Welcome

An increased risk of sudden death has been reported in patients with epilepsy since the late 1800s, and possibly much earlier (DeToledo et al., 1999; Doherty, 2004). In the past two decades sudden unexpected (or unexplained) death in epilepsy (SUDEP) has received increasing attention and been recognized as more widespread than previously believed. Despite the recent increase in awareness, “systematic well funded research remains limited. The medical literature contains much repetition, with effort spent on re-analysing data with substantial methodological limitations,” (Tomson et al., 2008). In addition to the need for better clinical and basic science studies, and improved epidemiological data, there is also a need for increased cognizance and understanding of SUDEP among neurologists and other physicians as well as patients and families.

A massive literature on SUDEP and closely related phenomena exists, with many studies having considerable overlap. Many isolated case reports have been published, and considering them collectively increases their informative power. To facilitate comparison of related articles, annotations are provided for most entries in this bibliography, and these are hyperlinked where applicable to other articles’ in the hope of permitting a more efficient synthesis of the literature. To further promote direct consultation of the primary literature, references are hyperlinked to their digital object identifier when available, permitting an interested reader one-click access to the original publication. An index is provided, and its entries link back to the site of their appearance in the bibliography. Because the SUDEP literature contains “much repetition,” it is hoped that this ‘active document’ approach will permit readers to navigate through relevant publications more efficiently, with the ultimate aim of improved patient care and better mechanistic understanding of the process, both of which have the potential to reduce SUDEP deaths.

Articles (organized by author)

A

• Ackerman, MJ (2005): Cardiac causes of sudden unexpected death in children and their relationship to seizures and syncope: Genetic testing for cardiac electropathies
• Adams, R (1827): Cases of diseases of the heart accompanied with pathological observations
• Akalin F, Tirtir A, and Yilmaz Y (2003): Increased QT dispersion in epileptic children
• Al-Aweel IC, Krishnamurthy KB, Hausdorff JM, Mietus JE, Ives JR, Blum AS, Schomer DL, and Goldberger AL (1999): Postictal heart rate oscillations in partial epilepsy
• Almansori M, Ijaz M, and Ahmed SN (2006): Cerebral arrhythmia influencing cardiac rhythm: A case of ictal bradycardia
• Altenmüller DM, Zehender M, and Schulze-Bonhage A (2004): High-grade atrioventricular block triggered by spontaneous and stimulation-induced epileptic activity in the left temporal lobe
• Anand BK and Dua S (1956): Circulatory and respiratory changes induced by electrical stimulation of limbic system (visceral brain)
• Annegers JF and Coan SP (1999): SUDEP: Overview of definitions and review of incidence data
• Annegers JF, Coan SP, Hauser WA, Leestma J, Duffell W, and Tarver B (1998): Epilepsy, vagal nerve stimulation by the NCP system, mortality, and sudden, unexpected, unexplained death
• Annegers JF, Coan SP, Hauser WA, and Leestma J (2000): Epilepsy, vagal nerve stimulation by the NCP system, all-cause mortality, and sudden, unexpected, unexplained death
• Antoniuk SA, Oliva LV, Bruck I, Malucelli M, Yabumoto S, and Castellano JL (2001): Sudden unexpected, unexplained death in epilepsy autopsied patients
• Arida RM, Scorza CA, Schmidt B, de Albuquerque M, Cavalheiro EA, and Scorza FA (2008): Physical activity in sudden unexpected death in epilepsy: Much more than a simple sport
• Asadi-Pooya AA and Sperling MR (2009): Clinical features of sudden unexpected death in epilepsy
• Aserinsky E (1965): Periodic respiratory pattern occurring in conjunction with eye movements during sleep
• Aurlien D, Taubøll E, and Gjerstad L (2007): Lamotrigine in idiopathic epilepsy – Increased risk of cardiac death?
• Aurlien D, Tauböll E, and Gjerstad L (2008): An insufficient effect of lamotrigine leading to fatal seizures
• Aurlien D, Leren TP, Taubøll E, and Gjerstad L (2009): New SCN5A mutation in a SUDEP victim with idiopathic epilepsy

B

• Bacon GM (1868): On the modes of death in epilepsy
• Bateman LM, Li CS, and Seyal M (2008): Ictal hypoxemia in localization-related epilepsy: Analysis of incidence, severity and risk factors
• Bateman LM, Spitz M, and Seyal M (2010): Ictal hypoventilation contributes to cardiac arrhythmia and sudep: Report on two deaths in video-eeg-monitored patients
• Bell GS and Sander JW (2006): Sudden unexpected death in epilepsy. Risk factors, possible 225 mechanisms and prevention: A reappraisal
• Bell GS, Peacock JL, and Sander JW (2009): Seasonality as a risk factor for sudden unexpected death in epilepsy: A study in a large cohort
• Benassi E, Bo GP, Cocito L, Maffini M, and Loeb C (1987): Carbamazepine and cardiac conduction disturbances
• Bennani FK and Connolly CE (1997): Sudden unexpected death in young adults including four cases of SADS: A 10-year review from the west of Ireland
• Beran RG, Weber S, Sungaran R, Venn N, and Hung A (2004): Review of the legal obligations of the doctor to discuss Sudden Unexplained Death in Epilepsy (SUDEP) – A cohort controlled comparative cross-matched study in an outpatient epilepsy clinic
• Berg A (2001): Mortality in epilepsy
• Bergfeldt L, Vallin H, Rosenqvist M, Insulander P, Nordlander R, and Aström H (1996): Sinus node recovery time assessment revisited: Role of pharmacologic blockade of the autonomic nervous system
• Bird J, Dembny K, Sandeman D, and Butler S(1995): Sudden unexplained death explained?
• Bird J, Dembny K, Sandeman D, and Butler S(1997): Sudden unexplained death in epilepsy: An intracranially monitored case
• Black A (2005): SUDEP – Whether to tell and when
• Blum AS, Ives JR, Goldberger AL, Al-Aweel IC, Krishnamurthy KB, Drislane FW, and Schomer DL (2000): Oxygen desaturations triggered by partial seizures: Implications for cardiopulmonary instability in epilepsy
• Blumhardt LD, Smith PE, and Owen L (1986): Electrocardiographic accompaniments of temporal lobe epileptic seizures
• Bowerman DL, Levisky JA, Urich RW, and Wittenberg PH (1978): Premature deaths in persons with seizure disorders – Subtherapeutic levels of anticonvulsant drugs in postmortem blood specimens
• Bowers RE, McKeen CR, Park BE, and Brigham KL (1979): Increased pulmonary vascular permeability follows intracranial hypertension in sheep
• Breckenridge CG and Hoff HE (1953): Ischemic and anoxic dissolution of the supramedullary control of respiration
• Britton J, Ghearing G, Benarroch E, and Cascino G(2006): The ictal bradycardia syndrome: Localization and lateralization
• Brna P, Camfield P, Camfield C, Messenger M, and Finley J (2006): When are episodes of loss of consciousness life-threatening?
• Brodie MJ and Ben-Menachem E: Should patients or their families be told of the risk of Sudden Unexplained Death in Epilepsy?
• Brodie MJ and Holmes GL (2008): Should all patients be told about sudden unexpected death in epilepsy (SUDEP)? Pros and Cons
• Brotherstone R, Blackhall B, and McLellan A (2009): Lengthening of corrected QT during epileptic seizures
• Brown SW, Mawer GE, Lawler W, Taylor DC, Shorvon S, Betts TA, Noronha MJ, Richens A, Chadwick D, and Besag FM (1990): Sudden death and epilepsy

C

• Callanan C(2008): Death in the shadows
• Camfield CS, Camfield PR, and Veugelers PJ (2002): Death in children with epilepsy: A population-based study
• Camfield P and Camfield C (2005): Sudden unexpected death in people with epilepsy: A pediatric perspective
• Carinci V, Barbato G, Baldrati A, and Di Pasquale G (2007): Asystole induced by partial seizures: A rare cause of syncope
• Carlson C, Arnedo V, Cahill M, and Devinsky O (2009): Detecting nocturnal convulsions: Efficacy of the MP5 monitor
• Carnel SB, Schraeder PL, and Lathers CM (1985): Effect of phenobarbital pretreatment on cardiac neural discharge and pentylenetetrazol-induced epileptogenic activity in the cat
• Chapman WP, Livingston KE, and Poppen JL (1950): An observation of the effect of the electrical stimulation of the temporal lobes on blood pressure in man
• Chapman WP, Livingston KE, and Poppen JL (1950): Effect upon blood pressure of electrical stimulation of tips of temporal lobes in man
• Chapman WP, Schroeder HR, Geyer G, Brazier MA, Fager C, Poppen JL, Solomon HC, and Yakolev PI (1954): Physiological evidence concerning importance of the amygdaloid nuclear region in the integration of circulatory function and emotion in man
• Chapman WP, Solomon HC, Schroeder HR, Brazier MA, Geyer G, Fager C, Poppen J, and Yakolev P(1956): Present status of the relationship of brain structures to the circulation and emotional expression in man
• Chiron C, Marchand MC, Tran A, Rey E, d’Athis P, Vincent J, Dulac O, and Pons G (2000): Stiripentol in severe myoclonic epilepsy in infancy: a randomised placebo-controlled syndrome-dedicated trial. STICLO study group
• Cho I, Kai M, Ichikado K, Naitoh M, Sakata T, and Suga M (2002): A case of neurogenic pulmonary edema associated with epileptic seizure
• Chungath M and Shorvon S (2008): The mortality and morbidity of febrile seizures
• Cockerell OC, Johnson AL, Sander JW, Hart YM, Goodridge DM, and Shorvon SD (1994): Mortality from epilepsy: Results from a prospective population-based study
• Cohen MI (1971): Switching of the respiratory phases and evoked phrenic responses produced by rostral pontine electrical stimulation
• Cohen MI (1981): Pulmonary afferent and central influences on respiratory phase-switching in the cat
• Cohen-Gadol AA, DiLuna ML, and Spencer DD (2004): Partial epilepsy presenting as episodic dyspnea: A specific network involved in limbic seizure propagation
• Colder BW, Frysinger RC, Wilson CL, Harper RM, and Engel J J (1996): Decreased neuronal burst discharge near site of seizure onset in epileptic human temporal lobes
• Colder BW, Wilson CL, Frysinger RC, Chao LC, Harper RM, and Engel J J (1996): Neuronal synchrony in relation to burst discharge in epileptic human temporal lobes
• Colder BW, Wilson CL, Frysinger RC, Harper RM, and Engel J J (1996): Interspike intervals during interictal periods in human temporal lobe epilepsy
• Constantin L, Martins JB, Fincham RW, and Dagli RD (1990): Bradycardia and syncope as manifestations of partial epilepsy
• Cooper PN (2008): Cardiac arrhythmias and SUDEP--Commentary on Hindocha et al.
• Corfield DR, Fink GR, Ramsay SC, Murphy K, Harty HR, Watson JD, Adams L, Frackowiak RS, and Guz A(1995): Activation of limbic structures during CO2-stimulated breathing in awake man
• Corfield DR, Fink GR, Ramsay SC, Murphy K, Harty HR, Watson JD, Adams L, Frack- owiak RS, and Guz A (1995): Evidence for limbic system activation during CO2-stimulated breathing in man
• Cortey A, Monin P, Hascoet JM, Hamon I, and Vert P(1994): Effects of phenobarbital on cerebral blood flow during hypoxia
• Coulter DL (1984): Partial seizures with apnea and bradycardia
• Coyle HP, Baker-Brian N, and Brown SW (1994): Coroners’ autopsy reporting of sudden unexplained death in epilepsy (SUDEP) in the UK
• Critchley HD, Corfield DR, Chandler MP, Mathias CJ, and Dolan RJ (2000): Cerebral correlates of autonomic cardiovascular arousal: A functional neuroimaging investigation in humans

D

• Danielsson BR, Lansdell K, Patmore L, and Tomson T(2003): Phenytoin and phenobarbital inhibit human HERG potassium channels
• Danielsson BR, Lansdell K, Patmore L, and Tomson T (2005): Effects of the antiepileptic drugs lamotrigine, topiramate and gabapentin on hERG potassium currents
• Darbin O, Casebeer D, and Naritoku DK (2003): Effects of seizure severity and seizure repetition on postictal cardiac arrhythmia following maximal electroshock
• Darbin O, Casebeer DJ, and Naritoku DK (2002): Cardiac dysrhythmia associated with the immediate postictal state after maximal electroshock in freely moving rat
• Dasheiff RM (1991): Sudden unexpected death in epilepsy: a series from an epilepsy surgery program and speculation on the relationship to sudden cardiac death
• Dasheiff RM and Dickinson LJ (1986): Sudden unexpected death of epileptic patient due to cardiac arrhythmia after seizure
• Davis AM and Wilkinson JL (1998): The long QT syndrome and seizures in childhood
• de Albuquerque M, Scorza CA, Arida RM, Cavalheiro EA, and Scorza FA (2009): The mystery of Gustave Flaubert's death: could sudden unexpected death in epilepsy be part of the context?
• De Burgh Daly M(1986): Interactions between respiration and circulation
• de la Grandmaison GL (2006): Is there progress in the autopsy diagnosis of sudden unexpected death in adults?
• DeGiorgio CM, Miller P, Meymandi S, and Gornbein JA (2008): N-3 fatty acids (fish oil) for epilepsy, cardiac risk factors, and risk of SUDEP: clues from a pilot, double-blind, exploratory study
• Delgado JM (1960): Circulatory effects of cortical stimulation
• Derby LE, Tennis P, and Jick H (1996): Sudden unexplained death among subjects with refractory epilepsy
• DeToledo JC, DeToledo MB, and Lowe MR (1999): Epilepsy and sudden death: notes from George Washington's diaries on the illness and death of Martha Parke-Custis (1756-1773)
• Devilat Barros M, Rivera Gómez G, Gómez Muñoz V, and Sepulveda Olmos JP(2004): Mortality in children with epilepsy. A clinical prospective study
• Devinsky O, Price BH, and Cohen SI (1986): Cardiac manifestations of complex partial seizures
• Devinsky O, Perrine K, and eodore WH(1994): Interictal autonomic nervous system function in patients with epilepsy
• Devinsky O, Pacia S, and Tatambhotla G (1997): Bradycardia and asystole induced by partial seizures: A case report and literature review
• Devinsky O (2004): Effects of Seizures on Autonomic and Cardiovascular Function
• Doherty MJ (2004): The sudden death of Patsy Custis, or George Washington on sudden unexplained death in epilepsy
• Donahue LM, Coates PW, Lee VH, Ippensen DC, Arze SE, and Poduslo SE (2000): The cardiac sodium channel mRNA is expressed in the developing and adult rat and human brain
• Donner EJ, Smith CR, and Snead r OC (2001): Sudden unexplained death in children with epilepsy
• Drake ME, Reider CR, and Kay A (1993): Electrocardiography in epilepsy patients without cardiac symptoms
• Drake J ME, Andrews JM, and Castleberry CM (1998): Electrophysiologic assessment of autonomic function in epilepsy
• Dravet C, Bureau M, Oguni H, Fukuyama Y, and Cokar O(2005): Severe myoclonic epilepsy in infancy: Dravet syndrome
• Druschky A, Hilz MJ, Hopp P, Platsch G, Radespiel-Tröger M, Druschky K, Kuwert T, Stefan H, and Neundörfer B (2001): Interictal cardiac autonomic dysfunction in temporal lobe epilepsy demonstrated by 123I-metaiodobenzylguanidine-SPECT
• Duffin J and Hockman CH (1972): Limbic forebrain and midbrain modulation and phase-switching of expiratory neurons
• Duffin J, Hockman CH, Rupert AH, and Vachon BR(1974): Limbic system modulation of respiratory neurones
• Durelli L, Mutani R, Sechi GP, Monaco F, Glorioso N, and Gusmaroli G (1985): Cardiac side effects of phenytoin and carbamazepine. A dose-related phenomenon?
• Dütsch M, Hilz MJ, and Devinsky O (2006): Impaired baroreflex function in temporal lobe epilepsy
• Dütsch M, Devinsky O, Doyle W, Marthol H, and Hilz MJ (2004): Cerebral autoregulation improves in epilepsy patients after temporal lobe surgery

E

• Earnest MP, omas GE, Eden RA, and Hossack KF(1992): The sudden unexplained death syndrome in epilepsy: demographic, clinical, and postmortem features]]
• El-Sayed HL, Kotby AA, Tomoum HY, El-Hadidi ES, El Behery SE, and El-Ganzory AM (2007): Non-invasive assessment of cardioregulatory autonomic functions in children with epilepsy
• Epstein MA, Sperling MR, and O’Connor MJ (1992): Cardiac rhythm during temporal lobe seizures
• Erickson T(1939): Cardiac activity during epileptic seizures
• Evrengül H, Tanriverdi H, Dursunoglu D, Kaftan A, Kuru O, Unlu U, and Kilic M (2005): Time and frequency domain analyses of heart rate variability in patients with epilepsy

F

• Falconer B and Rajs J(1976): Post-mortem findings of cardiac lesions in epileptics: a preliminary report
• Faught E, Duh MS, Weiner JR, Guérin A, and Cunnington MC (2008): Nonadherence to antiepileptic drugs and increased mortality: findings from the RANSOM study
• Faustmann PM and Ganz RE (1994): Central cardio-autonomic disorganization in interictal states of epilepsy detected by phase space analysis
• Ferri R, Curzi-Dascalova L, Arzimanoglou A, Bourgeois M, Beaud C, Nunes ML, Elia M, Musumeci SA, and Tripodi M (2002): Heart rate variability during sleep in children with partial epilepsy
• Ficker DM(2000): Sudden unexplained death and injury in epilepsy
• Ficker DM, Cascino GD, and Clements IP (1998): Cardiac asystole masquerading as temporal lobe epilepsy
• Ficker DM, So EL, Shen WK, Annegers JF, O’Brien PC, Cascino GD, and Belau PG (1998): Population-based study of the incidence of sudden unexplained death in epilepsy
• Finsterer J and Stöllberger C (2009): Cardiopulmonary surveillance to prevent SUDEP
• Fish D(1997): Sudden unexpected death in epilepsy: Impact on clinical practice
• Freeman R and Schachter SC (1995): Autonomic epilepsy
• Freytag E and Lindenberg R (1964): 294 medicolegal autopsies on epileptics: Cerebral findings
• Friedman DE and Gilliam FG (2009): Seizure-related injuries are underreported in pharmacoresistant localization-related epilepsy
• Frostig RD, Frysinger RC, and Harper RM(1990): Recurring discharge patterns in multiple spike trains
• Frysinger RC and Harper RM (1986): Cardiac and respiratory relationships with neural discharge in the anterior cingulate cortex during sleep-walking states
• Frysinger RC and Harper RM (1989): Cardiac and respiratory correlations with unit discharge in human amygdala and hippocampus
• Frysinger RC and Harper RM(1990): Cardiac and respiratory correlations with unit discharge in epileptic human temporal lobe
• Frysinger RC, Marks JD, Trelease RB, Schechtman VL, and Harper RM (1984): Sleep states attenuate the pressor response to central amygdala stimulation
• Frysinger RC, Zhang JX, and Harper RM (1988): Cardiovascular and respiratory relationships with neuronal discharge in the central nucleus of the amygdala during sleep-waking states
• Frysinger RC, Engel J, and Harper RM (1993): Interictal heart rate patterns in partial seizure disorders
• Fujiwara T (2006): Clinical spectrum of mutations in SCN1A gene: severe myoclonic epilepsy in infancy and related epilepsies

G

• Galimberti CA, Marchioni E, Barzizza F, Manni R, Sartori I, and Tartara A (1996): Partial epileptic seizures of different origin variably affect cardiac rhythm
• Garcia M, D’Giano C, Estellés S, Leiguarda R, and Rabinowicz A (2001): Ictal tachycardia: Its discriminating potential between temporal and extratemporal seizure foci
• Gatto EM, Zurrú MC, and González MA (1996): Prolonged QT syndrome presenting as epilepsy
• George JR and Davis GG (1998): Comparison of anti-epileptic drug levels in different cases of sudden death

Gilbert R, Salanti G, Harden M, and See S (2005): Infant sleeping position and the sudden infant death syndrome: systematic review of observational studies and historical review of recommendations from 1940 to 2002

• Glasscock E, Yoo JW, Chen TT, Klassen TL, and Noebels JL (2010): Kv1.1 potassium channel deficiency reveals brain-driven cardiac dysfunction as a candidate mechanism for sudden unexplained death in epilepsy
• Gomes RA, Kesrouani S, Cruz J, Silva AL, Henriques TMG, Albuquerque Md, Arida RM, Sonoda EYF, Cysneiros RM, Terra VC et al. (2009): Is there something special about cardiovascular abnormalities and sudden unexpected death in epilepsy among patients with chronic renal insufficiency in regular hemodialysis program?
• González Martínez F, Navarro Gutiérrez S, de León Belmar JJ, and Valero Serrano B (2005): Electrocardiographic disorders associated to recent onset epilepsy
• González-Pal S, Barrios-Grillo E, and Franco-Salazar G(2004): Causes of death in epilepsy patients who died in a Cuban psychiatric hospital
• Goodman JH, Homan RW, and Crawford IL(1990): Kindled seizures elevate blood pressure and induce cardiac arrhythmias
• Goodman JH, Homan RW, and Crawford IL (1999): Kindled seizures activate both branches of the autonomic nervous system
• Gospe S and Camfield P (2005): Cardiac causes of sudden death: Virtual panel discussion of posed questions
• Groner C (2006): Hard Choices: Whether and How to Address SUDEP With Epileptic Patients

H

• Hadziefendic S and Haxhiu MA (1999): CNS innervation of vagal preganglionic neurons controlling peripheral airways: A transneuronal labeling study using pseudorabies virus
• Hanna J (1997): Epilepsy and sudden death: A personal view
• Hanna N, Black M, Sander J, Smithson W, Appleton R, Brown S, Fish D et al.(2002): The national sentinel clinical audit of epilepsy related death: Epilepsy – Death in the shadow
• Harnod T, Yang CCH, Hsin YL, Shieh KR, Wang PJ, and Kuo TBJ (2008): Heart rate variability in children with refractory generalized epilepsy
• Harnod T, Yang CCH, Hsin YL, Wang PJ, Shieh KR, and Kuo TBJ (2009): Heart rate variability in patients with frontal lobe epilepsy
• Harper RM, Frysinger RC, Trelease RB, and Marks JD (1984): State-dependent alteration of respiratory cycle timing by stimulation of the central nucleus of the amygdala
• Harper RM, Frysinger RC, Marks JD, Zhang JX, and Trelease RB(1988): Cardiorespiratory control during sleep
• Harper RM, Frysinger RC, Ni HF, and Terreberry RR(1990): Suprapontine influences on respiratory patterning during sleep-waking states
• Harper RM, Rector D, Poe G, Frysinger RC, Kristensen M, and Gozel D(1996): Rostral brain regions contributing to respiratory control
• Harper RM, Gozal D, Bandler R, Spriggs D, Lee J, and Alger J (1998): Regional brain activation in humans during respiratory and blood pressure challenges
• Harper RM, Poe GR, Rector DM, and Kristensen MP (1998): Relationships between hippocampal activity and breathing patterns
• Harper RM, Bandler R, Spriggs D, and Alger JR (2000): Lateralized and widespread brain activation during transient blood pressure elevation revealed by magnetic resonance imaging
• Harper RM, Macey PM, Henderson LA, Woo MA, Macey KE, Frysinger RC, Alger JR, Nguyen KP, and Yan-Go FL (2003): fMRI responses to cold pressor challenges in control and obstructive sleep apnea subjects
• Hart Y, Sander J, and Sharvon S(1989): National General Practice Study of Epilepsy and Epileptic Seizures: Objectives and study methodology of the largest reported prospective cohort study of epilepsy
• Harvey AS, Nolan T, and Carlin JB(1993): Community-based study of mortality in children with epilepsy
• Hascoet JM, Monin P, and Vert P(1988): Persistence of impaired autoregulation of cerebral blood flow in the postictal period in piglets