Two Concomitant Cases with Febrile Infection-Related Epilepsy Syndrome (FIRES) treated with andldquo;3-Dimensional Combination Treatment Protocolandrdquo;

Abstract

Objective: Febrile Infection-Related Epilepsy Syndrome (FIRES) is an immun-mediated epileptic encephalopathy presents as a febrile infection related super refractory status epilepticus with mortality rate of up to 30% in intensive care unit in spite of pharmacologically induced coma and immunotherapies. Early interventional ketogenic diet and cannabinoids are suggested for treatment with unknown mechanisms. Case presentation: Here we report two concomitant FIRES cases treated with a “3 dimensional combined treatment protocol“ for FIRES including (1); super refractory status epilepticus treatment protocol with intravenous infusion of antiepileptic and anesthetic drugs, (2) immunomodulatory therapies and (3) early interventional anti-epileptogenic therapy (ketogenic diet plus cannabinoids). The therapeutic intensity of the treatment protocol was monitored with serial EEG monitoring and Functional Independence Scores-WeeFIM. Two previously healthy school children manifesting super refractory status epilepticus were treated with moderate success. Both patients recovered with drug refractory epilepsy and neurocognitive impairments (patient 1; WeeFIM Scores were: self-care: 17, motor: 24, cognition: 10 and patient 2; WeeFIM score remained totally dependent: self-care: 8, motor: 5, cognition: 5) at the 12 months of follow-up. Conclusion: Febrile infection-related epilepsy syndrome is a devasting catastrophic epileptic encephalopathy. Early interventional treatment protocols should be applied in the early period of super refractory status epilepticus.