Mortality Risks in New-Onset Childhood Epilepsy

Abstract

WHAT'S KNOWN ON THIS SUBJECT: Seizure-related death, including sudden death, is a frightening prospect. In part because risk and prevention are poorly understood, neurologists tend to avoid discussions of sudden death with families and young patients. WHAT THIS STUDY ADDS:Most deaths in children with epilepsy are not seizure related. Relative to the population, however, sudden and seizure-related deaths alone double overall mortality. In uncomplicated epilepsy, such deaths occur at rates comparable to individual leading causes of death in young people. abstract OBJECTIVES: Estimate the causes and risk of death, specifically seizure related, in children followed from onset of epilepsy and to contrast the risk of seizure-related death with other common causes of death in the population. METHODS:Mortality experiences from 4 pediatric cohorts of newly diagnosed patients were combined. Causes of death were classified as seizure related (including sudden unexpected death [SUDEP]), natural causes, nonnatural causes, and unknown.RESULTS: Of 2239 subjects followed up for .30 000 person-years, 79 died. Ten subjects with lethal neurometabolic conditions were ultimately excluded. The overall death rate (per 100 000 person-years) was 228; 743 in complicated epilepsy (with associated neurodisability or underlying brain condition) and 36 in uncomplicated epilepsy. Thirteen deaths were seizure-related (10 SUDEP, 3 other), accounting for 19% of all deaths. Seizure-related death rates were 43 overall, 122 for complicated epilepsy, and 14 for uncomplicated epilepsy. Death rates from other natural causes were 159, 561, and 9, respectively. Of 48 deaths from other natural causes, 37 were due to pneumonia or other respiratory complications.CONCLUSIONS: Most excess death in young people with epilepsy is not seizure-related. Mortality is significantly higher compared with the general population in children with complicated epilepsy but not uncomplicated epilepsy. The SUDEP rate was similar to or higher than sudden infant death syndrome rates. In uncomplicated epilepsy, sudden and seizure-related death rates were similar to or higher than rates for other common causes of death in young people (eg, accidents, suicides, homicides). Relating the risk of death in epilepsy to familiar risks may facilitate discussions of seizurerelated mortality with patients and families. Pediatrics 2013;132:124-131 AUTHORS:

Long‐term mortality of patients with West syndrome

“…All patients who died of pneumonia had symptomatic West syndrome. Our percentage of deaths due to pneumonia (47%) is well comparable with 50% of Berg et al1 …”

“…Ten of 13 deaths (77%) were associated with underlying cause of the seizures, and both symptomatic etiology and epileptic encephalopathy were independently associated with mortality 18. Among 2,239 participants followed for more than 300,000 person‐years, mortality was significantly higher in those with complicated epilepsy, whereas participants with uncomplicated epilepsy had no significant excess mortality compared with the general population 1. In a Finnish population study of 245 followed for 40 years,14 60 participants died.…”

“…SUDEP was found in 10% of all deceased participants or 2% of those who died before age 16. Much lower percentages of SUDEP of all deaths (<1%) have been presented 1, 19. The wide variation is probably a consequence of different definitions, inclusion criteria, and duration of follow‐up; the longer the follow‐up of childhood‐onset epilepsy, the higher the probability of SUDEPs.…”

Children’s Experiences of Epilepsy: A Systematic Review of Qualitative Studies

“…1,2 Up to 50% of children with epilepsy have symptomatic epilepsy, which is associated with a 10-fold increased mortality risk. 3 Morbidity and mortality are mainly attributable to injury, developmental comorbidities, and an increased risk of sudden death that persists into adulthood. 2, 4 -6 Although some children achieve remission, the overall risk of recurrence is close to 50%.…”

Rufinamide in patients with Lennox-Gastaut syndrome

“…19 In addition, the risk of sudden unexpected death in epilepsy is higher in patients with complicated forms of epilepsy (those associated with the presence of known structural brain lesion, abnormal neurologic examination, or significant intellectual disability), such as LGS, relative to patients with uncomplicated epilepsy (ie, in the absence of these factors). 20,21 Overall, LGS has a significant impact on the health-related QoL (HRQoL) of patients and their caregivers. 22 LGS is difficult to diagnose, in part due to its varied etiologies.…”