Background: Febrile infection-related epilepsy syndrome (FIRES) is a severe epileptic encephalopathy with acute onset following a febrile disease in previously healthy children. Its etiology has not been elucidated yet. There is no treatment algorithm with a positive response. Here, we aimed to present the summary of literature in terms of our experience in FIRES management in a secondary care hospital in the Southeastern Anatolia and immunotherapy outcomes.
Cases: We report three FIRES-associated super-refractory status epilepticus cases followed up by the Pediatric Neurology Unit at Mardin State Hospital. The cases known to be healthy were hospitalized due to focal seizures and seizures with secondary generalization which started after a febrile disease which had developed 3-5 days before. Since seizures persisted for more than 24 hours despite the effective treatment, the super-refractory status epilepticus was accepted. No infectious agent was detected. Progression to chronic refractory epilepsy with cognitive impairment was observed. Two patients received intravenous immunoglobulin (IVIG) and steroids, other received only IVIG. IVIG was continued monthly. The time of the first administration of IVIG was days 2-4. Partial response was obtained within 2-7 days. Monthly IVIG was observed to be protective against status epilepticus attacks triggered by fever. Although all cases received similar treatments at similar times, different outcomes from each other developed. One of them died, the others developed sequelae.
Conclusion: FIRES is a condition that can cause super-refractory status epilepticus. Although IVIG treatment alone does not appear to be effective in the acute phase, regular IVIG treatment may prevent recurrent exacerbations.