Adult-Onset Febrile Infection-Related Epilepsy Syndrome Treated with Short-Term Anakinra.

Abstract

In this case report, we discuss the oldest patient, at 47-year-old, on record to receive anakinra in treatment for febrile infection-related epilepsy syndrome (FIRES). Additionally, our patient was treated with a shorter course compared to that seen in the current literature. FIRES is rarely seen in adults and remains an area of investigation for best treatment practices due to the refractory and often devastating clinical course. Anakinra is a recombinant interleukin-1 receptor antagonist that effectively targets 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 in focal status epilepticus after 1 week of febrile illness. Focal status epilepticus remained refractory despite these of multiple anti-seizure medications, anesthetics, and steroids. 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. After anakinra initiation, sedative medications were fully weaned within 8 days and all epileptiform activity on electroencephalogram resolved within 2 weeks. The patient eventually returned to his prior cognitive baseline and achieved approximately 1 year of seizure freedom. These outcomes support the use of anakinra in treating adults with FIRES. Further studies with a focus on determining the underlying mechanism that accounts for variability in patient response to anakinra are essential. Such studies may aid in the development of ideal dosing and therapy duration of anakinra in FIRES.