff and Publishing team members, Sudden unexplained death in epilepsy preventive measures are currently focused on achieving adequate seizure control and seizure detection to ensure lifesaving interventions. Conversely, cortical thickening has been reported in postcentral gyri and limbic structures such as the insula, amygdala, and cingulate cortex (83). MeSH Availability of cardiorespiratory data in 10 of 16 sudden unexplained death in epilepsy cases in MORTEMUS allowed identification of a relatively consistent pattern leading to death. Another potential explanation is primary cardiac dysfunction, in the setting of a seizure-induced Takotsubo cardiomyopathy, although such a scenario transpires over a period of many hours to days (105; 23). Loonen ICM, Jansen NA, Cain SM, et al. We celebrate the stories, culture and traditions of Aboriginal and Torres Strait Islander Peoples of all communities who also work and live on this land. Advances in wearable and bed-based devices may facilitate sudden unexplained death in epilepsy risk management through multiple pathways (71; 93). Active management of epilepsy likely results in sudden unexplained death in epilepsy risk reduction; this includes aggressive pharmacological treatment, epilepsy surgery, vagal nerve or deep brain stimulation, and responsive neurostimulation (39; 24; 95; Sveinsson et al Neurology 2020b). Kv7.1 has been found in brain tissue and mice bearing the human mutation in this channel show seizures and malignant cardiac arrhythmias (36). Your continued use of this site is subject to the provisions of Epilepsy Action Australia's Legal & Privacy Policy, Epilepsy: In Memoriam you are not alone, Click here to read more on the Global Conversation, The risk increases if you have tonic clonic seizures, especially if they happen at night or when asleep, The more frequent the tonic clonic seizures, the higher the risk. Ictal asystole: a systematic review. Both ictal bradycardia and asystole were only found in seizures of focal onset (119). They don't usually cause problems unless they happen regularly, perhaps because of emotional distress. The use of anti-suffocation pillows has been advocated by some practitioners to improve respiration while sleeping, but their effects remain unproven because experimental studies are lacking. There are 17 long QT syndrome subtypes with monogenic mutations of 15 autosomal genes with variable penetrance (125). In a case series of 2 definite sudden unexplained death in epilepsy and 1 probable sudden unexplained death in epilepsy adult patients, and in a case report of a near-sudden unexplained death in epilepsy in a pediatric patient, the terminal event occurred without a prior generalized convulsive seizure or typical clinical seizure (65). Safety considerations for patients with epilepsy taking antiepileptic drugs alongside caffeine or other methylxanthine derivatives. However, the extent of the edema observed does not account for death. Walker F, Fish DR. Recording respiratory parameters in patients with epilepsy. Programs Briefs | Epilepsy Foundation, Discrimination in Federally Funded Programs Briefs, First Responders and Seizure Management Briefs, Resources and Seizure Action Plans for Summer Camp, Explaining Epilepsy to Friends and Family, Epilepsy Foundation Individual and Family Services, About Research and Funding at Epilepsy Foundation, The Epilepsy Learning Healthcare System (ELHS), Access the Rare Epilepsy Network Registry, #AimForZero: Striving Toward a Future Free from Sudden Unexpected Death in Epilepsy, Advocacy: Access Prescription Medications, Advocacy: Affordable Comprehensive Health Coverage, Teens Speak Up! Background: People with intellectual disability (ID) and epilepsy are more likely to die prematurely than the general population. All rights reserved. Buchanan GF, Murray NM, Hajek MA, Richerson GB. Nass RD, Motloch LJ, Paar V, et al. Multiple studies have focused on identifying risk factors for sudden unexplained death in epilepsy. From the Editor in Chief (interim), Subhash Banerjee, MD. 2020 Apr 2;4(4):CD011792. Having active seizures can put you at risk, and there are certain types of seizures which can increase a persons risk of SUDEP. Lancet Neurol. Neuropathological studies have also demonstrated a reduction in somatosatin neurons, neurokinin 1 receptor in the ventrolateral medulla, as well as a reduction of galanin and tryptophan hydroxylase in the ventrolateral medulla and raphe (88). For more informationclick here. Before A police spokeswoman said: Officers were called to Marchburn Park, Aberdeen at around 9.20am on Saturday, October 29 following the sudden death of a seven month old baby boy. Epilepsy-related causes can be further classified as indirect, such as suicide, or direct causes such as status epilepticus and sudden unexpected death in epilepsy (116; 26). Clinical spectrum of SCN5A mutations: long QT syndrome, Brugada syndrome, and cardiomyopathy. Christiansen, S. N., Jacobsen, S. B., Andersen, J. D., Kampmann, M.-L., Truds, L. C., Olsen, K. B., Morling, N. (2021). Administration of citalopram, a selective serotonin reuptake inhibitor, does not reduce mortality in Lmx1bf/f/p mice but it does in controls (16). Meritam P, Ryvlin P, Beniczky S. User-based evaluation of applicability and usability of a wearable accelerometer device for detecting bilateral tonic-clonic seizures: a field study. SUDEP stands for Sudden Unexpected Death in Epilepsy. Epilepsy and seizure-related deaths: Mortality statistics do not tell the complete story. Lancet Neurol 2013;12(10):966-77.**. Sudden unexplained death in epilepsy risk in males is 1.45 (95% CI 1.07-1.88) compared to females (41). Patients with KCNH2 mutations (long QT syndrome type 2) have a seizure phenotype more common (47%) than long QT syndrome types 1 (22%) and 3 (25%) (46). Pulmonary edema is the most frequent pulmonary finding in sudden unexplained death in epilepsy patients (71%), followed by pulmonary hemorrhage (3%) and lung hyperinflation (0.3%) (77). Marini C, Scheffer IE, Nabbout R, et al. Administration of fluoxetine has been shown to prevent seizure-induced respiratory arrest in a mouse model of sudden unexplained death in epilepsy (117; 33). Sunburn. Other genes related to epilepsy, sudden death, and cardiac disease include but are not limited to: FBN1, HCN1, SCN4A, EFHC1, CACNA1A, SCN11A, SCN10A, and DEPDC5 (21). [14] Many of the genes are involved in Long QT syndrome. The condition occurs in those who suffer from epilepsy and often times is onset by unexplained forces, resulting in a sudden death that has no clear cause following autopsies. Donahue LM, Coates PW, Lee VH, Ippensen DC, Arze SE, Poduslo SE. Sudden unexplained death in children with epilepsy. Copyright 2022, StatPearls Publishing LLC. Sudden unexpected death in epilepsy: people with nocturnal seizures may be at highest risk. This classification algorithm was then applied to the LTG cases, and SUDEP rates were calculated using patient-years of exposure as the denominator. government site. Epilepsy Behav 2019a;97:260-4. Ryvlin P, Ciumas C, Wisniewski I, Beniczky S. Wearable devices for sudden unexpected death in epilepsy prevention. Epilepsy centers provide you with a team of specialists to help you diagnose your epilepsy and explore treatment options. Similarly, in Scn8a mutant mice, apnea initiated during the tonic phase was noted in all the cases of seizure induced death preceding asystole; although it was also noted in nonfatal seizures, suggesting that apnea was necessary but not sufficient to produce death (127). JAMA Neurol 2018a;75(2):194-202. Other clinical risk factors associated with sudden unexplained death in epilepsy after adjustment for generalized convulsive seizures frequency are a history of substance abuse or alcohol dependence. Tomson T, Hirsch LJ, Friedman D, Bester N, Hammer A, Irizarry M, Ishihara L, Krishen A, Spaulding T, Wamil A, Leadbetter R. Epilepsia. Your lifestyle and treatment choices are important. Epilepsia 2014;55(2):e6-12. All monitored sudden unexplained deaths in epilepsy (11 of 16) and all near-sudden unexplained deaths in epilepsy had a seizure immediately prior to cardiorespiratory arrest. The person with epilepsy is often found dead in bed and doesn't appear to have had a convulsive seizure. Laryngoscope 1986;96(5):506-9. Hampel KG, Thijs RD, Elger CE, Surges R. Recurrence risk of ictal asystole in epilepsy. Epilepsia 2016;57(3):412-7. Cooper MS, McIntosh A, Crompton DE, et al. Because sudden unexplained death in epilepsy is a terminal event, this section does not apply. Epilepsia 2011;52 Suppl 1:28-38. Alexandre VB, Mercedes L, Valton L, et al. Severe peri-ictal respiratory dysfunction is common in Dravet syndrome. Cochrane Database Syst Rev 2020;(4)4:CD011792. In order to harmonize diagnostic criteria for sudden unexplained death in epilepsy, the following categories have been suggested (78): Definite sudden unexplained death in epilepsy: sudden, unexpected witnessed or unwitnessed and nondrowning death, occurring in benign circumstances in an individual with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a cause of death. National Association of Medical Examiners Position Paper: Recommendations for the Investigation and Certification of Deaths in People with Epilepsy. Accessibility HHS Vulnerability Disclosure, Help Bethesda, MD 20894, Web Policies Sudden unexplained death syndrome (SUDS) accounts for about 10% of deaths in patients with epilepsy. Ann Neurol 1997;42(4):588-94. Conversely, adenosine promotes seizure termination and suppresses cardiorespiratory function by activation of its A1 receptors whereas blockage of A1 receptors or activation of A2a receptors has the opposite effect (30; 40; 15; 87). In all monitored sudden unexplained death in epilepsy cases and in 7 of 9 of near-sudden unexplained death in epilepsy cases, including both fatal near-sudden unexplained death in epilepsy cases, the terminal seizure was a generalized convulsive seizure. 2020 Nov;10(11):e01838. Lacuey N, Zonjy B, Londono L, Lhatoo SD. Nonseizure SUDEP: Sudden unexpected death in epilepsy without preceding epileptic seizures. Increased risk of sudden unexpected death in epilepsy in females using lamotrigine: a nested, case-control study. Expression and functional role for ether-a-go-go currents in hippocampal astrocytes. The Importance of National Epilepsy Awareness Month. In contrast, postictal asystole has been described in sudden unexplained death in epilepsy and near-sudden unexplained death in epilepsy cases, suggesting a different physiopathology and potentially lethal implications (119; 122). Expert Opin Drug Metab Toxicol 2014;10(7):981-9. Changes in blood pressure have been described during and after seizures (44; 14). From healthy diet plans to helpful weight loss tools, here you'll find WebMD's latest diet news and information. A diagnosis of a genetic generalized epilepsy was made and his medication changed from carbamazepine and levetiracetam combination to 1 of lamotrigine (200 mg twice daily) and valproic acid (1500 mg twice daily). We use cookies to ensure that we give you the best experience on our website. Neurology 2017;88(7):701-5. Murugesan A, Rani MRS, Vilella L, et al. Causes of death in individuals with epilepsy include non-epilepsy-related conditions such as suicide, cancers, and cardiovascular disease, as well as epilepsy-related provoked causes, including status epilepticus, anti-seizure drug effects, motor vehicle and bicycle accidents, alcohol withdrawal, aspirational pneumonia, and drowning. Bookshelf Epub 2011 May 15. These disparities may be explained by lower access to specialty care and medications as well as poorer understanding of their disease in those patients from lowest-income communities compared to those from the highest income (19). Epilepsy: Taking Your Medicines Properly. To date, the major risk factor for sudden unexplained death in epilepsy is the presence and frequency of generalized convulsive seizures and ongoing seizures despite adequate treatment (41; 39; 107). 2000 Jan;7(1):34-7. doi: 10.1054/jocn.1999.0137. Neurology 2021;96(3):e352-65. 1998 Feb;39(2):206-12. doi: 10.1111/j.1528-1157.1998.tb01360.x. Taking medication as prescribed is important even when you are not experiencing tonic-clonic seizures. Lhatoo SD, Faulkner HJ, Dembny K, Trippick K, Johnson C, Bird JM. Hypoxemia following generalized convulsive seizures: Risk factors and effect of oxygen therapy. J Neurol Neurosurg Psychiatry 2018;89(10):1064-70. Postictal death is associated with tonic phase apnea in a mouse model of sudden unexpected death in epilepsy. The remainder were adults, with a mean age of 41.3 years (19 to 62). Levetiracetam is the sole medication in monotherapy that has been associated with a decreased sudden unexplained death in epilepsy risk event after adjusting for generalized convulsive seizure frequency (108). Uteshev VV, Tupal S, Mhaskar Y, Faingold CL. Purpose: The present study was conducted to determine the rate of sudden unexplained death in epilepsy (SUDEP) in a well-defined cohort of patients included in the lamotrigine (LTG) Patients with nocturnal seizures are at a higher risk and physical nocturnal supervision or supervision through remote listening devices is recommended, although the evidence for this is weak (61; 68). Being asleep during a seizure is likely to favour SUDEP occurrence. Neurology 2019;92(3):e183-93. Maguire MJ, Jackson CF, Marson AG, Nolan SJ. Nass RD, Hampel KG, Elger CE, Surges R. Blood pressure in seizures and epilepsy. Probable sudden unexplained death in epilepsy/probable sudden unexplained death in epilepsy plus: same as definite sudden unexplained death in epilepsy/sudden unexplained death in epilepsy plus but without autopsy. J Clin Invest 2018;128(3):1141-53. Mutations in KCNQ1, KCNH2, and SCN5A comprise the vast majority of long QT syndrome cases (46). A significant number of deaths in people with epilepsy may be Tami TA, Chu F, Wildes TO, Kaplan M. Pulmonary edema and acute upper airway obstruction. Epilepsia 2018a;59(3):555-61. Epub 2011 Nov 29. In a case control study, nocturnal generalized convulsive seizures were associated with a 9-fold risk for sudden unexplained death in epilepsy. These neurons project throughout the central nervous system, including cortex, hippocampus, and other brainstem nuclei, such as the major respiratory control centers (92). Well be right back. An increased risk of sudden unexplained death in epilepsy has also been reported in patients with early onset (< 16 years) and longstanding (> 15 years) epilepsy (41). An official website of the United States government. Kuo J, Zhao W, Li CS, Kennedy JD, Seyal M. Postictal immobility and generalized EEG suppression are associated with the severity of respiratory dysfunction. Bateman LM, Spitz M, Seyal M. Ictal hypoventilation contributes to cardiac arrhythmia and SUDEP: report on two deaths in video-EEG-monitored patients. Obstet Med. Lamberts RJ, Laranjo S, Kalitzin SN, et al. Each year, sudden unexpected death in epilepsy (SUDEP) occurs in about 1 out of every 1,000 people diagnosed with epilepsy. 2019 Sep;98(Pt A):266-272. doi: 10.1016/j.yebeh.2019.07.003. Ann Neurol 2021;89(5):1023-35. Online ahead of print. and transmitted securely. Dibu M, Spoor JKH, Dremmen M, von Sa CF, Hnggi D, Steiger HJ, Ryvlin P, Kamp MA. Herlenius E, Lagercrantz H, Yamamoto Y. Adenosine modulates inspiratory neurons and the respiratory pattern in the brainstem of neonatal rats. Postictal generalized electroencephalographic suppression is not related to total generalized convulsive seizures duration (101; 02) but is associated with longer tonic phase duration and with the presence of bilateral tonic symmetric extensor posturing (decerebration) (111; 02; 84). Nashef L, Walker F, Allen P, Sander JW, Shorvon SD, Fish DR. Apnoea and bradycardia during epileptic seizures: relation to sudden death in epilepsy. Jeff Buchhalter, MD, PhD, talks about the ketogenic diet as a treatment for seizures and how its use might reduce the risk for SUDEP. We acknowledge the Traditional Owners of the land where we work and live. Epub 2019 Aug 10. 1 Most, but not all, cases of SUDEP happen during or right after a seizure. There are known risk factors which increase the chance of death in people with epilepsy. Terminal asystole was always preceded by terminal apnea, suggesting apnea as the final mechanism of death, in line with previous animal models of sudden unexplained death in epilepsy (47; 94). Bethesda, MD 20894, Web Policies [4], SUDEP occurs in about 1 in 1,000 adults and 1 in 4,500 children with epilepsy a year. SUDEP is not well understood. Premortem use of MRI to determine sudden unexplained death in epilepsy risk is currently not recommended or utilized. There may be obvious signs a seizure has happened, though this isnt always the case. Epilepsy, defined as the tendency for unprovoked seizures, is a common neurologic disease affecting up to 1 in 26 individuals. SUDEP in the North American SUDEP Registry: the full spectrum of epilepsies. Lacuey N, Zonjy B, Hampson JP, et al. Epilepsy affects 6.38 per 1000 persons (95% CI 5.57-7.30), with a lifetime prevalence of 7.60 per 1000 persons (95% CI 6.1-9.38) (34). A very rare but serious complication of poorly controlled epilepsy is sudden unexplained death with epilepsy (SUDEP), which may occur more frequently in pregnancy. Compared with sharing a bedroom, living alone or sharing a household but not a bedroom are associated with an increased risk for sudden unexplained death in epilepsy, even after adjustment for generalized convulsive seizures frequency (107). These findings may represent the pathological basis for cardiorespiratory dysfunction in sudden unexplained death in epilepsy patients (73; 88; 87). We pay our respects to Elders past, present and emerging. Front Neurol. Sudden unexpected death in epilepsy (SUDEP) refers to the sudden death of a seemingly healthy individual with epilepsy, usually occurring during, or immediately after, a tonic-clonic It is associated with subtherapeutic postmortem serum antiepileptic drug (AED) levels Lacuey N, Hampson JP, Theeranaew W, et al. Epilepsy Behav Rep 2019;12:100333. Long QT syndrome: genetics and future perspective. Research suggests that 1 in 3,000 CYE will die of SUDEP annually, Guidelines summarize and evaluate available evidence with the aim of assisting health professionals in proposing the best management strategies for an individua 2018 Mar;8(1):119-135. doi: 10.23907/2018.009. Download our "Knowledge is Power" flyer for help seeking the best care and knowing what questions to ask your health care provider about your SUDEP risk. Four children (80%) but only one adult (3%) had fully therapeutic postmortem AED levels. In SUDEP cases, no other cause of death is found when an autopsy is done. Sudden unexpected death in epilepsy (SUDEP) has recently emerged as an important cause of death in people with epilepsy. Tanaka H, Katsuragi S, Hasegawa J, Tanaka K, Nakamura M, Hayata E, Nakata M, Sekizawa A, Ishiwata I, Ikeda T. Brain Sci. According to a combined analysis of case control studies, patients with generalized convulsive seizures have a 10-fold increased risk of sudden unexplained death in epilepsy compared to those without generalized convulsive seizures (41; 39). Epilepsy is a common condition in which the brain is affected causing seizures . Arch Dis Child 1983;58(2):151-3. Every article is reviewed by our esteemed Editorial Board for accuracy and currency. Careers. However, after adjusting individual antiepileptic drugs and number of antiepileptic drugs for generalized convulsive seizures frequency, they did not find increased risk, reinforcing current evidence that generalized convulsive seizures frequency is the strongest risk factor for sudden unexplained death in epilepsy (42; 96; 128; 107). The overall SUDEP rate (definite/ highly probable SUDEP and possible SUDEP combined) was calculated to be 3.5 in 1,000 patient-years of exposure to LTG. Sveinsson O, Andersson T, Carlsson S, Tomson T. Circumstances of SUDEP: a nationwide population-based case series. However, prolonged ictal central apnea can occur, is associated with severe hypoxemia, and may pose increased risk of death (57; 121). What seems apparent, however, is that postictal generalized electroencephalographic suppression is frequently found associated with other markers of seizure severity. When a child or adolescent with epilepsy dies, and their death is not caused by another illness or injury, this is called Sudden Unexpected Death in Epilepsy (SUDEP). Long QT syndrome is a genetically heterogeneous cardiac channelopathy that produces abnormally prolonged repolarization of cardiomyocyte action potentials (125). Sudden Unexpected Death in Epilepsy (SUDEP) Is an almost self-explanatory term: it applies to a sudden death in someone known to have epilepsy, in the absence of an obvious cause for the death. Sudden Unexpected Death in Epilepsy. Respiratory factors: impaired respiration and seizure-induced pulmonary dysfunction as well as central, Cerebral and autonomic nervous system dysregulation: cardiac arrhythmia and respiratory failure as a result of seizure-related changes to brain function and dysfunction of the, Anti epileptic drugs: most evidence suggests that, Vagal nerve stimulation: concerns have been raised that. Patients with Dravet syndrome have decreased heart rate variability and periictal respiratory dysfunction (50). Sudden unexplained death in epilepsy refers to sudden death in persons with epilepsy in whom postmortem examination is negative for toxicological or pathological causes of death (78). Nonseizure SUDEP: sudden unexpected death in epilepsy without preceding epileptic seizures. SUDEP is the number one cause of epilepsy-related death in people with pharmacoresistant epilepsy. SUDEP, or Sudden Unexpected Death In Epilepsy refers to when an otherwise healthy person with epilepsy dies, and there is no connection to any other cause of death, such as an accident, A mutation in the SCN1A gene, which encodes for the alpha subunit of the voltage-gated sodium channel, has been found in 70% to 80% of the cases, mainly occurring de novo, although mutations in other genes have been described (20; 70). Paroxysmal hypertension during a complex partial seizure. Ictal asystole has been proposed as benign and self-limiting, even as a mechanism of seizure termination, because asystole-induced reduction in cerebral blood flow potentially aborts perpetuation of the seizure discharge (98). Arrhythmia in heart and brain: KCNQ1 mutations link epilepsy and sudden unexplained death. Most sudden unexplained death in epilepsy cases occur at night, following generalized convulsive seizures, although sudden unexplained death in epilepsy without preceding seizure can occur, suggesting sudden unexplained death in epilepsy is a heterogeneous phenomenon. Pulmonary edema has also been described after seizures in living epilepsy patients based on bilateral edema on chest x-ray following generalized convulsive seizures (49). Neurology 2012;78(23):1868-76. 3540 Crain Highway, Suite 675,Bowie, MD 20716, 2022 Epilepsy Foundation, is a non-profit organization with a 501(c)(3) tax-exempt status. Breathing dysfunction. 2021 Jul 28;11(8):995. doi: 10.3390/brainsci11080995. Histopathologic studies of sudden unexplained death in epilepsy patients have shown focal gliosis within amygdaloid subnuclei as well as an increased right parahippocampal volume, in concordance with neuroimaging studies (114; 126; 103). AV conduction blocks, atrial flutter/fibrillation, and ventricular tachycardia/fibrillation have also been reported (119). Some families have found monitors to be a useful part of a risk reduction plan. Evidences for both cardiovascular and respiratory dysfunction have been reported and are reviewed below.

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