Sudden unexpected death in epilepsy patients is often misdiagnosed as sudden cardiac death

Sudden unexpected death in epilepsy patients (SUDEP) represents the second main neurologic cause of years of potential life lost, following stroke, with a cumulative lifetime risk reaching 8% for childhood-onset epilepsy.1 In light of these worrying figures, the American Academy of Neurology (AAN) has recently published practice guidelines on the risk of SUDEP. These guidelines emphasize the “considerable uncertainty”of SUDEP incidence in adults with epilepsy, currently estimated at 1.2/1,000 patient-years (95% CI 0.64–2.32).2 Difficulties in assessing the epidemiology of SUDEP are several, including large variation in SUDEP risk across epilepsy populations (up to 100-fold difference between newly diagnosed and refractory epilepsy)3 and a lack of a SUDEP code in the current International Classification of Diseases (ICD). As a result, SUDEP is underreported and underrecognized by medical examiners, coroners, and clinicians who complete death certificates.4 In fact, many physicians view SUDEP as a sudden cardiac death (SCD), for which the relation to seizures is often uncertain. This view is at odds with evidence gathered during the last decade, suggesting that most SUDEP occurs in the immediate aftermath of a generalized tonic-clonic seizure through centrally mediated autonomic failure, distinct from mechanisms at stake in SCD.5 Progress in distinguishing SUDEP from the general framework of SCD is thus needed.