Cecilia Westbrook, MD, PhD; Thanujaa Subramaniam, MD; Ryan M. Seagren, PharmD; Erick Tarula, MD; Dominic Co, MD; Meghan Furstenberg-Knauff, APNP; Adam Wallace, MD; David Hsu, MD, PhD; Eric Payne, MD
WMJ. 2019;118(3):135-139.
Abstract
Introduction: Febrile infection-related epilepsy syndrome (FIRES) is a syndrome of new-onset status epilepticus preceded by fever and highly refractory to treatment, thus resulting in high mortality and severe neurologic morbidity in surviving patients. Anakinra is an IL-1 receptor antagonist that has previously demonstrated efficacy in treating children with FIRES.
Case Presentation: A 21-year-old previously healthy woman presented with new-onset superrefractory status epilepticus following a febrile illness. This was subsequently diagnosed as FIRES after an extensive evaluation failed to identify an alternative etiology. The patient’s seizures were refractory to numerous antiepileptic drugs and immunomodulatory therapy. She was maintained under pharmacologic sedation for 31 days.
Management and Outcome: Anakinra was initiated on day 32 of her hospital stay, with swift and complete remission of her status epilepticus. Seizures ceased within 24 hours. The patient remains in remission with minimal side effects from the medication and no known long-term morbidity.
Discussion: Here we report what we believe is the second case of super-refractory status epilepticus due to FIRES responding to anakinra, and the first such case in an adult patient. Anakinra was well tolerated with few side effects. Our results are further evidence for the autoinflammatory nature of FIRES and support the use of anakinra early in the treatment to prevent long-term sequelae.
Author Affiliations: University of Wisconsin School of Medicine and Public Health, Madison, Wis (Subramaniam, Seagren, Tarula, Co, FurstenbergKnauff, Wallace, Hsu); University of Pittsburgh Medical Center, Pittsburgh, Penn (Westbrook); Mayo Clinic, Rochester, Minn (Payne).
Corresponding Author: David Hsu, MD, PhD, Department of Neurology, University of Wisconsin School of Medicine and Public Health, 7th floor MFCB, 1685 Highland Ave, Madison, WI 53705-2281; phone 608.263.5421; email hsu@neurology.wisc.edu.
Funding/Support: None declared.
Financial Disclosures: None declared.