Abstracts

Rationale: To describe the long-term outcome and try to find the effective treatment strategies in children with febrile infection-related epilepsy syndrome (FIRES), where the devastating outcomes are alleged to be associated with the duration of refractory status epilepticus.Methods: The children diagnosed as FIRES in a single Korean tertiary center were reviewed. The outcomes were measured according to the neurological status at last follow-up and the duration of refractory status epilepticus. The efficacy of initial therapy after midazolam during the refractory period of status epilepticus was also evaluated.Results: Twenty-six (19 male) patients were identified. The mean age at seizure onset is 6.6 years (range, 2 to 14 years) and the mean follow-up period is 5.4 years (range, 0.4 to 14.7 years). Mean duration of refractory status epilepticus was 65 days (range, 8 to 366 days). Only seven (26.9%) patients had good neurological outcomes with normal intellectual function (IQ >70). Twelve (46.1%) patients had extremely poor neurologic outcome such as quadriplegia or vegetative state including two deaths and seven showed moderate to severe intellectual disability. The patients with good neurological outcome showed significantly shorter duration of status epilepticus (20.6 ± 16.8 days) than those with poor outcomes (82.6 ± 76.5 days, p = 0.046). As the second line therapy for refractory status epilepticus, those who received high dose phenobarbital had shorter duration of status epilepticus compared to those with barbiturate coma therapy (24.5 ± 18.3 days, n = 6 vs. 85.5 ± 77.6 days, n = 18; p = 0.076). Ketogenic diet and immunoglobulin therapy were tried in seven and one patients, which was not efficacious.Conclusions: Most patients with FIRES showed poor neurological outcomes, which was associated with duration of status epilepticus. However, the cessation of status epilepticus was difficult. High dose phenobarbital therapy might be the possible choice in children with FIRES.