The short-term and long-term outcome of febrile infection-related epilepsy syndrome in children
Journal
Epilepsy and Behavior
Journal Volume
95
Pages
117-123
Date Issued
2019
Author(s)
Abstract
Background: The febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy which developed the refractory status epilepticus following or during a nonspecific febrile illness. To analyze the short-term and long-term outcome of FIRES in the children, we retrospectively analyzed the related data. Methods: The motor outcome was evaluated by modified Rankin scale (mRS). Poor motor outcome was defined as a mRS score of 4 or higher at discharge. Significant motor decline was defined as the mRS difference more than 2 before hospital admission and at discharge. Results: We totally enrolled 25 patients for analysis. Four patients were expired during hospitalization, and one patient was lost to follow-up after discharge. Therefore, a total 20 patients were finally analyzed. The age of disease onset ranged from 1.6 to 17.2 years (mean: 9.6 ± 4.4 years). Newly acquired epilepsy and cognitive deficit occurred in 100% and 61%, respectively. The duration of the anesthetic agents ranged from 7 to 149 days (mean: 34.2 ± 36.1 days). The duration of anesthetic agent usage (p = 0.011), refractory epilepsy (p = 0.003), and the use of ketogenic diet (p = 0.004) were significantly associated with the poor long-term motor outcome, and the number of anesthetic agents tended to be associated with the poor long-term motor outcome (p = 0.050). In-hospital mortality was 16%. Significant functional decline at discharge occurred in 100%. However, there was improvement in long-term follow-up. Conclusion: The outcome of FIRES is poor with significant mortality and morbidities. Refractory epilepsy with cognitive deficit in survived cases is common, but improvement is possible. ? 2019 Elsevier Inc.
SDGs
Other Subjects
anesthetic agent; anticonvulsive agent; corticotropin; fentanyl; immunoglobulin; ketamine; lidocaine; midazolam; propofol; steroid; thiopental; anesthetic agent; adolescent; Article; child; clinical article; cognitive defect; controlled study; disease association; drug resistant epilepsy; drug use; electroencephalogram; epilepsy; febrile infection related epilepsy syndrome; female; follow up; functional disease; hospital admission; hospital discharge; hospital mortality; hospitalization; human; ketogenic diet; long term care; male; morbidity; motor dysfunction; motor performance; outcome assessment; prognosis; Rankin scale; retrospective study; short course therapy; Taiwan; cognitive defect; complication; epileptic state; febrile convulsion; infant; ketogenic diet; motor dysfunction; preschool child; Adolescent; Anesthetics; Child; Child, Preschool; Cognitive Dysfunction; Diet, Ketogenic; Drug Resistant Epilepsy; Female; Follow-Up Studies; Humans; Infant; Male; Motor Disorders; Retrospective Studies; Seizures, Febrile; Status Epilepticus
Publisher
Academic Press Inc.
Type
journal article