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词条 Sudden unexpected death in epilepsy
释义

  1. Risk factors

  2. Mechanism

  3. Management

  4. Epidemiology

  5. References

  6. External links

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}}Sudden unexpected death in epilepsy (SUDEP) is a fatal complication of epilepsy.[1] It is defined as the sudden and unexpected, non-traumatic and non-drowning death of a person with epilepsy, without a toxicological or anatomical cause of death detected during the post-mortem examination.[2][3]

While the mechanisms underlying SUDEP are still poorly understood, it is possibly the most common cause of death as a result of complications from epilepsy, accounting for between 7.5 and 17% of all epilepsy-related deaths[2] and 50% of all deaths in refractory epilepsy.[4][5] The causes of SUDEP seem to be multifactorial[2] and include respiratory, cardiac and cerebral factors, as well as the severity of epilepsy and seizures.[5] Proposed pathophysiological mechanisms include seizure-induced cardiac and respiratory arrests.[4]

SUDEP occurs in about 1 in 1,000 adults and 1 in 4,500 children with epilepsy a year.[6] Rates of death as a result of prolonged seizures (status epilepticus) are not classified as SUDEP.[7]

Risk factors

Consistent risk factors include:

  • Severity of seizures, increased refractoriness of epilepsy and presence of generalized tonic-clonic seizures:[4] the most consistent risk factor is an increased frequency of tonic–clonic seizures.[5]
  • Poor compliance.[4] Lack of therapeutic levels of anti-epileptic drugs, non-adherence to treatment regimens, and frequent changes in regimens are risk factors for sudden death.[5]
  • Young age, and early age of seizures onset.[4]
  • Male gender[8]
  • Poly-therapy of epilepsy. It remains unclear whether this is an independent risk factor or a surrogate marker for severity of epilepsy.[9]
  • Being asleep during a seizure is likely to favour SUDEP occurrence.[10]

Mechanism

The mechanisms underlying SUDEP are not well understood but probably involve several pathophysiological mechanisms and circumstances. The most commonly involved are seizure-induced hypoventilation and cardiac arrhythmias but different mechanisms may be involved in different individuals, and more than one mechanism may be involved in any one individual.[11]

  • Cardiac factors: cardiac arrhythmias and other cardiac events are known to be involved in some cases of SUDEP.[5] Such arrhythmias are defined as ictal arrhythmias and include the ictal asystole which is a rare occurrence mostly in people that have temporal lobe epilepsy.[12][13]
  • Respiratory factors: impaired respiration and seizure induced pulmonary dysfunction as well as central apnea as a result of brain-stem respiratory centers suppression are known to play a role in some cases of SUDEP.[5]
  • Cerebral and autonomic nervous system dysregulation: cardiac arrhythmia and respiratory failure as a result of seizure related changes to brain function and dysfunction of the autonomic nervous system have been described in cases of SUDEP. These include cases of post-ictal generalized EEG suppression described as cerebral shutdown, but its significance remains unclear.[9]
  • Genetic factors: mutations in several genes have been associated with an increased susceptibility to SUDEP. Over 33% of these are related to mutations which lead to increased susceptibility for arrhythmia. Genes involved include the hyperpolarization-activated cyclic nucleotide-gated channels genes (HCN1, HCN2, HCN3, and HCN4).[2]
  • Anti epileptic drugs: most evidence suggests that antiepileptic drugs are not associated with an increased risk for SUDEP, but rather reduce its incidence.[14] Some studies however indicate that some antiepileptic drugs such as lamotrigine and carbamazepine, may increase the risk of SUDEP in certain individuals.[15] It is unclear if this is because of the potential cardio-respiratory adverse effects such as lengthening of the QT interval and reduction of heart rate known to be associated with these drugs under certain circumstances,[11] or because a high drug dosage could be a surrogate marker for poor seizure control.[16]
  • Vagal nerve stimulation: concerns have been raised that vagal nerve stimulation may induce bradycardia or cardiac arrest, and may exacerbate sleep apnoea common in people with epilepsy.[11]

Management

The lack of generally recognized clinical recommendations available are a reflection of the dearth of data on the effectiveness of any particular clinical strategy,[11] but on the basis of present evidence, the following may be relevant:

  • Epileptic seizure control with the appropriate use of medication and lifestyle counseling is the focus of prevention.[5]
  • Reduction of stress, participation in physical exercises, and night supervision might minimize the risk of SUDEP.[2]
  • Knowledge of how to perform the appropriate first-aid responses to seizure by persons who live with epileptic people may prevent death.[5]
  • People associated with arrhythmias during seizures should be submitted to extensive cardiac investigation[2] with a view to determining the indication for on-demand cardiac pacing.[11]
  • Successful epilepsy surgery may reduce the risk of SUDEP, but this depends on the outcome in terms of seizure control.[11]
  • The use of anti suffocation pillows have been advocated by some practitioners to improve respiration while sleeping, but their effectiveness remain unproven because experimental studies are lacking.[5]
  • Providing information to individuals and relatives about SUDEP is beneficial.[9]

Epidemiology

  • SUDEP accounts for 8-17% of deaths in people with epilepsy.[17]
  • The risk of sudden death in young adults with epilepsy is increased 24-fold compared to the general population.[9]
  • SUDEP is the number one cause of epilepsy related death in people with pharmaco-resistant epilepsy.[9]
  • Children with epilepsy have a cumulative risk of dying suddenly of 7% within 40 years.[9]
  • In children, SUDEP accounts for 34% of all sudden deaths.[18]

References

1. ^{{cite journal|last=Ryvlin|first=P|author2=Nashef, L |author3=Tomson, T |title=Prevention of sudden unexpected death in epilepsy: a realistic goal?|journal=Epilepsia|date=May 2013|volume=54 Suppl 2|pages=23–8|pmid=23646967|doi=10.1111/epi.12180}}
2. ^1 2 3 4 5 {{cite journal|last=Terra|first=VC|author2=Cysneiros, R |author3=Cavalheiro, EA |author4= Scorza, FA |title=Sudden unexpected death in epilepsy: from the lab to the clinic setting.|journal=Epilepsy & Behavior|date=Mar 2013|volume=26|issue=3|pages=415–20|pmid=23402930 |doi=10.1016/j.yebeh.2012.12.018}}
3. ^{{cite journal|last=Nashef|first=L|author2=So, EL |author3=Ryvlin, P |author4= Tomson, T |title=Unifying the definitions of sudden unexpected death in epilepsy.|journal=Epilepsia|date=Feb 2012|volume=53|issue=2|pages=227–33|pmid=22191982 |doi=10.1111/j.1528-1167.2011.03358.x}}
4. ^1 2 3 4 {{cite journal|last=Tolstykh|first=GP|author2=Cavazos, JE |title=Potential mechanisms of sudden unexpected death in epilepsy.|journal=Epilepsy & Behavior|date=Mar 2013|volume=26|issue=3|pages=410–4|pmid=23305781|doi=10.1016/j.yebeh.2012.09.017}}
5. ^1 2 3 4 5 6 7 8 {{cite journal|last=Devinsky|first=Orrin|title=Sudden, Unexpected Death in Epilepsy|journal=New England Journal of Medicine|date=10 November 2011|volume=365|issue=19|pages=1801–1811|doi=10.1056/NEJMra1010481}}
6. ^{{cite journal|last1=Harden|first1=C|last2=Tomson|first2=T|last3=Gloss|first3=D|last4=Buchhalter|first4=J|last5=Cross|first5=JH|last6=Donner|first6=E|last7=French|first7=JA|last8=Gil-Nagel|first8=A|last9=Hesdorffer|first9=DC|last10=Smithson|first10=WH|last11=Spitz|first11=MC|last12=Walczak|first12=TS|last13=Sander|first13=JW|last14=Ryvlin|first14=P|title=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.|journal=Neurology|date=25 April 2017|volume=88|issue=17|pages=1674–1680|pmid=28438841|doi=10.1212/WNL.0000000000003685}}
7. ^{{cite journal |vauthors=Tomson T, Nashef L, Ryvlin P | title = Sudden unexpected death in epilepsy: current knowledge and future directions | journal = Lancet Neurology | volume = 7 | issue = 11 | pages = 1021–31 |date=November 2008 | pmid = 18805738 | doi = 10.1016/S1474-4422(08)70202-3 | url = | issn = }}
8. ^{{Cite journal|pmid=27434597|year=2016|author1=Maguire|first1=M. J.|title=Cochrane Database of Systematic Reviews|journal=The Cochrane Database of Systematic Reviews|volume=7|pages=CD011792|last2=Jackson|first2=C. F.|last3=Marson|first3=A. G.|last4=Nolan|first4=S. J.|doi=10.1002/14651858.CD011792.pub2|chapter=Treatments for the prevention of Sudden Unexpected Death in Epilepsy (SUDEP)}}
9. ^1 2 3 4 5 {{cite journal|last=Surges|first=R|author2=Sander, JW |title=Sudden unexpected death in epilepsy: mechanisms, prevalence, and prevention.|journal=Current Opinion in Neurology|date=Apr 2012|volume=25|issue=2|pages=201–7|pmid=22274774|doi=10.1097/WCO.0b013e3283506714}}
10. ^{{cite journal|last=Nobili|first=L|author2=Proserpio, P |author3=Rubboli, G |author4=Montano, N |author5=Didato, G |author6= Tassinari, CA |title=Sudden unexpected death in epilepsy (SUDEP) and sleep.|journal=Sleep Medicine Reviews|date=Aug 2011|volume=15|issue=4|pages=237–46|pmid=20951616 |doi=10.1016/j.smrv.2010.07.006}}
11. ^1 2 3 4 5 {{cite journal|last=Shorvon|first=S|author2=Tomson, T |title=Sudden unexpected death in epilepsy.|journal=Lancet|date=Dec 10, 2011|volume=378|issue=9808|pages=2028–38|pmid=21737136|doi=10.1016/S0140-6736(11)60176-1}}
12. ^{{cite journal|last=So|first=N. K.|author2=Sperling, M. R. |title=Ictal asystole and SUDEP|journal=Neurology|date=31 July 2007|volume=69|issue=5|pages=423–424|doi=10.1212/01.wnl.0000268698.04032.bc}}
13. ^{{cite journal|last=Schuele|first=SU|author2=Bermeo, AC |author3=Alexopoulos, AV |author4=Locatelli, ER |author5=Burgess, RC |author6=Dinner, DS |author7= Foldvary-Schaefer, N |title=Video-electrographic and clinical features in patients with ictal asystole.|journal=Neurology|date=Jul 31, 2007|volume=69|issue=5|pages=434–41|pmid=17664402 |doi=10.1212/01.wnl.0000266595.77885.7f}}
14. ^{{cite journal|last=Ryvlin|first=P|author2=Cucherat, M |author3=Rheims, S |title=Risk of sudden unexpected death in epilepsy in patients given adjunctive antiepileptic treatment for refractory seizures: a meta-analysis of placebo-controlled randomised trials.|journal=Lancet Neurology|date=Nov 2011|volume=10|issue=11|pages=961–8|pmid=21937278|doi=10.1016/S1474-4422(11)70193-4}}
15. ^{{cite journal|last=Hesdorffer|first=DC|author2=Tomson, T |title=Sudden unexpected death in epilepsy. Potential role of antiepileptic drugs.|journal=CNS Drugs|date=Feb 2013|volume=27|issue=2|pages=113–9|pmid=23109241|doi=10.1007/s40263-012-0006-1}}
16. ^{{cite journal|last=Surges|first=R|author2=Thijs, RD |author3=Tan, HL |author4= Sander, JW |title=Sudden unexpected death in epilepsy: risk factors and potential pathomechanisms.|journal=Nature Reviews. Neurology|date=Sep 2009|volume=5|issue=9|pages=492–504|pmid=19668244 |doi=10.1038/nrneurol.2009.118}}
17. ^eMedicine - Sudden Unexpected Death in Epilepsy : Article by Shahin Nouri
18. ^{{cite web|title=Epilepsy Facts|url=http://www.cureepilepsy.org/aboutepilepsy/facts.asp|publisher=Citizens United for Research in Epilepsy|accessdate=18 March 2014}}

External links

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  • {{cite journal|last1=Harden|first1=C|last2=Tomson|first2=T|last3=Gloss|first3=D|last4=Buchhalter|first4=J|last5=Cross|first5=JH|last6=Donner|first6=E|last7=French|first7=JA|last8=Gil-Nagel|first8=A|last9=Hesdorffer|first9=DC|last10=Smithson|first10=WH|last11=Spitz|first11=MC|last12=Walczak|first12=TS|last13=Sander|first13=JW|last14=Ryvlin|first14=P|title=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.|journal=Neurology|date=25 April 2017|volume=88|issue=17|pages=1674–1680|doi=10.1212/WNL.0000000000003685|pmid=28438841}}
  • Defining SUDEP at epilepsy.com
  • About SUDEP, at EpilepsyFoundation.org
  • SUDEP Action, UK at SUDEP.org
  • SUDEP Aware, Canada at SUDEPaware.org
  • SUDEP Global Conversation at SUDEPglobalconversation.com

2 : Epilepsy|Medical terminology
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