Download PDF
Nervenheilkunde 2014; 33(05): 325-330
DOI: 10.1055/s-0038-1633400
Epilepsie
Schattauer GmbH

Autonome Funktionsstörungen und Epilepsie

SUDEPAutonomic dysfunction and epilepsySUDEP
F. Kerling
1   Epilepsiezentrum Rummelsberg, Neurologische Klinik, Krankenhaus Rummelsberg gGmbH
› Author Affiliations
Further Information

Publication History

eingegangen am: 10 November 2013

angenommen am: 13 November 2013

Publication Date:
02 February 2018 (online)

Also available at
    Permissions and Reprints

Zusammenfassung

Der plötzliche unerwartete Tod bei Epilepsie (SUDEP) ist definiert als plötzliches unerwartetes Ereignis, das nicht durch andere Ursachen erklärt werden kann und wohl im Zusammenhang mit einem Anfall – nicht jedoch mit einem Status epilepticus – steht. SUDEP ist die häufigste krankheitsbedingte Todesursache bei Patienten mit schwer behandelbarer Epilepsie. Epidemiologische Studien konnten Risikofaktoren für das Auftreten von SUDEP identifizieren. Hierzu zählen z. B. männliches Geschlecht, hohe Anfallsfrequenz, medikamentöse Polytherapie und junges Alter bei Epilepsiebeginn. Als hauptsächliche Pathomechanismen werden eine zentrale Apnoe, kardiale Arrhythmien oder Fehlregulationen im autonomen Nervensystem vermutet. Die einzige wirksam belegte Prophylaxe bei SUDEP ist eine Optimierung der Epilepsietherapie.

Summary

Sudden unexpected death in epilepsy (SUDEP) is defined as a sudden unexpected event which can not be explained by other causes included status epilepticus but may be associated with a seizure. SUDEP is the most frequent disease related cause in patients with refractory epilepsy with incidence rates up to 9 per 1 000 patient-years in pharmaco-resistant epilepsy. Several studies aimed to find risk factors for the appearance of SUDEP. Among others male sex, a high seizure frequency, tonic clonic seizures and polytherapy were identified as risk factors. Potential mechanisms in SUDEP include depressed autonomic function, seizure-related apnoea, cardiac arrhythmia and postictal cardiomyopathy. The only successful prevention of SUDEP is efficacious antiepileptic drug therapy. Further studies have to prove the use of other strategies like cardiac devices and pharmacological modulation of apnoea.

Schlüsselwörter

SUDEP - autonomes Nervensystem - zentrale Apnoe - kardiale Arrhythmie - chronische Epilepsie

Keywords

SUDEP - cardiac arrhythmia - central apnoea - chronic epilepsy
 

  • Literatur

  • 1 Nashef L. Sudden unexpected death in epilepsy: terminology and definitions. Epilepsia 1997; 38 (Suppl. 11) S6-8.
    CrossrefPubMedSearch in Google Scholar
  • 2 Annegers JF. United States perspective on definitions and classifications. Epilepsia 1997; 38 (Suppl. 11) S9-12.
    CrossrefPubMedSearch in Google Scholar
  • 3 Doherty MJ. The sudden death of Patsy Custis, or George Washington on sudden unexplained death in epilepsy. Epilepsy Behav 2004; 05 (04) 598-600.
    CrossrefPubMedSearch in Google Scholar
  • 4 Tomson T, Nashef L, Ryvlin P. Sudden unexpected death in epilepsy: current knowledge and future directions. Lancet Neurol 2008; 07 (11) 1021-31.
    CrossrefPubMedSearch in Google Scholar
  • 5 Surges R, Thijs RD, Tan HL, Sander JW. Sudden unexpected death in epilepsy: risk factors and potential pathomechanisms. Nat Rev Neurol 2009; 05 (09) 492-504.
    CrossrefPubMedSearch in Google Scholar
  • 6 Neligan A. et al. The long-term risk of premature mortality in people with epilepsy. Brain 2011; 134 (Pt 2): 388-95.
    CrossrefPubMedSearch in Google Scholar
  • 7 Ficker DM. et al. Population-based study of the incidence of sudden unexplained death in epilepsy. Neurology 1998; 51 (05) 1270-4.
    CrossrefPubMedSearch in Google Scholar
  • 8 Tellez-Zenteno JF, Ronquillo LH, Wiebe S. Sudden unexpected death in epilepsy: evidence-based analysis of incidence and risk factors. Epilepsy Res 2005; 65 (1–2): 101-15.
    CrossrefPubMedSearch in Google Scholar
  • 9 Ryvlin P, Nashef L, Tomson T. Prevention of sudden unexpected death in epilepsy: A realistic goal?. Epilepsia 2013; 54 (Suppl. 02) 23-8.
    PubMedSearch in Google Scholar
  • 10 Sillanpaa M, Shinnar S. Long-term mortality in childhood-onset epilepsy. N Engl J Med 2010; 363 (26) 2522-9.
    CrossrefPubMedSearch in Google Scholar
  • 11 Hesdorffer DC. et al. Combined analysis of risk factors for SUDEP. Epilepsia 2011; 52 (06) 1150-9.
    CrossrefPubMedSearch in Google Scholar
  • 12 Marson AG. et al. The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial. Lancet 2007; 369 9566 1016-26.
    CrossrefPubMedSearch in Google Scholar
  • 13 Ryvlin P, Cucherat M, Rheims S. Risk of sudden unexpected death in epilepsy in patients given adjunctive antiepileptic treatment for refractory seizures: a meta-analysis of placebo-controlled randomised trials. Lancet Neurol 2011; 10 (11) 961-8.
    CrossrefPubMedSearch in Google Scholar
  • 14 Zhuo L. et al. Sudden unexpected death in epilepsy: Evaluation of forensic autopsy cases. Forensic Sci Int 2012; 223 (1–3): 171-5.
    CrossrefPubMedSearch in Google Scholar
  • 15 Surges R. et al. Postictal generalized electroencephalographic suppression is associated with generalized seizures. Epilepsy Behav 2011; 21 (03) 271-4.
    CrossrefPubMedSearch in Google Scholar
  • 16 Surges R. et al. Do alterations in inter-ictal heart rate variability predict sudden unexpected death in epilepsy?. Epilepsy Res 2009; |V87| (2–3): 277-80.
    PubMedSearch in Google Scholar
  • 17 Lhatoo SD. et al. An electroclinical case-control study of sudden unexpected death in epilepsy. Ann Neurol 2010; 68 (06) 787-96.
    CrossrefPubMedSearch in Google Scholar
  • 18 Surges R, Sander JW. Sudden unexpected death in epilepsy: mechanisms, prevalence, and prevention. Curr Opin Neurol 2012; 25 (02) 201-7.
    CrossrefPubMedSearch in Google Scholar
  • 19 Surges R. 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.
    CrossrefPubMedSearch in Google Scholar
  • 20 Surges R. et al. Pathologic cardiac repolarization in pharmacoresistant epilepsy and its potential role in sudden unexpected death in epilepsy: a casecontrol study. Epilepsia 2010; 51 (02) 233-42.
    CrossrefPubMedSearch in Google Scholar
  • 21 Rejdak K. et al. Analysis of ventricular late potentials in signal-averaged ECG of people with epilepsy. Epilepsia 2011; 52 (11) 2118-24.
    CrossrefPubMedSearch in Google Scholar
  • 22 Tu et al. Post-mortem review and genetic analysis of sudden unexpected death in epilepsy (SUDEP) cases. Brain Pathol 2011; 21 (02) 201-8.
    CrossrefPubMedSearch in Google Scholar
  • 23 Le Gal F. et al. A case of SUDEP in a patient with Dravet syndrome with SCN1A mutation. Epilepsia 2010; 51 (09) 1915-8.
    CrossrefPubMedSearch in Google Scholar
  • 24 Skluzacek JV. et al. Dravet syndrome and parent associations: the IDEA League experience with comorbid conditions, mortality, management, adaptation, and grief. Epilepsia 2011; 52 (Suppl. 02) 95-101.
    CrossrefPubMedSearch in Google Scholar
  • 25 Espinosa P. et al. Sudden unexpected near death in epilepsy: malignant arrhythmia from a partial seizure. Neurology 2009; 72 (19) 1702-3.
    CrossrefPubMedSearch in Google Scholar
  • 26 Moseley BD. et al. Electrocardiographic and oximetric changes during partial complex and generalized seizures. Epilepsy Res 2011; 95 (03) 237-45.
    CrossrefPubMedSearch in Google Scholar
  • 27 Surges R, Scott CA, Walker MC. Enhanced QT shortening and persistent tachycardia after generalized seizures. Neurology 2010; 74 (05) 421-6.
    CrossrefPubMedSearch in Google Scholar
  • 28 Strzelczyk A. et al. Postictal increase in T-wave alternans after generalized tonic-clonic seizures. Epilepsia 2011; 52 (11) 2112-7.
    CrossrefPubMedSearch in Google Scholar
  • 29 Kerling F. et al. Relation between ictal asystole and cardiac sympathetic dysfunction shown by MIBGSPECT. Acta Neurol Scand 2008; 120 (02) 123-9.
    Search in Google Scholar
  • 30 Britton JW. et al. The ictal bradycardia syndrome: localization and lateralization. Epilepsia 2006; 47 (04) 737-44.
    CrossrefPubMedSearch in Google Scholar
  • 31 Rocamora R. et al. Cardiac asystole in epilepsy: clinical and neurophysiologic features. Epilepsia 2003; 44 (02) 179-85.
    CrossrefPubMedSearch in Google Scholar
  • 32 Rugg-Gunn FJ. et al. Cardiac arrhythmias in focal epilepsy: a prospective long-term study. Lancet 2004; 364 9452 2212-9.
    CrossrefPubMedSearch in Google Scholar
  • 33 Schuele SU. et al. Ictal asystole: a benign condition?. Epilepsia 2008; 49 (01) 168-71.
    CrossrefPubMedSearch in Google Scholar
  • 34 P-Codrea S. et al. Sudden unexpected death in epilepsy: is death by seizures a cardiac disease?. Am J Forensic Med Pathol 2005; 26 (02) 99-105.
    PubMedSearch in Google Scholar
  • 35 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.
    CrossrefPubMedSearch in Google Scholar
  • 36 Moseley BD. et al. How common is ictal hypoxemia and bradycardia in children with partial complex and generalized convulsive seizures?. Epilepsia 2010; 51 (07) 1219-24.
    CrossrefPubMedSearch in Google Scholar
  • 37 Seyal M, Bateman LM. Ictal apnea linked to contralateral spread of temporal lobe seizures: Intracranial EEG recordings in refractory temporal lobe epilepsy. Epilepsia 2009; 50 (12) 2557-62.
    CrossrefPubMedSearch in Google Scholar
  • 38 So EL, Sam MC, Lagerlund TL. Postictal central apnea as a cause of SUDEP: evidence from near- SUDEP incident. Epilepsia 2000; 41 (11) 1494-7.
    CrossrefPubMedSearch in Google Scholar
  • 39 Tavee J, Morris H. 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.
    CrossrefPubMedSearch in Google Scholar
  • 40 Pezzella M. et al. Severe pulmonary congestion in a near miss at the first seizure: further evidence for respiratory dysfunction in sudden unexpected death in epilepsy. Epilepsy Behav 2009; 14 (04) 701-2.
    CrossrefPubMedSearch in Google Scholar
  • 41 Ryvlin P. et al. Incidence and mechanisms of cardiorespiratory arrests in epilepsy monitoring units (MORTEMUS): a retrospective study. Lancet Neurol 2013; 12 (10) 966-77.
    CrossrefPubMedSearch in Google Scholar
  • 42 Seyal M. et al. Seizure-related cardiac repolarization abnormalities are associated with ictal hypoxemia. Epilepsia 2011; 52 (11) 2105-11.
    CrossrefPubMedSearch in Google Scholar
  • 43 Persson H, Ericson M, Tomson T. Heart rate variability in patients with untreated epilepsy. Seizure 2007; 16 (06) 504-8.
    CrossrefPubMedSearch in Google Scholar
  • 44 Mativo P. et al. Study of cardiac autonomic function in drug-naive, newly diagnosed epilepsy patients. Epileptic Disord 2010; 12 (03) 212-6.
    PubMedSearch in Google Scholar
  • 45 Ronkainen E. et al. Suppressed circadian heart rate dynamics in temporal lobe epilepsy. J Neurol Neurosurg Psychiatry 2005; 76 (10) 1382-6.
    CrossrefPubMedSearch in Google Scholar
  • 46 Druschky A. et al. Interictal cardiac autonomic dysfunction in temporal lobe epilepsy demonstrated by [(123)I]metaiodobenzylguanidine- SPECT. Brain 2001; 124 (Pt 12): 2372-82.
    CrossrefPubMedSearch in Google Scholar
  • 47 Freitas J. et al. Age-specific periictal electroclinical features of generalized tonic-clonic seizures and potential risk of sudden unexpected death in epilepsy (SUDEP). Epilepsy Behav. 2013 Sep 4, online..
    PubMedSearch in Google Scholar
  • 48 Faught RE. et al. Impact of nonadherence to antiepileptic drugs on health care utilization and costs: findings from the RANSOM study. Epilepsia 2009; 50 (03) 501-9.
    PubMedSearch in Google Scholar
  • 49 Bell GS. et al. Premature mortality in refractory partial epilepsy: does surgical treatment make a difference?. J Neurol Neurosurg Psychiatry 2010; 81 (07) 716-8.
    CrossrefPubMedSearch in Google Scholar
  • 50 Schachter SC. Therapeutic effects of vagus nerve stimulation in epilepsy and implications for sudden unexpected death in epilepsy. Clin Auton Res 2006; 16 (01) 29-32.
    CrossrefPubMedSearch in Google Scholar
  • 51 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.
    CrossrefPubMedSearch in Google Scholar
  • 52 Bateman LM. et al. Serotonin reuptake inhibitors are associated with reduced severity of ictal hypoxemia in medically refractory partial epilepsy. Epilepsia. 2010 May 14, online..
    PubMedSearch in Google Scholar
  • 53 Brodie MJ, Holmes GL. Should all patients be told about sudden unexpected death in epilepsy (SUDEP)? Pros and Cons. Epilepsia 2008; 49 (Suppl. 09) 99-101.
    CrossrefPubMedSearch in Google Scholar
  • 54 Gayatri NA. 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.
    CrossrefPubMedSearch in Google Scholar