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Abstract Details

Presentation and Long-term Neurologic and Neurodevelopmental Profiles in Pediatric Patients with Febrile Infection-Related Epilepsy Syndrome (FIRES): A Single Institution Review
Autoimmune Neurology
P6 - Poster Session 6 (12:00 PM-1:00 PM)
FIRES is a presumed immune-mediated abrupt onset of seizures provoked by febrile illness evolving to refractory status epilepticus and ultimately chronic refractory epilepsy. There is limited data on long term neurologic and neurodevelopmental outcomes in FIRES, especially with the introduction of new immunomodulation treatments.

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.

A retrospective chart review was performed on patients with diagnosis of FIRES.  Demographic, clinical data, and neuropsychological evaluations were collected.

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.

The neurologic and neurodevelopmental outcomes of this cohort of pediatric FIRES patients varied. All patients remain on AEDs for seizure control, some with continued daily seizures and others well controlled.  All demonstrated decline in overall intellectual functioning with significant impairments in working memory, processing speed, and executive function. All continue to require additional supports in school setting, including those with normal IQ.
Kristen Fisher, DO (Baylor College of Medicine)
Dr. Fisher has nothing to disclose.
No disclosure on file
Nikita Shukla, MD (BCM) The institution of Dr. Shukla has received research support from Roche.
No disclosure on file
No disclosure on file