In this case report, we discuss the oldest patient, at 47-years-old, on record to receive anakinra in treatment for FIRES. Additionally, our patient was treated with a shorter course compared to that seen in the current literature.

Febrile infection-related epilepsy syndrome (FIRES) is a presentation rarely seen in adults and remains an area of investigation for best treatment practices due to the refractory and often devastating nature of the clinical course. Anakinra is a recombinant interleukin-1 receptor antagonist that has been shown to effectively target central nervous system inflammation implicated in the pathogenesis of FIRES. The current literature regarding anakinra use in FIRES mostly represents the pediatric population with dose schedules continued into the chronic phase of the disease. There is a dearth of information regarding the response to anakinra in adult FIRES patients as well as the appropriate treatment duration. 

This patient presented with seizures after one week of febrile illness and was found to be in focal status epilepticus on EEG. Focal status epilepticus remained refractory despite the use of multiple anti-seizure medications, anesthetics, steroids, and IVIg. An extensive workup yielded no clear underlying etiology to account for his presentation. Anakinra was started 9 days after seizure onset and continued for 2 weeks. Sedative medications were fully weaned 8 days after anakinra initiation and all epileptiform activity on EEG resolved within 2 weeks of anakinra initiation. The patient eventually returned to his prior cognitive baseline and achieved approximately one year of seizure freedom. 

These outcomes provide support for the use of anakinra in the treatment of adults with FIRES. Further studies with a focus on determining the underlying mechanism that accounts for variability in patient response to anakinra is essential. Such studies may aid in the development of ideal dosing and therapy duration of anakinra in FIRES.