Possible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report

Abstract

We describe the efficacy of high-dose barbiturates and early administration of a parenteral ketogenic diet (KD) as initial treatments for acute status epilepticus (SE) in an 8-year-old girl with febrile infection-related epilepsy syndrome (FIRES). The patient was admitted to our hospital with refractory focal SE. Abundant epileptic discharges over the left frontal region were observed on electroencephalogram (EEG). Treatment with continuous infusion of thiamylal for 4 hours, increased incrementally to 40 mg/kg/h, successfully ended the clinical SE, and induced a burst-suppression coma. The infusion rate was then gradually decreased to 4 mg/kg/h over the next 12 hours. Parenteral KD was administered from days 6 to 21 of illness. Continuous infusion of thiamylal was switched to midazolam on day 10 without causing seizures or EEG exacerbations. The patient has remained seizure free in the 15 months since hospital discharge. The effectiveness of KD for the treatment of FIRES has attracted attention amongst clinicians, but KD treatment may need to last for 2 to 4 days before it can stop SE, a time period that could cause irreversible brain damage. Considering the severity of SE in our patient and the dose of barbiturates needed to treat it, we consider this case to have had a good clinical outcome. The results suggest that rapid termination of seizure using high-dose barbiturates in conjunction with early administration of parenteral KD could reduce the development of chronic epilepsy in patients with FIRES.

Case report: Febrile infection-related epilepsy syndrome in a 14-year-old girl with multiple organ failure and lethal outcome

“…Treatment focused on aggressive antiepileptic therapy, establishing a KD, and starting steroids; those therapies were described as most effective at that time ( Gaspard et al, 2018 ; Lin et al, 2020 ; Baba et al, 2021 ). However, treatment of seizures in cryptogenic FIRES is described as difficult, with seizure activity lasting several weeks with little or no response to antiepileptic treatment and anesthetics often being required ( Rachfalska et al, 2021 ; Cerovic et al, 2023 ).…”

Enteral lorazepam is a promising weaning strategy for midazolam‐responsive febrile infection‐related epilepsy syndrome (FIRES): a case series

“…In these patients, prolonged periods of pharmacological coma to control electrographic and electroclinical seizures are often required [8]. Usually, gamma-aminobutyric acid (GABA) mimetic agents, such as midazolam, thiopentone and high-dose phenobarbitone, have been the more effective agents [17][18][19][20]. Among these, midazolam is usually the first • 673 used anti-seizure medication for pharmacological coma, though with variable efficacy [17][18][19]21].…”

Calculation and management of ketogenic diet parenteral nutrition in super-refractory status epilepticus

“…KD-PN was first applied in 1990 to a 5-year-old girl with Lennox-Gastaut syndrome [9]. From 1990 to 2020, 6 retrospective studies [10][11][12][13][14][15] and 8 case reports [9,[16][17][18][19][20][21][22] have reported the use of KD-PN for epilepsy, involving a total of 47 patients (Fig. 1).…”