Prevention of sudden unexpected death in epilepsy: current status and future perspectives

ABSTRACT

Introduction: Sudden unexpected death in epilepsy (SUDEP) affects about 1 in 1000 people with epilepsy, and even more in medically refractory epilepsy. As most people are between 20 and 40 years when dying suddenly, SUDEP leads to a considerable loss of potential life years. The most important risk factors are nocturnal and tonic-clonic seizures, underscoring that supervision and effective seizure control are key elements for SUDEP prevention. The question of whether specific antiepileptic drugs are linked to SUDEP is still controversially discussed. Knowledge and education about SUDEP among health-care professionals, patients, and relatives are of outstanding importance for preventive measures to be taken, but still poor and widely neglected.

Areas covered: This article reviews epidemiology, pathophysiology, risk factors, assessment of individual SUDEP risk and available measures for SUDEP prevention. Literature search was done using Medline and Pubmed in October 2019.

Expert opinion: Significant advances in the understanding of SUDEP were made in the last decade which allow testing of novel strategies to prevent SUDEP. Promising current strategies target neuronal mechanisms of brain stem dysfunction, cardiac susceptibility for fatal arrhythmias, and reliable detection of tonic-clonic seizures using mobile health technologies.

Abbreviations: AED, antiepileptic drug; CBZ, carbamazepine; cLQTS, congenital long QT syndrome; EMU, epilepsy monitoring unit; FBTCS, focal to bilateral tonic-clonic seizures; GTCS, generalized tonic-clonic seizures; ICA, ictal central apnea; LTG, lamotrigine; PCCA, postconvulsive central apnea; PGES, postictal generalized EEG suppression; SRI, serotonin reuptake inhibitor; SUDEP, sudden unexpected death in epilepsy; TCS, tonic-clonic seizures.

KEYWORDS:

• Epilepsy
• sudden death
• antiepileptic drugs
• lamotrigine
• tonic-clonic seizures
• prevention
• premature mortality
• seizure detection devices

Article highlights

• SUDEP is a rare but fatal complication of epilepsy

• The majority of SUDEP cases are linked to epileptic seizures, a minor portion occurs in the absence of signs of epileptic seizures

• SUDEP mostly occurs in association with nocturnal and generalized or focal to bilateral tonic-clonic seizures

• The lethal, probably reversible cascade includes postictal central apnea followed by bradyarrhythmia and asystole

• Effective seizure control and nocturnal supervision are key elements to prevent SUDEP in most cases

• Automatic seizure detection devices are likely to reduce the SUDEP risk

• Machine learning algorithms and novel wearable technologies may improve seizure prediction and detection, significantly boosting SUDEP prevention

• Education of patients, relatives, and health-care professionals about SUDEP is highly recommended

Declaration of interest

D Aurlien has nothing to disclose. RD Nass has received fees for lectures and consultancy from the following companies: Eisai. R Surges has received fees for lectures and consultancy from the following companies: Bial, Desitin, Eisai, LivaNova, Novartis and UCB Pharma. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or conflict with the subject matter or materials discussed in this manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.