Sudden cardiac death (SCD) and sudden unexpected death in epilepsy (SUDEP)
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Oddział Neurologii, Szpital Miejski w Zabrzu
Katedra i Zakład Podstawowych Nauk Medycznych, Wydział Zdrowia Publicznego w Bytomiu, Śląski Uniwersytet Medyczny w Katowicach
Beata Łabuz-Roszak   

Katedra i Zakład Podstawowych Nauk Medycznych, Wydział Zdrowia Publicznego w Bytomiu, Śląski Uniwersytet Medyczny w Katowicach, ul. Piekarska 18, 41-902 Bytom
Ann. Acad. Med. Siles. 2019;73:60–64
Sudden unexpected death in people previously considered healthy represents an important challenge for modern medicine. The causes of this phenomenon are constantly being sought and attempts are being made to understand its pathomechanism. Among those at great risk are patients with epilepsy. Every unexpected death in a patient with epilepsy who was generally healthy in normal life activity and no other cause of the death was identified, is considered sudden unexpected death in epilepsy (SUDEP). Despite the difficulty in predicting sudden death, special attention should be paid to those at high risk, especially affected by cardiovascular diseases and epilepsy.
Zipes D.P., Wellens H.J. Sudden cardiac death. Circulation 1998; 98(21): 2334–2351.
Neuspiel D.R., Kuller L.H. Sudden and unexpected natural death in childhood and adolescence. JAMA 1985; 254(10): 1321–1325.
Martin C.A., Matthews G.D., Huang C.L. Sudden cardiac death and inherited channelopathy: the basic electrophysiology of the myocyte and myocardium in ion channel disease. Heart 2012; 98(7): 536–543, doi: 10.1136/heartjnl-2011-300953.
Dłużniewski M., Kucha M., Grzywanowska-Łaniewska I., Pikto-Pietkiewicz W. Nagła śmierć sercowa. Co lekarz praktyk wiedzieć powinien. Tom I–II. Wyd. Czelej. Lublin 2009.
Kauferstein S., Kiehne N., Jenewein T., Biel S., Kopp M., König R., Erkapic D., Rothschild M., Neumann T. Genetic analysis of sudden unexplained death: a multidisciplinary approach. Forensic Sci. Int. 2013; 229(1–3): 122–127, doi: 10.1016/j.forsciint.2013.03.050.
Wang D., Shah K.R., Um S.Y., Eng L.S., Zhou B., Lin Y., Mitchell A.A., Nicaj L., Prinz M., McDonald T.V., Sampson B.A., Tang Y. Cardiac channelopathy testing in 274 ethnically diverse sudden unexplained deaths. Forensic Sci. Int. 2014; 237: 90–99, doi: 10.1016/j.forsciint.2014.01.014.
Peters R.W. Propranolol and the morning increase in sudden cardiac death: (The Beta-Blocker Heart Attack Trial Experience). Am. J. Cardiol. 1990; 66(16): 57G–59G.
Willich S.N., Levy D., Rocco M.B., Tofler G.H., Stone P.H., Muller J.E. Circadian variation in the incidence of sudden cardiac death in the Framingham Heart Study population. Am. J. Cardiol. 1987; 60(10): 801–806.
Brugada P. Brugada syndrome: More than 20 years of scientific excitement. J. Cardiol. 2016; 67(3): 215–220, doi: 10.1016/j.jjcc.2015.08.009.
Zumhagen S., Zeidler E.M., Stallmeyer B., Ernsting M., Eckardt L., Schulze-Bahr E. Tpeak-Tend interval and Tpeak-Tend/QT ratio in patients with Brugada syndrome. Europace 2016; 18(12): 1866–1872, doi: 10.1093/europace/euw033.
Kauferstein S., Kiehne N., Peigneur S., Tytgat J., Bratzke H. Cardiac channelopathy causing sudden death as revealed by molecular autopsy. Int. J. Legal Med. 2013; 127(1): 145–151, doi: 10.1007/s00414-012-0679-5.
Zentner D., Thompson T., Taylor J., Bogwitz M., Trainer A., Vohra J., Winship I., James P.A. A rapid scoring tool to assess mutation probability in patients with inherited cardiac disorders. Eur. J. Med. Genet. 2018; 61(2): 61–67, doi: 10.1016/j.ejmg.2017.10.020.
Ingles J., Sarina T., Yeates L., Hunt L., Macciocca I., McCormack L., Winship I., McGaughran J., Atherton J., Semsarian C. Clinical predictors of genetic testing outcomes in hypertrophic cardiomyopathy. Genet. Med. 2013; 15(12): 972–977, doi: 10.1038/gim.2013.44.
Yu S., Li G., Huang C.L., Lei M., Wu L. Late sodium current associated cardiac electrophysiological and mechanical dysfunction. Pflugers Arch. 2018; 470(3): 461–469, doi: 10.1007/s00424-017-2079-7.
Auerbach D.S., Biton Y., Polonsky B., McNitt S., Gross R.A., Dirksen R.T., Moss A.J. Risk of cardiac events in Long QT syndrome patients when taking antiseizure medications. Transl. Res. 2018; 191: 81-92.e7, doi: 10.1016/j.trsl.2017.10.002.
Sauer A.J., Moss A.J., McNitt S., Peterson D.R., Zareba W., Robinson J.L., Qi M., Goldenberg I., Hobbs J.B., Ackerman M.J., Benhorin J., Hall W.J., Kaufman E.S., Locati E.H., Napolitano C., Priori S.G., Schwartz P.J., Towbin J.A., Vincent G.M., Zhang L. Long QT syndrome in adults. J. Am. Coll. Cardiol. 2007; 49(3): 329–337.
Goldenberg I., Moss A.J. Long QT syndrome. J. Am. Coll. Cardiol. 2008; 51(24): 2291–2300, doi: 10.1016/j.jacc.2008.02.068.
Molnar J., Rosenthal J.E., Weiss J.S., Somberg J.C. QT interval dispersion in healthy subjects and survivors of sudden cardiac death: circadian variation in a twenty-four-hour assessment. Am. J. Cardol. 1997; 79(9): 1190–1193.
Priori S.G., Napolitano C., Gasparini M., Pappone C., Della Bella P., Brignole M., Giordano U., Giovannini T., Menozzi C., Bloise R., Crotti L., Terreni L., Schwartz P.J. Clinical and genetic heterogeneity of right bundle branch block and ST-segment elevation syndrome: A prospective evaluation of 52 families. Circulation 2000; 102(20): 2509–2515.
Brugada J., Brugada R., Antzelevitch C., Towbin J., Nademanee K., Brugada P. Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V1 to V3. Circulation 2002; 105(1): 73–78.
Tester D.J., Spoon D.B., Valdivia H.H., Makielski J.C., Ackerman M.J. Targeted mutational analysis of the RyR2-encoded cardiac ryanodine receptor in sudden unexplained death: a molecular autopsy of 49 medical examiner/coroner’s cases. Mayo Clin. Proc. 2004; 79(11): 1380–1384.
Ackerman M.J., Tester D.J., Jones G.S., Will M.L., Burrow C.R., Curran M.E. Ethnic differences in cardiac potassium channel variants implications for genetic susceptibility to sudden cardiac death and genetic testing for congenital long QT syndrome. Mayo Clin. Proc. 2003; 78(12): 1479–1487.
Albert C.M., Nam E.G., Rimm E.B., Jin H.W., Hajjar R.J., Hunter D.J., MacRae C.A., Ellinor P.T. Cardiac sodium channel gene variants and sudden cardiac death in women. Circulation 2008; 117(1): 16–23.
Basso C., Carturan E., Pilichou K., Rizzo S., Corrado D., Thiene G. Sudden cardiac death with normal heart: molecular autopsy. Cardiovasc. Pathol. 2010; 19(6): 321–325, doi: 10.1016/j.carpath.2010.02.003.
Nilsson L., Farahmand B.Y., Persson P.G., Thiblin I., Tomson T. Risk factors for sudden unexpected death in epilepsy: a case-control study. Lancet 1999; 353(9156): 888–893.
Cockerell O.C. The mortality of epilepsy. Curr. Opin. Neurol. 1996; 9(2): 93–96.
Annegers J.F. United States perspective on definitions and classifications. Epilepsia 1997; 38(11 Suppl.): S9–12, doi: 10.1111/j.1528-1157.1997.tb06137.x.
Duble S.N., Thomas S.V. Sudden unexpected death in epilepsy. Indian J. Med. Res. 2017; 145(6): 738–745, doi: 10.4103/ijmr.IJMR_548_17.
Allen L.A., Harper R.M., Kumar R., Guye M., Ogren J.A., Lhatoo S.D., Lemieux L., Scott C.A., Vos S.B., Rani S., Diehl B. Dysfunctional brain networking among autonomic regulatory structures in temporal lobe epilepsy patients at high risk of Sudden Unexpected Death in Epilepsy. Front. Neurol. 2017; 8: 544, doi: 10.3389/fneur.2017.00544.
Téllez-Zenteno J.F., Ronquillo L.H., Wiebe S. Sudden unexpected death in epilepsy: evidence-based analysis of incidence and risk factors. Epilepsy Res. 2005; 65(1–2): 101–115.
Ficker D.M. Sudden unexplained death and injury in epilepsy. Epilepsia 2000; 41 Suppl. 2: S7–S12.
Langan Y., Nashef L., Sander J.W. Case-control study of SUDEP. Neurology 2005; 64(7): 1131–1133.
Walczak T.S., Leppik I.E., D’Amelio M., Rarick J., So E., Ahman P., Ruggles K., Cascino G.D., Annegers J.F., Hauser W.A. Incidence and risk factors in sudden unexpected death in epilepsy: a prospective cohort study. Neurology 2001; 56(4): 519–525.
Ryvlin P., Montavont A., Kahane P. Sudden unexpected death in epilepsy: from mechanisms to prevention. Curr. Opin. Neurol. 2006; 19(2): 194–199.
Langan Y., Sander J.W. Sudden unexpected death in patients with epilepsy. Definition, epidemiology and therapeutic implications. CNS Drugs 2000; 13(5): 337–349.
Timmings P.L. Sudden unexpected death in epilepsy: is carbamazepine implicated? Seizure 1998; 7(4): 289–291.
Walczak T. Do antiepileptic drugs play a role in sudden unexpected death in epilepsy? Drug Saf. 2003; 26(10): 673–683.
Aurlien D., Taubøll E., Gjerstad L. Lamotrigine in idiopathic epilepsy – increased risk of cardiac death? Acta Neurol. Scand. 2007; 115(3): 199–203.
Aurlien D., Leren T.P., Taubøll E., Gjerstad L. New SCN5A mutation in a SUDEP victim with idiopathic epilepsy. Seizure 2009; 18(2): 158–160, doi: 10.1016/j.seizure.2008.07.008.
Leestma J.E., Annegers J.F., Brodie M.J., Brown S., Schraeder P., Siscovick D., Wannamaker B.B., Tennis P.S., Cierpial M.A., Earl N.L. Sudden unexplained death in epilepsy: observations from a large clinical development program. Epilepsia 1997; 38(1): 47–55.
Stavem K., Guldvog B. Long-term survival after epilepsy surgery compared with matched epilepsy controls and the general population. Epilepsy Res. 2005; 63(1): 67–75.
Vickrey B.G., Hays R.D., Rausch R., Engel J. Jr, Visscher B.R., Ary C.M., Rogers W.H., Brook R.H. Outcomes in 248 patients who had diagnostic evaluations for epilepsy surgery. Lancet 1995; 346(8988): 1445–1449.
Sperling M.R., Harris A., Nei M., Liporace J.D., O’Connor M.J. Mortality after epilepsy surgery. Epilepsia 2005; 46(Suppl. 11): 49–53.
Rufo-Campos M. Mortality in epilepsies. Rev. Neurol. 2000; 30 Suppl. 1: S110–114.
Earnest M.P., Thomas G.E., Eden R.A., Hossack K.F. The sudden unexplained death syndrome in epilepsy: demographic, clinical, and postmortem features. Epilepsia 1992; 33(2): 310–316.
Li L.M., Roche J., Sander J.W. Ictal ECG changes in temporal lobe epilepsy. Arq. Neuropsiquiatr. 1995; 53(3-B): 619–624.
Blumhardt L.D., Smith P.E., Owen L. Electrocardiographic accompaniments of temporal lobe epileptic seizures. Lancet 1986; 1(8489): 1051–1056.
Davis A.M., Natelson B.H. Brain-heart interactions. The neurocardiology of arrhythmia and sudden cardiac death. Tex. Heart Inst. J. 1993; 20(3): 158–169.
Tokgözoglu S.L., Batur M.K., Topçuoglu M.A., Saribas O., Kes S., Oto A. Effects of stroke localization on cardiac autonomic balance and sudden death. Stroke 1999; 30(7): 1307–1311.
Shanlin R.J., Sole M.J., Rahimifar M., Tator C.H., Factor S.M. Increased intracranial pressure elicits hypertension, increased sympathetic activity, electrocardiographic abnormalities and myocardial damage in rats. J. Am. Coll. Cardiol. 1988; 12(3): 727–736.
Garaizar C. Sudden unexpected and unexplained death in epilepsy. Rev. Neurol. 2000; 31(5): 436–441.
Tigaran S., Rasmussen V., Dam M., Pedersen S., Høgenhaven H., Friberg B. ECG changes in epilepsy patients. Acta Neurol. Scand. 1997; 96(2): 72–75.
Opeskin K., Thomas A., Berkovic S.F. Does cardiac conduction pathology contribute to sudden unexpected death in epilepsy? Epilepsy Res. 2000; 40(1): 17–24.
Cheung R.T., Hachinski V. The insula and cerebrogenic sudden death. Arch. Neurol. 2000; 57(12): 1685–1688.
Nei M., Ho R.T., Abou-Khalil B.W., Drislane F.W., Liporace J., Romeo A., Sperling M.R. EEG and ECG in sudden unexplained death in epilepsy. Epilepsia 2004; 45(5): 338–345.
Heron S.E., Scheffer I.E., Berkovic S.F., Dibbens L.M., Mulley J.C. Channelopathies in idiopathic epilepsy. Neurotherapeutics 2007; 4(2): 295–304.