How targeted therapy can help overcome febrile infection-related epilepsy syndrome
In their findings, experts achieved a promising functional and neurologic outcome.
Febrile infection-related epilepsy syndrome (FIRES) is a severe neurological condition characterized by the sudden onset of seizures following a febrile illness, often leading to significant morbidity. In a recent study led by Dana Harrar, M.D., Ph.D., director of Critical Care Neurology at Children’s National Hospital, a team of experts followed the case of a 5-year-old previously healthy male who presented with acute neurological decline. This culminated in super-refractory status epilepticus following a fever.
The study outlines a novel treatment regimen that involved early and aggressive cytokine-directed immunomodulatory therapy, alongside a comprehensive seizure management strategy that intentionally avoided barbiturate use. By tailoring treatment to the patient’s evolving cytokine profile and incorporating supportive measures such as the ketogenic diet and antiseizure medications, experts achieved a promising functional and neurologic outcome.
“Now, 2½ years later, he attends mainstream school, has attention-deficit hyperactivity disorder (ADHD), mild neurocognitive impairment and well-controlled epilepsy,” the experts write. “By using cytokine-directed immunotherapy and avoiding a barbiturate coma, we were able to successfully treat a pediatric patient with febrile infection-related epilepsy syndrome and achieve a good outcome.”
The study’s findings show that the understanding of FIRES is advancing as experts uncover the underlying mechanisms of this challenging neurological disorder. Physician-scientists in this field developed innovative treatment strategies targeting immune dysregulation and associated seizures. By following recent consensus recommendations and utilizing molecularly targeted immunotherapy along with serial cytokine profiling, Dr. Harrar and team successfully treated the 5-year-old patient, leading to a positive neurological outcome.
You can read the full study, Successful Management of Febrile Infection-Related Epilepsy Syndrome Using Cytokine-Directed Therapy, in the Journal of Child Neurology.
