Plötzlicher, unerwarteter Tod bei Epilepsie

ZUSAMMENFASSUNG

Der plötzliche, unerwartete Tod eines Menschen mit Epilepsie wird mit dem englischen Akronym SUDEP bezeichnet (sudden unexpected death in epilepsy). Etwa einer von 1000 Epilepsiepatienten stirbt hieran, vor allem jene mit häufigeren nächtlichen generalisierten konvulsiven Anfällen. Die Mehrzahl der SUDEP-Fälle ist durch postiktuale Störungen der Atem- und Kreislauffunktion mit primärer zentraler Apnoe und sekundärer Asystolie verursacht. Die beteiligten zellulären Pathomechanismen sind jedoch noch ungeklärt. Die Aufklärung aller Patienten und Angehörigen wird durch angloamerikanische Fachgesellschaften empfohlen und von den meisten auch gewünscht. Entscheidend für die Prävention ist eine konsequente Anfallskontrolle. Zudem kann eine nächtliche Supervision das Risiko für SUDEP relevant mindern, dafür stehen klinisch geprüfte alltagstaugliche Anfallsdetektionssysteme zur Verfügung.

ABSTRACT

Sudden, unexpected death in epilepsy (SUDEP) affects about 1 in 1000 people with epilepsy. The most important risk factors are nocturnal and generalized convulsive seizures. Postictal primary central apnoea and secondary bradycardia and asystole cause SUDEP probably in most cases, the underlying cellular pathomechanisms are not elucidated yet. Comprehensive information of patients and relatives is recommended by medical societies in the USA and UK and desired by most patients and relatives. Full seizure control is key to SUDEP prevention. Nocturnal supervision significantly reduces the SUDEP risk, appropriate and clinically tested seizure detection systems are available.

Schlüsselwörter

SUDEP - Epilepsie - Anfälle - Prävention

Key words

SUDEP - epilepsy - seizures - prevention

• Literatur

• 1 Nashef L, So EL, Ryvlin P. et al Unifying the definitions of sudden unexpected death in epilepsy. Epilepsia 2012; 53 (02) 227-33
• 2 Lhatoo SD, Nei M, Raghavan M. et al Nonseizure SUDEP: Sudden unexpected death in epilepsy without preceding epileptic seizures. Epilepsia 2016; 57 (07) 1161-8
• 3 Doherty MJ. The sudden death of Patsy Custis, or George Washington on sudden unexplained death in epilepsy. Epilepsy Behav 2004; 5 (04) 598-600
• 4 Harden C, Tomson T, Gloss D. et al Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society. Neurology 2017; 88 (17) 1674-80
• 5 Nilsson L, Farahmand BY, Persson PG. et al Risk factors for sudden unexpected death in epilepsy: a case-control study. Lancet 1999; 353 9156 888-93
• 6 Bell GS, Gaitatzis A, Bell CL. et al Drowning in people with epilepsy: how great is the risk?. Neurology 2008; 71 (08) 578-82
• 7 Neligan A, Bell GS, Johnson AL. et al The long-term risk of premature mortality in people with epilepsy. Brain 2011; 134 Pt 2 388-95
• 8 Devinsky O, Spruill T, Thurman D. et al Recognizing and preventing epilepsy-related mortality: A call for action. Neurology 2016; 86 (08) 779-86
• 9 Surges R, Thijs RD, Tan HL. et al Sudden unexpected death in epilepsy: risk factors and potential pathomechanisms. Nat Rev Neurol 2009; 5 (09) 492-504
• 10 Lhatoo SD, Sander JW. Cause-specific mortality in epilepsy. Epilepsia 2005; 46 (Suppl. 11) 36-9
• 11 Hesdorffer DC, Tomson T, Benn E. et al Do antiepileptic drugs or generalized tonic-clonic seizure frequency increase SUDEP risk? A combined analysis. Epilepsia 2012; 53 (02) 249-52
• 12 Lamberts RJ, Thijs RD, Laffan A. et al Sudden unexpected death in epilepsy: people with nocturnal seizures may be at highest risk. Epilepsia 2012; 53 (02) 253-7
• 13 George JR, Davis GG. Comparison of anti-epileptic drug levels in different cases of sudden death. J Forensic Sci 1998; 43 (03) 598-603
• 14 Clark D, Riney K. A population-based post mortem study of sudden unexpected death in epilepsy. J Clin Neurosci 2016; 23: 58-62
• 15 Ficker DM, So EL, Shen WK. et al Population-based study of the incidence of sudden unexplained death in epilepsy. Neurology 1998; 51 (05) 1270-4
• 16 Thurman DJ, Hesdorffer DC, French JA. Sudden unexpected death in epilepsy: assessing the public health burden. Epilepsia 2014; 55 (10) 1479-85
• 17 Tomson T, Sveinsson O, Carlsson S. et al Evolution over time of SUDEP incidence: A nationwide population-based cohort study. Epilepsia 2018; 59 (08) e120-e4
• 18 Ryvlin P, Nashef L, Lhatoo SD. et al Incidence and mechanisms of cardiorespiratory arrests in epilepsy monitoring units (MORTEMUS): a retrospective study. Lancet Neurol 2013; 12 (10) 966-77
• 19 Bateman LM, Li CS, Seyal M. Ictal hypoxemia in localization-related epilepsy: analysis of incidence, severity and risk factors. Brain 2008; 131 Pt 12 3239-45
• 20 Nashef L, Walker F, Allen P. et al Apnoea and bradycardia during epileptic seizures: relation to sudden death in epilepsy. J Neurol Neurosurg Psychiatry 1996; 60 (03) 297-300
• 21 Seyal M, Bateman LM, Albertson TE. et al Respiratory changes with seizures in localization-related epilepsy: analysis of periictal hypercapnia and airflow patterns. Epilepsia 2010; 51 (08) 1359-64
• 22 Singh K, Katz ES, Zarowski M. et al Cardiopulmonary complications during pediatric seizures: a prelude to understanding SUDEP. Epilepsia 2013; 54 (06) 1083-91
• 23 Kennedy JD, Hardin KA, Parikh P. et al Pulmonary edema following generalized tonic clonic seizures is directly associated with seizure duration. Seizure 2015; 27: 19-24
• 24 Kloster R, Engelskjon T. Sudden unexpected death in epilepsy (SUDEP): a clinical perspective and a search for risk factors. J Neurol Neurosurg Psychiatry 1999; 67 (04) 439-44
• 25 Middleton O, Atherton D, Bundock E. et al National Association of Medical Examiners position paper: Recommendations for the investigation and certification of deaths in people with epilepsy. Epilepsia 2018; 59 (03) 530-43
• 26 Peng W, Danison JL, Seyal M. Postictal generalized EEG suppression and respiratory dysfunction following generalized tonic-clonic seizures in sleep and wakefulness. Epilepsia 2017; 58 (08) 1409-14
• 27 Tavee J, Morris H. 3 rd. Severe postictal laryngospasm as a potential mechanism for sudden unexpected death in epilepsy: a near-miss in an EMU. Epilepsia 2008; 49 (12) 2113-7
• 28 Marchi A, Giusiano B, King M. et al Postictal electroencephalographic (EEG) suppression: A stereo-EEG study of 100 focal to bilateral tonic-clonic seizures. Epilepsia 2019; 60 (01) 63-73
• 29 Lamberts RJ, Gaitatzis A, Sander JW. et al Postictal generalized EEG suppression: an inconsistent finding in people with multiple seizures. Neurology 2013; 81 (14) 1252-6
• 30 Lamberts RJ, Laranjo S, Kalitzin SN. et al Postictal generalized EEG suppression is not associated with periictal cardiac autonomic instability in people with convulsive seizures. Epilepsia 2018; 54 (03) 523-9
• 31 Kuo J, Zhao W, Li CS. et al Postictal immobility and generalized EEG suppression are associated with the severity of respiratory dysfunction. Epilepsia 2016; 57 (03) 412-7
• 32 Lhatoo SD, Faulkner HJ, Dembny K. et al An electroclinical case-control study of sudden unexpected death in epilepsy. Ann Neurol 2010; 68 (06) 787-96
• 33 Dasheiff RM, Dickinson LJ. Sudden unexpected death of epileptic patient due to cardiac arrhythmia after seizure. Arch Neurol 1986; 43 (02) 194-6
• 34 Espinosa PS, Lee JW, Tedrow UB. et al Sudden unexpected near death in epilepsy: malignant arrhythmia from a partial seizure. Neurology 2009; 72 (19) 1702-3
• 35 Ferlisi M, Tomei R, Carletti M. et al Seizure induced ventricular fibrillation: a case of near-SUDEP. Seizure 2013; 22 (03) 249-51
• 36 Cunnington C, Garg S, Balachandran KP. Seizure-associated takotsubo cardiomyopathy presenting with unheralded ventricular fibrillation. Int J Cardiol 2012; 162 (01) e21-3
• 37 van der Lende M, Surges R, Sander JW. et al Cardiac arrhythmias during or after epileptic seizures. J Neurol Neurosurg Psychiatry 2016; 87 (01) 69-74
• 38 Nass RD, Motloch LJ, Paar V. et al Blood markers of cardiac stress after generalized convulsive seizures. Epilepsia 2019 Jan 15; ePub ahead
• 39 Surges R, Walker MC. Peri-ictal heart rates depend on seizure-type. Seizure 2010; 19 (07) 453
• 40 Surges R, Taggart P, Sander JW. et al Too long or too short? New insights into abnormal cardiac repolarization in people with chronic epilepsy and its potential role in sudden unexpected death. Epilepsia 2010; 51 (05) 738-44
• 41 Surges R, Scott CA, Walker MC. Enhanced QT shortening and persistent tachycardia after generalized seizures. Neurology 2010; 74 (05) 421-6
• 42 Surges R, Adjei P, Kallis C. et al Pathologic cardiac repolarization in pharmacoresistant epilepsy and its potential role in sudden unexpected death in epilepsy: a case-control study. Epilepsia 2010; 51 (02) 233-42
• 43 Strzelczyk A, Adjei P, Scott CA. et al Postictal increase in T-wave alternans after generalized tonic-clonic seizures. Epilepsia 2011; 52 (11) 2112-7
• 44 Duplyakov D, Golovina G, Lyukshina N. et al Syncope, seizure-induced bradycardia and asystole: two cases and review of clinical and pathophysiological features. Seizure 2014; 23 (07) 506-11
• 45 Schuele SU, Bermeo AC, Alexopoulos AV. et al Video-electrographic and clinical features in patients with ictal asystole. Neurology 2007; 69 (05) 434-41
• 46 Natelson BH, Suarez RV, Terrence CF. et al Patients with epilepsy who die suddenly have cardiac disease. Arch Neurol 1998; 55 (06) 857-60
• 47 Fialho GL, Wolf P, Walz R, Lin K. Increased cardiac stiffness is associated with autonomic dysfunction in patients with temporal lobe epilepsy. Epilepsia 2018; 59 (06) e85-e90
• 48 Devinsky O, Kim A, Friedman D. et al Incidence of cardiac fibrosis in SUDEP and control cases. Neurology 2018; 91 (01) e55-e61
• 49 Lamberts RJ, Blom MT, Wassenaar M. et al Sudden cardiac arrest in people with epilepsy in the community: Circumstances and risk factors. Neurology 2015; 85 (03) 212-8
• 50 Ryvlin P, Cascino GD. Sudden unexpected death in epilepsy patients is often misdiagnosed as sudden cardiac death. Neurology 2017; 89 (09) 878-9
• 51 Hampel KG, Jahanbekam A, Elger CE. et al Seizure-related modulation of systemic arterial blood pressure in focal epilepsy. Epilepsia 2016; 57 (10) 1709-18
• 52 Hampel KG, Elger CE, Surges R. Impaired Baroreflex Sensitivity after Bilateral Convulsive Seizures in Patients with Focal Epilepsy. Front Neurol 2017; 8: 210
• 53 Tupal S, Faingold CL. Evidence supporting a role of serotonin in modulation of sudden death induced by seizures in DBA/2 mice. Epilepsia 2006; 47 (01) 21-6
• 54 Tecott LH, Sun LM, Akana SF. et al Eating disorder and epilepsy in mice lacking 5-HT2c serotonin receptors. Nature 1995; 374 6522 542-6
• 55 Shen HY, Li T, Boison D. A novel mouse model for sudden unexpected death in epilepsy (SUDEP): role of impaired adenosine clearance. Epilepsia 2010; 51 (03) 465-8
• 56 Loonen ICM, Jansen NA, Cain SM. et al Brainstem spreading depolarization and cortical dynamics during fatal seizures in Cacna1a S218L mice. Brain 2019 Jan 12; ePub ahead
• 57 Aiba I, Wehrens XH, Noebels JL. Leaky RyR2 channels unleash a brainstem spreading depolarization mechanism of sudden cardiac death. Proc Natl Acad Sci U S A 2016; 113 (33) E4895-903
• 58 Stewart M. An explanation for sudden death in epilepsy (SUDEP). J Physiol Sci 2018; 68 (04) 307-20
• 59 Blum AS. Respiratory physiology of seizures. J Clin Neurophysiol 2009; 26 (05) 309-15
• 60 Bagnall RD, Crompton DE, Semsarian C. Genetic Basis of Sudden Unexpected Death in Epilepsy. Front Neurol 2017; 8: 348
• 61 Neubauer J, Lecca MR, Russo G. et al Post-mortem whole-exome analysis in a large sudden infant death syndrome cohort with a focus on cardiovascular and metabolic genetic diseases. Eur J Hum Genet 2017; 25 (04) 404-9
• 62 Wandschneider B, Koepp M, Scott C. et al Structural imaging biomarkers of sudden unexpected death in epilepsy. Brain 2015; 138 Pt 10 2907-19
• 63 Mueller SG, Bateman LM, Laxer KD. Evidence for brainstem network disruption in temporal lobe epilepsy and sudden unexplained death in epilepsy. Neuroimage Clin 2014; 5: 208-16
• 64 Michalak Z, Obari D, Ellis M. et al Neuropathology of SUDEP: Role of inflammation, blood-brain barrier impairment, and hypoxia. Neurology 2017; 88 (06) 551-61
• 65 Patodia S, Somani A, O’Hare M. et al The ventrolateral medulla and medullary raphe in sudden unexpected death in epilepsy. Brain 2018; 141 (06) 1719-33
• 66 Hesdorffer DC, Tomson T, Benn E. et al Combined analysis of risk factors for SUDEP. Epilepsia 2011; 52 (06) 1150-9
• 67 Ryvlin P, So EL, Gordon CM. et al Long-term surveillance of SUDEP in drug-resistant epilepsy patients treated with VNS therapy. Epilepsia 2018; 59 (03) 562-72
• 68 Devinsky O, Friedman D, Duckrow RB. et al Sudden unexpected death in epilepsy in patients treated with brain-responsive neurostimulation. Epilepsia 2018; 59 (03) 555-61
• 69 Simeone KA, Matthews SA, Rho JM. et al Ketogenic diet treatment increases longevity in Kcna1-null mice, a model of sudden unexpected death in epilepsy. Epilepsia 2016; 57 (08) e178-82
• 70 Elger CE, Berkenfeld R. S1-Leitlinie Erster epileptischer Anfall und Epilepsien im Erwachsenenalter. 2017 [23.01.2019]; Available from www.dgn.org/leitlinien
• 71 Surges R, von Wrede R, Porschen T. et al Knowledge of sudden unexpected death in epilepsy (SUDEP) among 372 patients attending a German tertiary epilepsy center. Epilepsy Behav 2018; 80: 360-4
• 72 Strzelczyk A, Zschebek G, Bauer S. et al Predictors of and attitudes toward counseling about SUDEP and other epilepsy risk factors among Austrian, German, and Swiss neurologists and neuropediatricians. Epilepsia 2016; 57 (04) 612-20
• 73 Fong CY, Lim WK, Kong AN. et al Provision of sudden unexpected death in epilepsy (SUDEP) information among Malaysian parents of children with epilepsy. Epilepsy Behav 2017; 75: 6-12
• 74 Gayatri NA, Morrall MC, Jain V. et al Parental and physician beliefs regarding the provision and content of written sudden unexpected death in epilepsy (SUDEP) information. Epilepsia 2010; 51 (05) 777-82
• 75 Tonberg A, Harden J, McLellan A. et al A qualitative study of the reactions of young adults with epilepsy to SUDEP disclosure, perceptions of risks, views on the timing of disclosure, and behavioural change. Epilepsy Behav 2015; 42: 98-106
• 76 Langan Y, Nashef L, Sander JW. Case-control study of SUDEP. Neurology 2005; 64 (07) 1131-3
• 77 van der Lende M, Hesdorffer DC. Sander et al Nocturnal supervision and SUDEP risk at different epilepsy care settings. Neurology 2018; 91 (16) e1508-e18
• 78 Catcheside PG, Mohtar AA, Reynolds KJ. Airflow resistance and CO2 rebreathing properties of anti-asphyxia pillows designed for epilepsy. Seizure 2014; 23 (06) 462-7
• 79 Ryvlin P, Ciumas C, Wisniewski I. et al Wearable devices for sudden unexpected death in epilepsy prevention. Epilepsia 2018; 59 (Suppl. 01) 61-6
• 80 Narechania AP, Garic II, Sen-Gupta I. et al Assessment of a quasi-piezoelectric mattress monitor as a detection system for generalized convulsions. Epilepsy Behav 2013; 28 (02) 172-6
• 81 Beniczky S, Polster T, Kjaer TW. et al Detection of generalized tonic-clonic seizures by a wireless wrist accelerometer: a prospective, multicenter study. Epilepsia 2013; 54 (04) e58-61
• 82 Poh MZ, Loddenkemper T, Reinsberger C. et al Convulsive seizure detection using a wrist-worn electrodermal activity and accelerometry biosensor. Epilepsia 2012; 53 (05) e93-7
• 83 Arends J, Thijs RD, Gutter T. et al Multimodal nocturnal seizure detection in a residential care setting: A long-term prospective trial. Neurology 2010; 91 (21) e2010-e9
• 84 Elger CE, Johnston SA, Hoppe C. Diagnosing and treating depression in epilepsy. Seizure 2017; 44: 184-93
• 85 Favale E, Rubino V, Mainardi P. et al Anticonvulsant effect of fluoxetine in humans. Neurology 1995; 45 (10) 1926-7
• 86 Richerson GB, Boison D, Faingold CL. et al From unwitnessed fatality to witnessed rescue: Pharmacologic intervention in sudden unexpected death in epilepsy. Epilepsia 2016; 57 (Suppl. 01) 35-45