Effectiveness of Electroconvulsive Therapy for Refractory Status Epilepticus in Febrile Infection–Related Epilepsy Syndrome

Abstract

Febrile infection–related epilepsy syndrome (FIRES) is a rare condition which evolves into refractory status epilepticus (SE), with poor outcome in most cases. Conventional antiepileptic drugs fail to control SE in FIRES patients. We report the case of a previously healthy 4-year-old boy who was diagnosed with FIRES. One week after pharyngitis and high fever he started seizures, followed by refractory SE. Benzodiazepines, phenytoin, high-dose barbiturates that induce burst suppression, high doses of corticosteroids, plasmapheresis, immunoglobulins, propofol, lidocaine, ketamine, inhaled desflurane, ketogenic diet, lacosamide, and therapeutic hypothermia were tried at different times in a period of 8 weeks, but all of them were ineffective. Electroconvulsive therapy (ECT) has been used in refractory SE in children. We report a case in which ECT was successfully used for treatment of refractory SE in a pediatric patient with FIRES syndrome.

Keywords

FIRES - children - status epilepticus - refractory - electroconvulsive therapy

• References

• 1 van Baalen A, Stephani U, Kluger G, Häusler M, Dulac O. FIRES: febrile infection responsive epileptic (FIRE) encephalopathies of school age. Brain Dev 2009; 31 (1) 91-93 , author reply 92–93
• 2 van Baalen A, Häusler M, Boor R , et al. Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood. Epilepsia 2010; 51 (7) 1323-1328
• 3 Kramer U, Chi CS, Lin KL , et al. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia 2011; 52 (11) 1956-1965
• 4 Singh RK, Joshi SM, Potter DM, Leber SM, Carlson MD, Shellhaas RA. Cognitive outcomes in febrile infection-related epilepsy syndrome treated with the ketogenic diet. Pediatrics 2014; 134 (5) e1431-e1435
• 5 Nabbout R, Mazzuca M, Hubert P , et al. Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES). Epilepsia 2010; 51 (10) 2033-2037
• 6 Kamel H, Cornes SB, Hegde M, Hall SE, Josephson SA. Electroconvulsive therapy for refractory status epilepticus: a case series. Neurocrit Care 2010; 12 (2) 204-210
• 7 Griesemer DA, Kellner CH, Beale MD, Smith GM. Electroconvulsive therapy for treatment of intractable seizures. Initial findings in two children. Neurology 1997; 49 (5) 1389-1392
• 8 Shin HW, O'Donovan CA, Boggs JG , et al. Successful ECT treatment for medically refractory nonconvulsive status epilepticus in pediatric patient. Seizure 2011; 20 (5) 433-436
• 9 Caraballo RH, Reyes G, Avaria MF , et al. Febrile infection-related epilepsy syndrome: a study of 12 patients. Seizure 2013; 22 (7) 553-559
• 10 van Baalen A, Häusler M, Plecko-Startinig B , et al. Febrile infection-related epilepsy syndrome without detectable autoantibodies and response to immunotherapy: a case series and discussion of epileptogenesis in FIRES. Neuropediatrics 2012; 43 (4) 209-216
• 11 Capizzi G, Vittorini R, Torta F , et al. Lidocaine treatment in refractory status epilepticus resulting from febrile infection-related epilepsy syndrome: a case report and follow-up. Neuropediatrics 2015; 46 (1) 65-68
• 12 Sackeim HA. The anticonvulsant hypothesis of the mechanisms of action of ECT: current status. J ECT 1999; 15 (1) 5-26
• 13 Zeiler FA, Matuszczak M, Teitelbaum J, Gillman LM, Kazina CJ. Electroconvulsive therapy for refractory status epilepticus: a systematic review. Seizure 2016; 35: 23-32