Abstracts

Rationale: Status epilepticus (SE) is one of the most common life-threatening medical emergencies in children, with an annual incidence ranging from 10 to 58 per 100,000 (Riviello. Neurology, 2006. 67(9): p. 1542-50). Febrile status epilepticus (FSE) remains the most common etiology of pediatric status epilepticus with frequency ranging from 22-32% among various studies. (Freilich. Curr Opin Pediatr 2014, 26:655 – 661). The relationship between prolonged febrile seizures and development of epilepsy and mesial temporal sclerosis remains unclear. The FEBSTAT trial reported increased risk for acute hippocampal injury and abnormal hippocampal development in children with febrile status, however the long term outcomes are still not known as this trial is still in progress (Shinnar. Neurology 71:170–176 and 79:871-7.). In this study, we aim to characterize children presenting with refractory febrile status epilepticus to a tertiary children’s hospital and to elucidate their long-term outcomes. Methods: This is a single-center retrospective observational study. We identified all patients between ages 1 month and 5 years old who were admitted to Children’s Medical Center pediatric ICU with refractory febrile status epilepticus between January 2008 and July 2017. Refractory febrile status epilepticus was defined as a status epilepticus where the sole acute provocation was fever which persists despite the administration of at least two parenteral anti-epileptic medications (AED) including a benzodiazepine. Exclusion criteria includes: acute central nervous system infection or insult, prior history of epilepsy or seizures, developmental delay or known neurological disorders. Results: A total of 674 unique patients presented with status epilepticus,  72 of them met criteria for febrile status epilepticus. Out of those, 15 patients were determined to have refractory febrile status epilepticus. The demographics and baseline characteristics are shown in table 1. All patient underwent brain magnetic resonance imaging (MRI), 2 of them (13%) were abnormal. Electroencephalogram (EEG) was performed in all patients as well and was abnormal in 80% of the patients. Of the abnormal EEGs, 28.5% had normal background. 60% had epileptiform activities of these 40% spike or sharp and slow wave discharges, 13% had periodic lateralized epileptiform discharges, and 6% had generalized periodic epileptiform discharges (table 2). Ictal seizures were seen in 3 patients, all were focal in onset. All the children received benzodiazepines as first line therapy, 53% required two further loads AED, and 40% required three. Four patients required advancement to anesthetic medications (26%). All patients were discharged home and 13% required outpatient therapy at discharge. Three patients (20%) were discharged without AED, 9 (60%) were discharged on 1 AED and 3 (9.2%) were discharged on 2 AED. Twelve patients were followed up in clinic. Mean duration of follow up was 1.75 years (± 0.94) with a range between 0.5 to 3.5 years. Only three patients remained on AED, with two patients planned to wean off medication. Modified Rankin Scores were good in all 12 patients seen in follow up. None of the children have had recurrence of status epilepticus and only one child has been diagnosed with epilepsy and mild cognitive impairment and was found to have a KCNMA1 mutation on further testing. Conclusions: • Outcomes in children with refractory febrile status epilepticus remain positive with majority of children without future recurrence of status epilepticus• Patients discharged on anti-seizure medications had a high likelihood of being able to wean off medication on follow up. • Progression of therapy to anesthetic infusions was seen in less than one third of patients. Funding: None