Rachel Sawdy, CPNP, , MSN, APNP
Pediatric Neurology Nurse Practitioner
Medical College of Wisconsin
Milwaukee, Wisconsin
Disclosure information not submitted.
Kathleen Maloney, MSN, APNP, CPNP-AC
Pediatric Critical Care Nurse Practitioner
Medical College of Wisconsin, Wisconsin, United States
Disclosure information not submitted.
Title: Extinguishing FIRES: Intrathecal Dexamethasone for Febrile Infection-Related Epilepsy Syndrome
Introduction: Febrile infection-related epilepsy syndrome (FIRES) is a rare clinical presentation, often characterized as a cytokine storm, that impacts previously healthy children. It resembles an epileptic encephalopathy that is extremely refractory to most standard treatments. The pathogenesis remains elusive, and mortality and morbidity remains high. Anakinra is the one of most utilized treatments, but the literature lacks alternative treatments when refractory to anakinra.
Description: A 9-year-old, previously healthy male, was admitted with acute encephalopathy after being diagnosed with pharyngitis 6 days prior to presentation. Over the following 2 weeks, his neurological exam deteriorated with increased electroclinical and electrographic seizures refractory to traditional medical therapies including multiple anti-epileptic drugs, three anesthetic infusions, steroids, IVIG, and plasmapheresis. On hospital day 14, the decision was made to start anakinra for the treatment of FIRES. Unfortunately, despite this, he continued to have frequent seizures. In a small Japan study, 6 patients with FIRES received intrathecal dexamethasone (IT-DEX), resulting in decreased seizure activity, ability to wean anesthetic infusions without significant increase in seizure burden, and to hypothesize decreased duration of critical illness. Given the success of this study, on hospital day 23, he received his first dose of IT-DEX. Over 2 weeks, he received 5 total doses and was weaned off all anesthetic infusions with an improved electroencephalogram (EEG). He was successfully extubated and began intensive rehabilitation. Overall, compared with the reported poor outcomes of FIRES, our patient did remarkably well.
Discussion: FIRES is a neurologically devastating syndrome with high mortality and morbidity in otherwise previously healthy children. Proposed guidelines and various treatment strategies are slowly becoming more available in the literature. While treatment with anakinra has been successful in previous FIRES cases, our results suggest that the addition of IT-DEX may allow for quicker weaning of anesthetic infusions and better cognitive outcomes when initiated early in the treatment course. Our case is the first reported use of IT-DEX for treatment of FIRES in the United States.