Sudden unexpected death in epilepsy. A critical view of the literature

Angela Neve, Giorgia Giussani, Ettore Beghi, Lice Sudep, Oriano Mecarelli, Pasquale Striano, Paolo Tinuper, Ettore Beghi
2016 Clinical Cases and Reviews in Epilepsy   unpublished
Sudden unexplained death in epilepsy (SUDEP) is a sudden, unexpected, witnessed or unwitnessed, nontraumatic and nondrowning death, occurring in benign circumstances, in an individual with epilepsy, with or without evidence for a seizure and excluding documented status epilepticus (seizure duration ≥30 minutes or seizures without recovery in between), in which postmortem examination does not reveal a cause of death. Lower diagnostic levels are assigned when cases met most or all of these
more » ... all of these criteria, but data suggested more than one possible cause of death. The incidence of SUDEP ranged from 0.09 to 2.4 per 1,000 person years. Differences can be attributed to the age of the study populations (with peaks in the 20-40 yr age group) and the severity of the disease. Young age, disease severity (in particular, a history of generalized tonic-clonic seizures), having symptomatic epilepsy, and the response to AEDs are possible independent predictors of SUDEP. The pathophysiological mechanisms are not fully known due to the limited data available and because SUDEP is not always witnessed and has been electrophysiologically monitored only in few cases with simultaneous assessment of respiratory , cardiac and brain activity. The pathophysiolog-ical basis of SUDEP may vary according to different circumstances that make that particular seizure, in that specific moment and in that patient a fatal event. The main hypothesized mechanisms are cardiac dysfunction (included potential effects of antiepileptic drugs, genetically determined chan-nelopathies, acquired heart diseases), respiratory dysfunction (included postictal arousal deficit for the respiratory mechanism, acquired respiratory diseases) and postictal EEG depression. KEY WORDS: epilepsy, SUDEP, incidence, patho-physiological mechanisms. Definitions Sudden unexpected death in epilepsy (SUDEP) is "sudden, unexpected, witnessed or unwitnessed, non-traumatic and non-drowning death in patients with 54
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