PGR – 1 Neurorehabilitation of a Pediatric Patient Diagnosed with Febrile Infection-Related Epilepsy Syndrome (FIRES)

Abstract Objective Given its complexity and rarity, pediatric autoimmune encephalitis (AE) presents a significant challenge to clinicians. AE can precipitate febrile infection-related epilepsy syndrome (FIRES), further complicating a patient’s course. This case study will explore the progression of neurocognitive recovery across inpatient and outpatient settings to inform future research and clinical management of FIRES. Method Previously healthy, the current patient was a 6-year-old White non-Hispanic/Latine monolingual English-speaking female who initially presented with status epilepticus in the setting of a fever. Initial electroencephalogram (EEG) captured numerous left temporal focal seizures, and brain magnetic resonance imaging (MRI) revealed edema within the fornix-fimbria complex. Unremarkable lumbar puncture and infectious studies suggested her course was most consistent with FIRES. Results The patient was administered serial neurocognitive screening measures during her inpatient stay, targeted discharge neuropsychological testing, and comprehensive outpatient follow-up neuropsychological testing (see Appendix). Upon admission, initial screening was consistent with a minimally conscious state. She made gradual progress while inpatient and discharge testing revealed vulnerabilities in attention, executive functioning, and learning/memory. Outpatient testing approximately seven months post-discharge revealed below-average intellectual ability, with impairments in academics, cognitive efficiency, disruptive behavior, and fine motor skills. Diagnostic impressions included attention-deficit/hyperactivity disorder (ADHD) and a mild neurocognitive disorder. Conclusions Although the mechanism of neurocognitive difficulties in children with FIRES remains unclear, the current case study highlights the unique benefit of multifaceted rehabilitative care in managing poly-etiologic factors and the progression of recovery in patients with FIRES. Unfortunately, many patients do not return to their premorbid level of functioning; thus, future research is essential.