Introduction Febrile infection-related epilepsy syndrome (FIRES) is a condition characterized by new onset super refractory status epilepticus triggered by a febrile infection in a previously healthy child with a generally poor prognosis. We report a case of FIRES in a previously healthy 8-year-old girl with a favourable outcome due to early institution of the interleukin-6 inhibitor Tocilizumab. Methods The patient was admitted with acute onset focal seizures progressing to super refractory status epilepticus (SRSE) after a three day history of fever and sore throat. Her EEG showed electrographic seizures arising predominantly from the left hemisphere. CSF analysis, autoimmune panel and infection work-up were negative. Despite escalating treatment with multiple anti-seizure medications(ASMs) along with high dose bolus phenobarbitone, Midazolam and Ketamine infusions, immunotherapy, seizures remained refractory. Her serum interleukin-6 levels were significantly elevated while interleukin1 was normal. On day 20 of hospitalization, Tocilizumab was started at a dose of 12mg/kg every 2weeks, with a total of three doses given over six weeks. Results Tocilizumab led to a significant reduction in electroclinical seizure burden within one week of initiation, allowing the gradual discontinuation of other infusions. The patient was discharged home in good health after 2 weeks. At three-month follow-up, she remains well, active on 3 ASMs with good cognitive function, full independence in daily routines, online school attendance with only rare focal unaware seizures at a frequency of 1 to 2 short seizures per month. Conclusion This case highlights the importance of considering Tocilizumab early in the management of FIRES.

Sukanya Vrushabhendra
Al Jalila Childrens Specialty hospital
United Arab Emirates

Lma Mohamed
Al Jalila Childrens Specialty hospital
United Arab Emirates

Samar Almuntaser
Al Jalila Childrens Specialty hospital
United Arab Emirates

Hesham Obeidat
Al Jalila Childrens Specialty hospital
United Arab Emirates

Anjaly Nair
Al Jalila Childrens Specialty hospital
United Arab Emirates

Ali Elisir
Al Jalila Childrens Specialty hospital
United Arab Emirates

Nidheesh Chencheri
Al Jalila Childrens Specialty hospital
United Arab Emirates

Walid Abuhammour
Al Jalila Childrens Specialty Hospital
United Arab Emirates

Mohamed Babiker
Al Jalila Childrens Specialty Hospital
United Arab Emirates

Pawan Kashyape
Al Jalila Childrens Specialty Hospital
United Arab Emirates