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Unifying the definitions of sudden unexpected death in epilepsy
Open accessAbstract
Summary: Sudden unexpected death in epilepsy (SUDEP) is a category of death in people with epilepsy occurring in the absence of a known structural cause of death and is most likely heterogeneous with regard to mechanisms and circumstances. SUDEP is particularly difficult to investigate in research studies for several reasons, including its relatively low incidence, its unpredictable occurrence often in unwitnessed settings, and its low rate of complete autopsy examinations. Over the past two decades, two complementary definitions have been used in most SUDEP studies, but often with variations. We propose here a unified SUDEP definition and classification to resolve current ambiguities and to retrieve cases that would not have been further studied if the previous definitions were used. The proposed Unified SUDEP Definition and Classification contains, in addition to concepts inherent in the previous definitions, nine main recommendations. (1) The word “unexpected,” and not the word “unexplained,” should be uniformly used in the term SUDEP . (2) The SUDEP category should be applied when appropriate, whether or not a terminal seizure is known to have occurred. (3) The “Possible SUDEP” category should be used only for cases with competing causes of death, with cases left unclassified when data are insufficient to reasonably permit their classification. (4) Cases that would otherwise fulfill the definition of SUDEP should be designated as “SUDEP Plus” when evidence indicates that a preexisting condition, known before or after autopsy, could have contributed to the death, which otherwise is classified as SUDEP (e.g., coronary insufficiency with no evidence of myocardial infarction or long‐QT syndrome with no documented primary ventricular arrhythmia leading to death). (5) To be considered SUDEP, the death should have occurred within 1 h from the onset of a known terminal event. (6) For status epilepticus as an exclusion criterion for SUDEP, the duration of seizure activity should be 30 min or more. (7) A specific category of SUDEP due to asphyxia should not be designated, the distinction being largely impractical on circumstantial or autopsy evidence, with more than one mechanism likely to be contributory in many cases. (8) Death occurring in water but without circumstantial or autopsy evidence of submersion should be classified as “Possible SUDEP.” If any evidence of submersion is present, the death should not be classified as SUDEP. (9) A category of “Near‐SUDEP” should be agreed to include cases in which cardiorespiratory arrest was reversed by resuscitation efforts with subsequent survival for more than 1 h. Scenarios that demonstrate the basis for each SUDEP category are described. If disagreement exists about which category fits a particular case, we suggest the use of consensus decision by a panel of informed reviewers to adjudicate the classification of the case.
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“The primary cause of death is sudden unexpected death in epilepsy (SUDEP) 17 ; other causes include cerebral oedema following status epilepticus 18 , chest infections and accidental causes 19 .”
From Developmental and epileptic encephalopathies (2024) · cites this paper as [17]
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Cites
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Sorted by citations
Thijs, R. D., Surges, R., O'Brien, T. J., & Sander, J. W. A. S. (2019) · The Lancet · doi:10.1016/s0140-6736(18)32596-0 · Cited by 2,359
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Self-citationSudden unexpected death in epilepsy: epidemiology, mechanisms, and prevention
Devinsky, O., Hesdorffer, D. C., Thurman, D. J., Lhatoo, S. D., & Richerson, G. B. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(16)30158-2 · Cited by 743
The consequences of refractory epilepsy and its treatment
Laxer, K. D., Trinka, E., Hirsch, L. J., Cendes, F., Langfitt, J. T., Delanty, N., Resnick, T., & Benbadis, S. R. (2014) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2014.05.031 · Cited by 681
“Sudden unexpected death in epilepsy is defined as “sudden, unexpected, witnessed or unwitnessed, nontraumatic, and nondrowning death, occurring in benign circumstances, in an individual with epilepsy, with or without evidence of a preceding seizure and excluding documented status epilepticus [72].”
BackgroundSudden unexpected death in epilepsy
Shorvon, S. D., & Tomson, T. (2011) · The Lancet · doi:10.1016/s0140-6736(11)60176-1 · Cited by 510
Self-citationThe Human Phenotype Ontology in 2024: phenotypes around the world
Gargano, M. A., Matentzoglu, N., Coleman, B., Addo-Lartey, E. B., Anagnostopoulos, A. V., Anderton, J., Avillach, P., Bagley, A. M., Bakštein, E., Balhoff, J. P., et al. (2024) · Nucleic Acids Research · doi:10.1093/nar/gkad1005 · Cited by 410
“Sudden unexpected death in epilepsy (SUDEP), an unexpected non-traumatic and non-drowning death in an otherwise healthy individual with epilepsy, has been an important addition to the HPO (HP:0033258) ( 18 ).”
BackgroundRecognizing and preventing epilepsy-related mortality
Devinsky, O., Spruill, T. M., Thurman, D. J., & Friedman, D. J. (2015) · Neurology · doi:10.1212/wnl.0000000000002253 · Cited by 327
“1 Because the incidence of epilepsy is greatest among the young, SUDEP, along with status epilepticus, accidents, drownings, and suicide, the most common epilepsy-related causes of death, together lead to substantial premature mortality.”
BackgroundInfluentialCooper, M. S., McIntosh, A. M., Crompton, D. E., McMahon, J. M., Schneider, A., Farrell, K., Ganesan, V., Gill, D. S., Kivity, S., Lerman‐Sagie, T., et al. (2016) · Epilepsy Research · doi:10.1016/j.eplepsyres.2016.10.006 · Cited by 323
Sveinsson, O. A., Andersson, T., Carlsson, S., & Tomson, T. (2017) · Neurology · doi:10.1212/wnl.0000000000004094 · Cited by 300
“30,274/27,501 (52/48) 1,055/835 (56/44) 42/26 (62/38) 43/26 (62/38) Age, y, median (range) 46 (3–106) Not applicable Not applicable Not applicable Age at death, y, median (range) Not applicable 78 (4–100) 50 (4–88) 50 (4–88) Psychiatric comorbidity, n (%)c 16,821 (29) 774 (41) 32 (47) 32 (46) Antiepileptic drug treatm…”
BackgroundSelf-citationSudden unexpected death in a mouse model of Dravet syndrome
Kalume, F. K., Westenbroek, R. E., Cheah, C. S., Yu, F. H., Oakley, J. C., Scheuer, T., & Catterall, W. A. (2013) · Journal of Clinical Investigation · doi:10.1172/jci66220 · Cited by 286
“Status epilepticus, defined as a single seizure or series of recurring seizures lasting more than 30 minutes (26), is an exclusion criterion for the definition of SUDEP in patients (27, 28).”
BackgroundBagnall, R. D., Crompton, D. E., Petrovski, S., Lam, L., Cutmore, C., Garry, S. I., Sadleir, L. G., Dibbens, L. M., Cairns, A. G., Kivity, S., et al. (2015) · Annals of Neurology · doi:10.1002/ana.24596 · Cited by 289
“SUDEP cases were classified as definite SUDEP, definite SUDEP plus, probable SUDEP, or possible SUDEP.(2) The human research ethics committees of Austin Health (Melbourne, Australia), Tel Aviv Sourasky Medical Center (Tel Aviv, Israel), and Royal Prince Alfred Hospital (Sydney, Australia) approved the study.”
BackgroundSCN1A‐related phenotypes: Epilepsy and beyond
Scheffer, I. E., & Nabbout, R. (2019) · Epilepsia · doi:10.1111/epi.16386 · Cited by 245
The landscape of epilepsy-related GATOR1 variants
Baldassari, S., Picard, F., Verbeek, N. E., van Kempen, M. J. A., Brilstra, E. H., Lesca, G., Conti, V., Guerrini, R., Bisulli, F., Licchetta, L., et al. (2018) · Genetics in Medicine · doi:10.1038/s41436-018-0060-2 · Cited by 251
Sodium Channel β Subunits: Emerging Targets in Channelopathies
O’Malley, H. A., & Isom, L. L. (2015) · Annual Review of Physiology · doi:10.1146/annurev-physiol-021014-071846 · Cited by 247
“SUDEP is defined as sudden death that is not a result of trauma or drowning, does not result from status epilepticus, and in which post-mortem examination does not uncover a toxicological or anatomical cause (66).”
BackgroundBreathing Inhibited When Seizures Spread to the Amygdala and upon Amygdala Stimulation
Dlouhy, B. J., Gehlbach, B. K., Kreple, C. J., Kawasaki, H., Oya, H., Buzza, C., Granner, M. A., Welsh, M. J., Howard, M. A., Wemmie, J. A., et al. (2015) · Journal of Neuroscience · doi:10.1523/jneurosci.0888-15.2015 · Cited by 231
“Death is not caused by trauma, drowning, or status epilepticus, and the cause remains unknown even after autopsy (Nashef et al., 2012).”
BackgroundSUDEP and epilepsy‐related mortality in pregnancy
Edey, S., Moran, N. F., & Nashef, L. A. M. (2014) · Epilepsia · doi:10.1111/epi.12621 · Cited by 203
Self-citationMortality in Dravet syndrome: A review
Shmuely, S., Sisodiya, S. M., Gunning, W. B., Sander, J. W. A. S., & Thijs, R. D. (2016) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2016.09.007 · Cited by 186
Involvement of GATOR complex genes in familial focal epilepsies and focal cortical dysplasia
Weckhuysen, S., Marsan, E., Lambrecq, V., Marchal, C., Morin‐Brureau, M., An-Gourfinkel, I., Baulac, M., Fohlen, M., Zetchi, C. K., Seeck, M., et al. (2016) · Epilepsia · doi:10.1111/epi.13391 · Cited by 181
“One individual (F/III-1) with focal epilepsy died of probable SUDEP.”
ResultInfluentialPostconvulsive central apnea as a biomarker for sudden unexpected death in epilepsy (SUDEP)
Vilella, L., Lacuey, N., Hampson, J. P., Rani, M. R. S., Sainju, R. K., Friedman, D. J., Nei, M., Strohl, K. P., Scott, C. A., Gehlbach, B. K., et al. (2018) · Neurology · doi:10.1212/wnl.0000000000006785 · Cited by 181
Self-citationLevira, F., Thurman, D. J., Sander, J. W., Hauser, W. A., Hesdorffer, D. C., Masanja, H., Odermatt, P., Logroscino, G., Newton, C. R., & the Epidemiology Commission of the International League Against Epilepsy (2017) · Epilepsia · doi:10.1111/epi.13603 · Cited by 197
“The definitions and classification of SUDEP are mainly consistent with the earlier recommendations,(20) which have been updated since publication of most the studies we reviewed.(21)”
Serotonin neurones have anti‐convulsant effects and reduce seizure‐induced mortality
Buchanan, G. F., Murray, N. M., Hajek, M. A., & Richerson, G. B. (2014) · The Journal of Physiology · doi:10.1113/jphysiol.2014.277574 · Cited by 172
“The current definition of SUDEP allows for death to occur without evidence of an associated seizure (Nashef et al. 2012).”
BackgroundThe Epileptic Heart: Concept and clinical evidence
Verrier, R. L., Pang, T. D., Nearing, B. D., & Schachter, S. C. (2020) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2020.106946 · Cited by 169
“The definition of sudden unexpected death in epilepsy (SUDEP) by the International League Against Epilepsy is “a sudden, unexpected death in a person with epilepsy, with or without evidence of a seizure preceding the death, in which there is no evidence of other disease, injury, or drowning that caused the death” [5].”
BackgroundMortality Risks in New-Onset Childhood Epilepsy
Berg, A. T., Nickels, K. C., Wirrell, E. C., Geerts, A. T., Callenbach, P. M. C., ARTS, W. F. M., Rios, C. R., CAMFIELD, P. R., & Camfield, C. (2013) · PEDIATRICS · doi:10.1542/peds.2012-3998 · Cited by 161
Myers, K. A., Bello‐Espinosa, L. E., Symonds, J. D., Zuberi, S. M., Clegg, R., Sadleir, L. G., Buchhalter, J., & Scheffer, I. E. (2018) · Epilepsia · doi:10.1111/epi.14438 · Cited by 151
SUDEP in the North American SUDEP Registry
Verducci, C., Hussain, F., Donner, E. J., Moseley, B. D., Buchhalter, J., Hesdorffer, D. C., Friedman, D. J., & Devinsky, O. (2019) · Neurology · doi:10.1212/wnl.0000000000007778 · Cited by 137
Nonseizure SUDEP: Sudden unexpected death in epilepsy without preceding epileptic seizures
Lhatoo, S. D., Nei, M., Raghavan, M., Sperling, M. R., Zonjy, B., Lacuey, N., & Devinsky, O. (2016) · Epilepsia · doi:10.1111/epi.13419 · Cited by 134
“Deaths in patients with epilepsy occurring >1 h after a known terminal event, interrupted by temporarily successful cardiopulmonary resuscitation (CPR), are classified as near- Epilepsia, 57(7):1161–1168, 2016 doi: 10.1111/epi.13419 SUDEP.9 Because two of the three cases presented succumbed in this fashion after what…”
BackgroundInfluentialDevelopmental and epileptic encephalopathies
Scheffer, I. E., Zuberi, S., Mefford, H. C., Guerrini, R., & McTague, A. (2024) · Nature Reviews Disease Primers · doi:10.1038/s41572-024-00546-6 · Cited by 158
“Different genetic DEEs vary considerably in their SUDEP risk, which is increased in DEEs due to sodium channelopathies 19 .”
InfluentialIncidence of sudden unexpected death in epilepsy in children is similar to adults
Keller, A. E., Whitney, R., Li, S., Pollanen, M. S., & Donner, E. J. (2018) · Neurology · doi:10.1212/wnl.0000000000005762 · Cited by 128
“Two child neurologists (E.J.D., R.W.) independently classified cases according to criteria described by Nashef et al.3 Discrepancies were discussed until consensus was reached.”
BackgroundMethodInfluentialMortality in patients with psychogenic nonepileptic seizures
Nightscales, R., McCartney, L., Auvrez, C., Tao, G., Barnard, S. N., Malpas, C. B., Perucca, P., McIntosh, A. M., Chen, Z. Z., Sivathamboo, S., et al. (2020) · Neurology · doi:10.1212/wnl.0000000000009855 · Cited by 128
“For patients diagnosed with epilepsy or both epilepsy and PNES, SUDEP was classified according to established criteria.(23) Cases of “definite,” “probable,” or “near” SUDEP or SUDEP plus or “possible SUDEP” were determined by 2 epileptologists (T.”
MethodStructural imaging biomarkers of sudden unexpected death in epilepsy
Wandschneider, B., Koepp, M. J., Scott, C. A., Micallef, C., Balestrini, S., Sisodiya, S. M., Thom, M., Harper, R. M., Sander, J. W. A. S., Vos, S. B., et al. (2015) · Brain · doi:10.1093/brain/awv233 · Cited by 127
“Characteristics of subjects who died suddenly Those deceased were classified as probable (n = 10) or definite (n = 2) SUDEP, according to a recent classification (Nashef et al., 2012).”
MethodTomson, T., Surges, R., Delamont, R. S., Haywood, S., & Hesdorffer, D. C. (2016) · Epilepsia · doi:10.1111/epi.13234 · Cited by 126
“A proposal for unified definitions of SUDEP was recently made.(4) Most case–control studies include definite as well as probable SUDEP in different proportions.”
BackgroundSelf-citationChahal, C., Salloum, M. N., Alahdab, F., Gottwald, J. A., Tester, D., Anwer, L. A., So, E. L., Murad, M., Louis, E. S. S., Ackerman, M., et al. (2019) · Journal of the American Heart Association : Cardiovascular and Cerebrovascular Disease · doi:10.1161/jaha.119.012264 · Cited by 109
“6) as per Nashef et al (Table 2).(1) In brief, SUDEP is categorized per the following criteria: (1) definite if an autopsy does not reveal a cause of death (including patients with a structurally normal heart); (2) definite-plus if an autopsy was performed and there may be an alternate contributing factor; (3) probabl…”
BackgroundSelf-citationBrainstem spreading depolarization and cortical dynamics during fatal seizures in Cacna1a S218L mice
Loonen, I. C. M., Jansen, N. A., Cain, S. M., Schenke, M., Voskuyl, R. A., Yung, A. C., Bohnet, B., Kozłowski, P., Thijs, R. D., Ferrari, M. D., et al. (2018) · Brain · doi:10.1093/brain/awy325 · Cited by 120
Autonomic manifestations of epilepsy: emerging pathways to sudden death?
Thijs, R. D., Ryvlin, P., & Surges, R. (2021) · Nature Reviews Neurology · doi:10.1038/s41582-021-00574-w · Cited by 111
Self-citationEpilepsy Is a Risk Factor for Sudden Cardiac Arrest in the General Population
Bardai, A., Lamberts, R. J., Blom, M. T., Spanjaart, A. M., Berdowski, J., van der Staal, S. R., Brouwer, H. J., Koster, R. W., Sander, J. W. A. S., Thijs, R. D., et al. (2012) · PLoS ONE · doi:10.1371/journal.pone.0042749 · Cited by 112
“Case-control studies, eyewitness accounts, and ictal recordings suggest that SUDEP is a peri-ictal event likely triggered by a convulsive seizure [7].”
BackgroundInfluentialJeppesen, J., Fuglsang‐Frederiksen, A., Brugada, R., Pedersen, B., Rubboli, G., Johansen, P., & Beniczky, S. (2014) · Epilepsia · doi:10.1111/epi.12614 · Cited by 111
Voltage-Gated Sodium Channel β Subunits and Their Related Diseases
Bouza, A. A., & Isom, L. L. (2017) · Handbook of experimental pharmacology · doi:10.1007/164_2017_48 · Cited by 111
“SUDEP is defined as the sudden and unexpected death of a person with epilepsy without any identifiable cause of death during autopsy (Nashef et al. 2012).”
BackgroundThe ventrolateral medulla and medullary raphe in sudden unexpected death in epilepsy
Patodia, S., Somani, A., O’Hare, M., Venkateswaran, R., Liu, J. Y. W., Michalak, Z., Ellis, M. J., Scheffer, I. E., Diehl, B., Sisodiya, S. M., et al. (2018) · Brain · doi:10.1093/brain/awy078 · Cited by 110
“These were further categorized into nine definite SUDEP (complete and negative autopsy including toxicology), the remaining five being probable or possible SUDEP (incomplete autopsy examination or competing cause of death identified) (Nashef et al., 2012); (ii) Seven cases with Dravet syndrome obtained from ESBTB and…”
MethodSudden unexpected death in epilepsy (SUDEP): what every neurologist should know
Shankar, R., Donner, E. J., McLean, B., Nashef, L. A. M., & Tomson, T. (2017) · Epileptic Disorders · doi:10.1684/epd.2017.0891 · Cited by 109
Self-citationHeart rate variability measurement in epilepsy: How can we move from research to clinical practice?
Myers, K. A., Sivathamboo, S., & Perucca, P. (2018) · Epilepsia · doi:10.1111/epi.14587 · Cited by 109
Non-EEG seizure-detection systems and potential SUDEP prevention: State of the art
de Vel, A. V., Cuppens, K., Bonroy, B., Milošević, M., Jansen, K., Huffel, S. V., Vanrumste, B., Lagae, L., & Ceulemans, B. (2013) · Seizure · doi:10.1016/j.seizure.2013.02.012 · Cited by 109
Sudden Unexpected Death in Epilepsy
Hesdorffer, D. C., & Tomson, T. (2012) · CNS Drugs · doi:10.1007/s40263-012-0006-1 · Cited by 98
“Etiologia objawowa padaczki jest również związana z większym ryzykiem zgonu(10,11).”
Self-citationDonnan, A. M., Schneider, A. L., Russ-Hall, S., Churilov, L., & Scheffer, I. E. (2023) · Neurology · doi:10.1212/wnl.0000000000207080 · Cited by 97
Sudden unexpected death in epilepsy: The neuro-cardio-respiratory connection
Manolis, T. A., Manolis, A. S., Melita, H., & Manolis, A. S. (2018) · Seizure · doi:10.1016/j.seizure.2018.12.007 · Cited by 97
Genetic Basis of Sudden Unexpected Death in Epilepsy
Bagnall, R. D., Crompton, D. E., & Semsarian, C. (2017) · Frontiers in Neurology · doi:10.3389/fneur.2017.00348 · Cited by 94
“The most common cause of death that is related to epilepsy is sudden unexpected death in epilepsy (SUDEP), defined as “a sudden, unexpected, witnessed or unwitnessed, non-traumatic, and non-drowning death in patients with epilepsy with or without evidence for a seizure, and excluding documented status epilepticus, in…”
BackgroundInfluentialThe brain–heart interaction in epilepsy: implications for diagnosis, therapy, and SUDEP prevention
Costagliola, G., Orsini, A. A., Coll, M., Brugada, R., Parisi, P., & Striano, P. (2021) · Annals of Clinical and Translational Neurology · doi:10.1002/acn3.51382 · Cited by 93
“It is defined as the sudden, unexpected death in people with epilepsy independently of the evidence of a seizure when trauma, drowning, and status epilepticus have been excluded, and postmortem examination does not identify a cause of death.(2) SUDEP occurs in about 1.”
BackgroundAssociation of sleep with sudden unexpected death in epilepsy
Ali, A., Wu, S., Issa, N. P., Rose, S., Towle, V. L., Warnke, P. C., & Tao, J. X. (2017) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2017.08.021 · Cited by 92
“When there was an alternative cause of death, SUDEP was considered possible [10].”
Serotonin and sudden unexpected death in epilepsy
Petrucci, A. N., Joyal, K. G., Purnell, B. S., & Buchanan, G. F. (2019) · Experimental Neurology · doi:10.1016/j.expneurol.2019.113145 · Cited by 93
Tao, J. X., Yung, I., Lee, A. J., Rose, S., Jacobsen, J., & Ebersole, J. S. (2013) · Epilepsia · doi:10.1111/epi.12094 · Cited by 91
“SUDEP is defined as sudden, unexpected, nontraumatic, and nondrowning death in absence of documented status epilepticus or an identifiable anatomic or toxicologic cause for death at autopsy (Nashef, 1997; Nashef et al., 2012).”
BackgroundImpact of fenfluramine on the expected SUDEP mortality rates in patients with Dravet syndrome
Cross, J. H., Galer, B. S., Gil‐Nagel, A., Devinsky, O., Ceulemans, B., Lagae, L. G., Schoonjans, A., Donner, E. J., Wirrell, E. C., Kothare, S. V., et al. (2021) · Seizure · doi:10.1016/j.seizure.2021.10.024 · Cited by 87
Genome-wide Polygenic Burden of Rare Deleterious Variants in Sudden Unexpected Death in Epilepsy
Leu, C., Balestrini, S., Maher, B. H., Hernández-Hernández, L., Gormley, P., Hämäläinen, E., Heggeli, K., Schoeler, N. E., Nový, J., Willis, J., et al. (2015) · EBioMedicine · doi:10.1016/j.ebiom.2015.07.005 · Cited by 85
“The cause of deathwas classified into definite, probable, or near-SUDEP, according to the most recent proposed system: definite SUDEP required post mortem examination, without an identified toxicological or anatomical cause of death (Nashef et al., 2012).”
BackgroundSelf-citationGenomic biomarkers of SUDEP in brain and heart
Glasscock, E. (2013) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2013.09.019 · Cited by 85
“Such deaths are usually unwitnessed and by definition post-mortem examination fails to identify an obvious toxicological or anatomical culprit [3].”
BackgroundGenetic investigation of sudden unexpected death in epilepsy cohort by panel target resequencing
Coll, M., Allegue, C., Partemi, S., Matés, J., del Olmo, B., Campuzano, Ó., Pascali, V. L., Iglesias, A., Striano, P., Oliva, A., et al. (2015) · International Journal of Legal Medicine · doi:10.1007/s00414-015-1269-0 · Cited by 83
Cerebellar, limbic, and midbrain volume alterations in sudden unexpected death in epilepsy
Allen, L. A., Vos, S. B., Kumar, R., Ogren, J. A., Harper, R. K., Winston, G. P., Balestrini, S., Wandschneider, B., Scott, C. A., Sebastien Ourselin, A., et al. (2019) · Epilepsia · doi:10.1111/epi.14689 · Cited by 83
Underestimation of sudden deaths among patients with seizures and epilepsy
Devinsky, O., Friedman, D. J., Cheng, J. Y., Moffatt, E., Kim, A., & Tseng, Z. H. (2017) · Neurology · doi:10.1212/wnl.0000000000004292 · Cited by 83
“SUDEP was determined using the criteria of Nashef et al. 6 Definite SUDEP was a sudden death in benign circumstances (excluding status epilepticus) in a person with epilepsy without an alternative COD on postmortem examination.”
MethodNocturnal supervision and SUDEP risk at different epilepsy care settings
van der Lende, M., Hesdorffer, D. C., Sander, J. W. A. S., & Thijs, R. D. (2018) · Neurology · doi:10.1212/wnl.0000000000006356 · Cited by 81
“and classified as non-SUDEP deaths or as possible, probable, and definite SUDEP according to the Unified SUDEP definitions.(14) As an additional criterion to portray the diagnosis of “probable SUDEP,” we developed a point score based on established SUDEP circumstances.”
MethodEpileptic heart: A clinical syndromic approach
Verrier, R. L., Pang, T. D., Nearing, B. D., & Schachter, S. C. (2021) · Epilepsia · doi:10.1111/epi.16966 · Cited by 80
Thom, M., Michalak, Z., Wright, G., Dawson, T., Hilton, D. A., Joshi, A., Diehl, B., Koepp, M. J., Lhatoo, S. D., Sander, J. W. A. S., et al. (2015) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12265 · Cited by 79
Voltage-gated sodium channel β subunits: The power outside the pore in brain development and disease
Hull, J. M., & Isom, L. L. (2017) · Neuropharmacology · doi:10.1016/j.neuropharm.2017.09.018 · Cited by 79
“Sudden Unexpected Death in Epilepsy (SUDEP) is defined as death in patients with epilepsy that is not due to status epilepticus, trauma or drowning (Nashef et al., 2012).”
Backgroundvan Andel, J. M., Thijs, R. D., de Weerd, A., Arends, J. B. A. M., & Leijten, F. S. S. (2016) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2016.01.003 · Cited by 78
“(seizure duration ≥30 min or seizures without recovery in between), in which postmortem examination does not reveal a cause of death” [36].”
SUDEP and other causes of mortality in childhood-onset epilepsy
Sillanpää, M. L., & Shinnar, S. (2013) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2013.04.016 · Cited by 78
“When one uses the Nashef criteria [26,27], which are becoming more accepted as it becomes clear that SUDEP most likely does occur in the context of a brief though unwitnessed seizure [35–40], the magnitude of the problem becomes clear.”
A systematic review of sudden unexpected death in epilepsy (SUDEP) in childhood
Abdel‐Mannan, O., Taylor, H. M., Donner, E. J., & Sutcliffe, A. G. (2018) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2018.11.006 · Cited by 77
Sudden unexpected death in epilepsy (SUDEP): What do patients think?
Xu, Z., Ayyappan, S., & Seneviratne, U. K. (2014) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2014.11.007 · Cited by 76
“Definite SUDEP is defined as “sudden, unexpected, witnessed or unwitnessed, nontraumatic and nondrowning death, occurring in benign circumstances, in an individualwith epilepsy, with or without evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a cause o…”
BackgroundThe lifelong course of chronic epilepsy: the Chalfont experience
Nový, J., Belluzzo, M., Caboclo, L. O. S. F., Catarino, C. B., Yogarajah, M., Martinian, L., Peacock, J. L., Bell, G. S., Koepp, M. J., Thom, M., et al. (2013) · Brain · doi:10.1093/brain/awt117 · Cited by 74
“Sudden unexpected death in epilepsy (SUDEP) was defined as death in which no cause was found at autopsy (i.e. requiring post-mortem examination), occurring in benign conditions, without evidence of status epilepticus or traumatic event (Nashef et al., 2012).”
BackgroundSudden cardiac arrest in people with epilepsy in the community: Circumstances and risk factors
Lamberts, R. J., Blom, M. T., Wassenaar, M., Bardai, A., Leijten, F. S., de Haan, G.-J., Sander, J. W., Thijs, R. D., & Tan, H. L. (2015) · Neurology · doi:10.1212/wnl.0000000000001755 · Cited by 72
“SUDEP was defined as sudden, unexpected, witnessed or unwitnessed, nontraumatic and nondrowning death, occurring in benign circumstances, in an individual with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus (seizure duration $30 minutes or seizures without recovery in betw…”
BackgroundMortality and SUDEP in epilepsy patients treated with vagus nerve stimulation
Granbichler, C. A., Nashef, L. A. M., Selway, R., & Polkey, C. E. (2015) · Epilepsia · doi:10.1111/epi.12888 · Cited by 73
“By 2013 these devices had been implanted in >65,000 epilepsy patients worldwide.4 It has been shown to reduce the frequency and intensity of seizures, but the exact mechanism of action remains unclear.5 In this audit we aimed to analyze overall mortality over time in a hospital-based cohort of patients with refractory…”
MethodInfluentialSelf-citationStrzelczyk, A., Zschebek, G., Bauer, S., Baumgartner, C., Grond, M., Hermsen, A., Kieslich, M., Krämer, G., Kurlemann, G., May, T. W., et al. (2016) · Epilepsia · doi:10.1111/epi.13337 · Cited by 73
Self-citationWillems, L. M., Watermann, N., Richter, S., Kay, L., Hermsen, A., Knake, S., Rosenow, F., & Strzelczyk, A. (2018) · Frontiers in Neurology · doi:10.3389/fneur.2018.00414 · Cited by 73
Autonomic aspects of sudden unexpected death in epilepsy (SUDEP)
Barot, N., & Nei, M. (2018) · Clinical Autonomic Research · doi:10.1007/s10286-018-0576-1 · Cited by 71
Research conference summary from the 2014 International Task Force on ATP1A3 -Related Disorders
Rosewich, H., Sweney, M., DeBrosse, S. D., Ess, K. C., Ozelius, L. J., Andermann, E., Andermann, F., Andrasco, G., Belgrade, A., Brashear, A., et al. (2017) · Neurology Genetics · doi:10.1212/nxg.0000000000000139 · Cited by 71
“org/ng.(26) Proposed risk factors include the use of multiple anticonvulsants, poor medication compliance, uncontrolled seizures, and the presence of additional neurologic deficits, in addition to epilepsy, among others.”
BackgroundSCN 8A mutations in Chinese children with early onset epilepsy and intellectual disability
Kong, W., Zhang, Y., Gao, Y., Liu, X., Gao, K., Xie, H., Wang, J., Wu, Y., Zhang, Y., Wu, X., et al. (2015) · Epilepsia · doi:10.1111/epi.12925 · Cited by 70
Pulmonary and cardiac pathology in sudden unexpected death in epilepsy (SUDEP)
Nascimento, F. A., Tseng, Z. H., Palmière, C., Maleszewski, J. J., Shiomi, T., McCrillis, A. M., & Devinsky, O. (2017) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2017.05.013 · Cited by 70
Hesdorffer, D. C., Crandall, L. A., Friedman, D., & Devinsky, O. (2015) · Epilepsia · doi:10.1111/epi.13066 · Cited by 68
“Similar circumstances of death also occur in sudden unexplained infant death (SUID)/SIDS,(10) sudden unexpected death in epilepsy (SUDEP), and in some deaths labeled as undetermined.(11) In SUDEP, a seizure during sleep may trigger the pathophysiologic cascade leading to death,(12) with most deaths unwitnessed and wit…”
BackgroundIncidence rate of sudden death in epilepsy: A systematic review and meta-analysis
Sætre, E., & Abdelnoor, M. (2018) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2018.06.037 · Cited by 67
Jansen, N. A., Schenke, M., Voskuyl, R. A., Thijs, R. D., van den Maagdenberg, A. M. J. M., & Tolner, E. A. (2019) · Journal of Neuroscience · doi:10.1523/jneurosci.1713-19.2019 · Cited by 67
“Single seizures or seizure clusters lasting 30 min were regarded as status epilepticus (Trinka et al., 2015) and, as a SUDEP exclusion criterion (Nashef et al., 2012), excluded from further analyses.”
BackgroundDead in the Night: Sleep-Wake and Time-Of-Day Influences on Sudden Unexpected Death in Epilepsy
Purnell, B. S., Thijs, R. D., & Buchanan, G. F. (2018) · Frontiers in Neurology · doi:10.3389/fneur.2018.01079 · Cited by 67
“is used for the purposes of this review unless otherwise specified (22).”
MethodSynchronization-induced spike termination in networks of bistable neurons
Uzuntarla, M., Torres, J. J., Çalım, A., & Barreto, E. (2018) · Neural Networks · doi:10.1016/j.neunet.2018.11.007 · Cited by 64
“[64] Nashef L, So EL, Ryvlin P, Tomson T.”
BackgroundPremature mortality in poor health and low income adults with epilepsy
Kaiboriboon, K., Schiltz, N. K., Bakaki, P. M., Lhatoo, S. D., & Koroukian, S. M. (2014) · Epilepsia · doi:10.1111/epi.12789 · Cited by 65
“We identified probable sudden unexpected death in epilepsy (SUDEP) as the COD in the case of unexpected death with or without evidence of seizure, excluding documented status epilepticus.(11) We used the term probable SUDEP because autopsy data were not available.”
MethodImpaired CO2-Induced Arousal in SIDS and SUDEP
Buchanan, G. F. (2019) · Trends in Neurosciences · doi:10.1016/j.tins.2019.02.002 · Cited by 65
“, an event where the victim would have likely died but survived at least for 1 h because of resuscitative efforts); sudden unexplained death in childhood (SUDC) [18] or sudden unexplained death in the young (SUDY); and near-SUDEP [2,7].”
Blood Pressure in Seizures and Epilepsy
Nass, R. D., Hampel, K. G., Elger, C. E., & Surges, R. (2019) · Frontiers in Neurology · doi:10.3389/fneur.2019.00501 · Cited by 64
“This is in line with smaller scope studies reporting similar interictal BP values in people with FBTCS and healthy controls (85) as well as in individuals with epilepsy who later died of SUDEP and two matched control groups with and without epilepsy (86).”
BackgroundInfluentialSurges, R., Shmuely, S., Dietze, C. S., Ryvlin, P., & Thijs, R. D. (2021) · Epileptic Disorders · doi:10.1684/epd.2021.1254 · Cited by 63
Self-citationThe Interaction Between Sleep and Epilepsy
Roliz, A. H., & Kothare, S. V. (2022) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-022-01219-1 · Cited by 63
Sudden unexpected death in Dravet syndrome: Respiratory and other physiological dysfunctions
Kalume, F. K. (2013) · Respiratory Physiology & Neurobiology · doi:10.1016/j.resp.2013.06.026 · Cited by 60
“Sudden unexpected death in epilepsy (SUDEP) is a category of deaths in people with epilepsy that occurs abruptly without warning or other apparent medical causes (Nashef et al., 2012).”
BackgroundKiani, R., Tyrer, F., Jesu, A. J. M., Bhaumik, S., Gangavati, S., Walker, G., Kazmi, S., & Barrett, M. (2013) · Journal of Intellectual Disability Research · doi:10.1111/jir.12047 · Cited by 62
“Given the newly published recommendations (Nashef et al. 2012) for including cases with a competing COD as a possible SUDEP, we believe that the number of cases and the SMRs we found are an underestimate.”
Friedman, D. J., Kannan, K. S., Faustin, A., Shroff, S., Thomas, C., Heguy, A., Serrano, J., Snuderl, M., & Devinsky, O. (2018) · npj Genomic Medicine · doi:10.1038/s41525-018-0048-5 · Cited by 65
“Sudden unexpected death in epilepsy (SUDEP) is a death in a previously well person with epilepsy that does not result from trauma, drowning, status epilepticus and in whom the post-mortem examination does not reveal an alternative cause of death.(3) SUDEP is perhaps the leading cause of epilepsy-related death with an…”
BackgroundMethodMishra, V., Karumuri, B. K., Gautier, N. M., Liu, R., Hutson, T. N., Vanhoof‐Villalba, S. L., VLACHOS, I. K., Iasemidis, L. D., & Glasscock, E. (2017) · Human Molecular Genetics · doi:10.1093/hmg/ddx104 · Cited by 61
Miller, W. R., Young, N., Friedman, D. J., Buelow, J. M., & Devinsky, O. (2014) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2013.12.020 · Cited by 60
“Sudden unexpected death in epilepsy includes all epilepsy-related deaths not due to trauma, drowning, status epilepticus, or other identifiable causes [1].”
BackgroundTupal, S., & Faingold, C. L. (2019) · Epilepsia · doi:10.1111/epi.14658 · Cited by 59
“A major cause for this premature mortality increase is sudden unexpected death in epilepsy (SUDEP), which occurs under benign circumstances associated with terminal seizures but excludes drowning, trauma, toxicologic effect, or status epilepticus–related deaths.(3) The lifetime risk of SUDEP among patients with epilep…”
BackgroundIdentifying and mitigating Sudden Unexpected Death in Epilepsy (SUDEP) risk factors
Watkins, L. V., Shankar, R., & Sander, J. W. A. S. (2018) · Expert Review of Neurotherapeutics · doi:10.1080/14737175.2018.1439738 · Cited by 57
“In 2012, a unified SUDEP definition with seven classifications was proposed to facilitate case definition in clinical and research work [19] (Appendix 1) in view of the variability in access to routine postmortem examinations and lack of standardized recording of findings.”
BackgroundResolving ambiguities in SUDEP classification
Devinsky, O., Bundock, E. A., Hesdorffer, D. C., Donner, E. J., Moseley, B. D., Cihan, E., Hussain, F., & Friedman, D. J. (2018) · Epilepsia · doi:10.1111/epi.14195 · Cited by 57
“…patient; found dead submersed (head face down) in lake/pool where she swam Immersion suggests drowning and excludes SUDEP Our revisions to Nashef et al. (2012) SUDEP case scenarios are in italics. aThe 60-min cutoff is an arbitrary divide between SUDEP and Resuscitated SUDEP (formerly…”
BackgroundMethodInfluentialVentilatory response to CO2 in patients with epilepsy
Sainju, R. K., Dragon, D. N., Winnike, H. B., Nashelsky, M. B., Granner, M. A., Gehlbach, B. K., & Richerson, G. B. (2019) · Epilepsia · doi:10.1111/epi.14660 · Cited by 57
Update on the Genetic Basis of Sudden Unexpected Death in Epilepsy
Coll, M., Oliva, A., Grassi, S., Brugada, R., & Campuzano, Ó. (2019) · International Journal of Molecular Sciences · doi:10.3390/ijms20081979 · Cited by 57
“Proposed sudden unexpected death in epilepsy (SUDEP) definition and classification [5].”
BackgroundMethodInfluentialSudden unexpected death in epilepsy in lamotrigine randomized‐controlled trials
Tomson, T., Hirsch, L. J., Friedman, D. J., Bester, N., Hammer, A. E., Irizarry, M. C., Ishihara, L., Krishen, A., Spaulding, T. C., Wamil, A., et al. (2012) · Epilepsia · doi:10.1111/j.1528-1167.2012.03689.x · Cited by 56
“They independently reviewed all treatment-blinded case narratives and autopsy reports, where available, and classified the deaths using both Annegers (1997) and Nashef et al. (2012) criteria.”
BackgroundMethodSelf-citationMechanisms of sudden unexplained death in epilepsy
Goldman, A. M. (2015) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000184 · Cited by 56
“If a patient is successfully resuscitated following a cardiopulmonary arrest, the event is classified as near-SUDEP [1].”
BackgroundInfluentialHata, Y., Yoshida, K., Kinoshita, K., & Nishida, N. (2016) · Brain Pathology · doi:10.1111/bpa.12390 · Cited by 55
Wenker, I. C., Teran, F. A., Wengert, E. R., Wagley, P. K., Panchal, P. S., Blizzard, E. A., Saraf, P., Wagnon, J. L., Goodkin, H. P., Meisler, M. H., et al. (2021) · Annals of Neurology · doi:10.1002/ana.26053 · Cited by 55
“not due to status epilepticus, nor does a postmortem examination reveal another cause of death.(1) SUDEP is the most”
BackgroundTobacco smoking, epilepsy, and seizures
Rong, L., Frontera, A. T., & Benbadis, S. R. (2014) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2013.11.022 · Cited by 53
“Suddenunexplaineddeath in epilepsy is defined as sudden, unexpected, witnessed or unwitnessed, nontraumatic, and nondrowning death in patientswith epilepsy, with orwithout evidence for a seizure and excluding documented status epilepticus, in which postmortem examination does not reveal a toxicological or anatomic cau…”
BackgroundMazzola, L., & Rheims, S. (2021) · Frontiers in Neurology · doi:10.3389/fneur.2021.642645 · Cited by 46
“SUDEP is a non-traumatic and non-drowning death in patients with epilepsy, unrelated to a documented status epilepticus, in which postmortem examination does not reveal a toxicological or anatomic cause of death (3, 4).”
Background
Metadata
- Authors
- Nashef, L., So, E. L., Ryvlin, P., & Tomson, T.
- Affiliations
- King's College Hospital (GB)
- Mayo Clinic (US)
- Lyon 1 Université (FR)
- Karolinska Institutet (SE)
- Corresponding author
- So, Elson L.
- Venue
- Epilepsia
- Publisher
- Wiley
- Published
- 2011-12-22
- Volume
- 53
- Issue
- 2
- Pages
- 227-233
- ISSN
- 0013-9580 (ISSN-L)
- 1528-1167
- OpenAlex
- W1549808427
- Cited by
- 632
- References
- 26
- Citations by year
- 2026: 34 · 2025: 54 · 2024: 39 · 2023: 57 · 2022: 38 · 2021: 70 · 2020: 69 · 2019: 59 · 2018: 55 · 2017: 39 · 2016: 30 · 2015: 35 · 2014: 24 · 2013: 20 · 2012: 8
- FWCI
- 11.60
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Epilepsia (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Epilepsy research and treatment
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Emergency Medicine › Cardiac Arrest and Resuscitation
- Keywords
- sudden unexpected death in epilepsy, status epilepticus, cardiac arrest, SUDEP incidence, SUDEP risk factors, SUDEP prevention
- MeSH terms
- Death, Sudden, Epilepsy, Humans
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