Chapter 8 - Sudden unexpected death in epilepsy: Respiratory mechanisms

Abstract

Epilepsy is one of the most common chronic neurologic diseases, with a prevalence of 1% in the US population. Many people with epilepsy live normal lives, but are at risk of sudden unexpected death in epilepsy (SUDEP). This mysterious comorbidity of epilepsy causes premature death in 17%-50% of those with epilepsy. Most SUDEP occurs after a generalized seizure, and patients are typically found in bed in the prone position. Until recently, it was thought that SUDEP was due to cardiovascular failure, but patients who died while being monitored in hospital epilepsy units revealed that most SUDEP is due to postictal central apnea. Some cases may occur when seizures invade the amygdala and activate projections to the brainstem. Evidence suggests that the pathophysiology is linked to defects in the serotonin system and central CO2 chemoreception, and that there is considerable overlap with mechanisms thought to be involved in sudden infant death syndrome (SIDS). Future work is needed to identify biomarkers for patients at highest risk, improve ascertainment, develop methods to alert caregivers when SUDEP is imminent, and find effective approaches to prevent these fatal events.