Does sudden unexpected death in children with epilepsy occur more frequently in those with high seizure frequency?

Abstract

-People with epilepsy are more likely to die prematurely and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP). Several studies have reported a moderate or high seizure frequency among SUDEP cases and SUDEP is considered rare in patients in remission. Method: We reviewed the occurrence of SUDEP in our epilepsy unit over an 8-year period to identify a potential association between seizure frequency and SUDEP occurrence in children with epilepsy. Results: From 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children are related to chronic uncontrolled epilepsy (daily -50.0%, two to four/week -41.7%, monthly -8.3%). Conclusion: SUDEP is not a rare event in children and increased mortality was recorded in those individuals who had not responded to pharmacologic treatment. Improved seizure control seems to be one of the most important measures to prevent SUDEP.KEy WorDS: epilepsy, children, sudden death, seizure frequency. Morte súbita em crianças com epilepsia ocorre naquelas que apresentam maior frequência de crises?Resumo -Pessoas com epilepsia têm maior chance de morrer prematuramente e a principal causa de morte relacionada à epilepsia é a morte súbita em epilepsia (SUDEP). Vários estudos têm relatado uma freqüência de crises moderada ou elevada em pacientes com SUDEP e SUDEP é considerada rara em pacientes em remissão. Método: revisamos a ocorrência de SUDEP em nossa unidade de epilepsia por um período de oito anos com o objetivo de identificar uma possível associação entre freqüência de crises e a ocorrência de SUDEP em crianças com epilepsia. Resultados: De 835 pacientes avaliados, 12 evoluíram com SUDEP e a maioria das crianças apresentava epilepsia crônica não controlada (50% com crises diárias; 41,7% com duas a quatro crises/ semana; 8,3% com crises mensais). Conclusão: SUDEP não é um evento raro em crianças e maior mortalidade foi observada em indivíduos que não responderam ao tratamento medicamentoso. Melhor controle de crises parece ser uma das principais medidas na prevenção de SUDEP.PAlAVrAS-chAVE: epilepsia, crianças, morte súbita, frequência de crises. People with epilepsy are more likely to die prematurely than those without epilepsy and the most common epilepsy-related category of death is sudden unexpected death in epilepsy (SUDEP) 1,2 . A number of associated factors for SUDEP have been reported but the results are not wholly consistent between studies. These include presence of generalized tonic-clonic seizures, polytherapy with antiepileptic drugs, young age, duration of the seizure disorder ranging from 15 to 20 years, early onset of epilepsy and more-frequent seizures 1,2 . In line with the latter risk factors, the impact of high seizure frequency on the risk of SUDEP has been evaluated from different angles: most studies including information on seizure control have reported a moderate or high sei-

Decreased heart rate and enhanced sinus arrhythmia during interictal sleep demonstrate autonomic imbalance in generalized epilepsy

“…A recent longitudinal study that followed children with epilepsy undergoing pharmacologic treatment over several years concluded that "SUDEP is not a rare event in children" (Terra et al 2009). As 1.4% of the subjects succumbed to SUDEP during the study, the authors noted that premature mortality rates associated with SUDEP could diminish if more pediatric research were conducted.…”

Sudden death in a child with epilepsy: potential cerebellar mechanisms?

“…Although few data exist about the risk of SUDEP in children, a recent study by our research group demonstrated that increased SUDEP occurrence appeared in children unresponsive to antiepileptic therapy, with no increase in risk observed in patients who remained seizure free 31 . An additional important issue is evidence that phenytoin can exert toxic effects on the cerebellum in patients with epilepsy 32 ; the toxicity is associated with ataxia, tremor, nystagmus and diplopia 32,33 .…”

Epileptologists probe vagus nerve stimulation in children with refractory epilepsy: a promise against sudden unexpected death in epilepsy

“…Briefly, from the 835 patients evaluated, 12 had suffered SUDEP and nearly all of the SUDEP cases in our children were related to chronic uncontrolled epilepsy (daily -50.0%, two to four/weeks -41.7%, monthly -8.3%). Furthermore, the high frequency of generalized tonic-clonic seizures and polytherapy with antiepileptic drugs may also be highlighted as risk factors in our study 19 . By assessing the information set out so far and considering that although the availability of pharmacological and surgical treatment for epilepsy has expanded, antiepileptic drugs and some resective procedures are still limited in clinic efficacy.…”

“…According to the excellent review article published recently by Sascha Meyer et al 18 , epileptic children and adolescents with refractory seizures, early-onset epilepsy, and developmental retardation seem to be at higher risk for SUDEP. Following this line of reasoning, we reviewed the occurrence of SUDEP in children in our epilepsy unit over an eight-year period 19 . Our study evaluated SUDEP incidence in a cohort of children aged between 0 and 18 years-old, who were evaluated in the Clinical Hospital of Ribeirão Preto in 2000 and followed-up until June, in 2008.…”

“…Following this line of reasoning, we reviewed the occurrence of SUDEP in children in our epilepsy unit over an eight-year period 19 . Our study evaluated SUDEP incidence in a cohort of children aged between 0 and 18 years-old, who were evaluated in the Clinical Hospital of Ribeirão Preto in 2000 and followed-up until June, in 2008.…”