Practice guideline summary: Sudden unexpected death in epilepsy incidence rates and risk factors

Abstract

Objective: To determine the incidence rates of sudden unexpected death in epilepsy (SUDEP) in different epilepsy populations and address the question of whether risk factors for SUDEP have been identified.Methods: Systematic review of evidence; modified Grading Recommendations Assessment, Development, and Evaluation process for developing conclusions; recommendations developed by consensus.Results: Findings for incidence rates based on 12 Class I studies include the following: SUDEP risk in children with epilepsy (aged 0-17 years) is 0.22/1,000 patient-years (95% confidence interval [CI] 0.16-0.31) (moderate confidence in evidence). SUDEP risk increases in adults to 1.2/ 1,000 patient-years (95% CI 0.64-2.32) (low confidence in evidence). The major risk factor for SUDEP is the occurrence of generalized tonic-clonic seizures (GTCS); the SUDEP risk increases in association with increasing frequency of GTCS occurrence (high confidence in evidence).Recommendations: Level B: Clinicians caring for young children with epilepsy should inform parents/ guardians that in 1 year, SUDEP typically affects 1 in 4,500 children; therefore, 4,499 of 4,500 children will not be affected. Clinicians should inform adult patients with epilepsy that SUDEP typically affects 1 in 1,000 adults with epilepsy per year; therefore, annually 999 of 1,000 adults will not be affected. For persons with epilepsy who continue to experience GTCS, clinicians should continue to actively manage epilepsy therapies to reduce seizures and SUDEP risk while incorporating patient preferences and weighing the risks and benefits of any new approach. Clinicians should inform persons with epilepsy that seizure freedom, particularly freedom from GTCS, is strongly associated with decreased SUDEP risk. This document summarizes information provided in the complete guideline, available at Neurology.org. Appendix e-6, cited in the full guideline (data supplement), is available at Neurology.org.Sudden unexpected death in epilepsy (SUDEP) is a poorly understood and catastrophic risk of epilepsy.The sensitive nature of discussions of this infrequent but important risk with patients and families has prompted the need for evidence-based information about SUDEP. The goal of this practice guideline is to examine evidence for the SUDEP incidence rate in epilepsy populations and for prognostic factors

Brainstem network disruption: A pathway to sudden unexplained death in epilepsy?

“…SUDEP is the leading cause of death in 10-50% of the patients with chronic drug-resistant epilepsy and is only surpassed by stroke in total potential years of life lost due to a neurological disease (Devinsky, Hesdorffer, Thurman, Lhatoo, & Richerson, 2016;Jones & Thomas, 2017;Thurman, Hesdorffer, & French, 2014;Harden et al, 2017;Sveinsson, Andersson, Carlsson, & Tomson, 2017). The death is not related to a status epilepticus, drowning or an accident, and autopsy does not provide a cause.…”

“…The death is not related to a status epilepticus, drowning or an accident, and autopsy does not provide a cause. SUDEP is the leading cause of death in 10-50% of the patients with chronic drug-resistant epilepsy and is only surpassed by stroke in total potential years of life lost due to a neurological disease (Devinsky, Hesdorffer, Thurman, Lhatoo, & Richerson, 2016;Jones & Thomas, 2017;Thurman, Hesdorffer, & French, 2014;Harden et al, 2017;Sveinsson, Andersson, Carlsson, & Tomson, 2017). Increased awareness of the SUDEP-related excess mortality in epilepsy patients has led to an intensification of the research efforts to unravel its cause and to the insight that SUDEP is a complex phenomenon that is unlikely to be explained by a single mechanism (Dlouhy, Gehlbach, & Richerson, 2016).…”

Morte Súbita em Epilepsia - O que é preciso saber?

“…A frequência anual das CTCGs, por sua vez, aumenta em até 5 vezes a probabilidade do desfecho em pacientes com um a dois episódios dessas crises anualmente, saltando para até 15 vezes nos casos de três ou mais crises anuais 20,24,33,[42][43][44][45][46] . Esse incremento no risco relativo se traduz para um risco absoluto de até 18 mortes a cada 1.000 pacientes/ano para pessoas com CTCGs frequentes (≥ 3 crises/ano) 34,46 .…”

“…Doença de longa data também afeta o prognóstico desses indivíduos. Os pacientes com tempo de doença superior a 15 anos possuem um risco até 1,95 vez superior quando comparado à população saudável de mesma faixa etária 20,[32][33][34][35][36][37] .…”

“…Uma vez que a frequência das CTCGs é o principal fator de risco da SUDEP, fica claro que o adequado manejo das crises é indispensável para sua prevenção 33,34,88 , de forma proativa, incluindo otimização do tratamento e encaminhamento a serviços especializados 33 . Em indivíduos com epilepsia refratária, por exemplo, a terapia otimizada em doses adequadas de DAEs pode reduzir em até 7 vezes o desfecho da SUDEP quando comparado ao grupo placebo 33,88 .…”