A case of febrile infection-related epilepsy syndrome (FIRES) in young adult: still a diagnostic and therapeutic challenge

Abstract

A new onset of status epilepticus in a previously healthy adult preceded by a recent minor febrile infection represents a diagnostic and therapeutic challenge in clinical practice. Considering the broad spectrum of epileptic encephalopathies caused by autoimmune mechanisms, differential diagnosis for new-onset refractory status epilepticus (NORSE) should include febrile infection-related epilepsy syndrome (FIRES), in order to not underestimate the underlying etiological condition triggering epilepsy in non-epileptic patients (Hon et al. in Recent Pat Inflamm Allergy Drug Discov 12:128-135, 2018). We report a case of acute encephalopathy with refractory seizures after a febrile illness (FIRES) in a young adult with complete remission of symptoms as well as dramatic improvement of EEG abnormalities following intravenous immunoglobulin and proper antiepileptic medications. We conducted an extensive workup including lumbar puncture, blood tests, EEG serial monitoring, MRI brain, total body CT scan, and PET brain with FDG to shed light on the etiology of the disease.

Seizure burden and neuropsychological outcomes of new-onset refractory status epilepticus: Systematic review

“…While significant progress has been made in delineating the diagnostic and treatment approaches for NORSE, less emphasis has been placed on studying clinical outcomes, including the long-term sequalae, after the hospital discharge. Further, the literature focuses primarily on seizure outcomes, as refractory epilepsy represents the most significant disability in survivors of NORSE ( 1 , 2 , 6 – 12 , 16 – 99 ). However, over two-thirds of patients experience moderate to severe cognitive disability following hospitalization or remain in a vegetative state ( 5 , 6 , 92 , 93 , 99 ).…”

“…However, over two-thirds of patients experience moderate to severe cognitive disability following hospitalization or remain in a vegetative state ( 5 , 6 , 92 , 93 , 99 ). Reports concerning functional limitations after NORSE are sparse and include components of formal functional assessment or narrative descriptions of impaired academic performance or activities of daily living ( 1 , 2 , 6 – 12 , 16 – 99 ). The emergence of psychiatric and behavioral disturbances after NORSE have also been described; however, the full spectrum of these complications have not been systematically assessed outside of the time of hospitalization.…”