We describe 8 patients admitted to Texas Children’s Hospital between 2017 and 2019 with a diagnosis of FIRES, their acute neurologic presentation and treatments, and long term neurologic and neurodevelopmental outcomes.

Mean age of presentation was 8.5 years (range 4-15 years). Mean seizure onset 3.6 days from febrile illness (range 1-6 days).  All patients required anesthetics for seizure control in addition to antiepileptics.  Immunotherapy included varying regimens of IVMP, IVIG, plasmapheresis, rituximab, and/or anakinra. The mean duration in burst suppression was 20.9 days (range 0-120).  Mean hospital stay was 109 days (range 48-260 days).  Neurocognitive function varied at discharge, with greater deficits seen in those with longer hospitalizations and duration of burst suppression. At last neurology follow up, 2 patients reported daily seizures, and remaining infrequent seizures on AEDs (range 2-7 medications). 5 patients underwent neuropsychology evaluation (ranging 6.7 – 29.2 months from onset), with IQ ranging borderline to average. All patients showed impaired working memory and processing speed, and many with executive functioning problems.  All currently require an individualized education plan in school.