Febrile‐infection related epilepsy syndrome (FIRES) is a devastating neurological condition characterized by a febrile illness preceding new onset refractory status epilepticus (NORSE). Increasing evidence suggests innate immune dysfunction as a potential pathological mechanism. We report an international retrospective cohort of 25 children treated with anakinra, a recombinant interleukin‐1 receptor antagonist, as an immunomodulator for FIRES. Anakinra was potentially safe with only one child discontinuing therapy due to infection. Earlier anakinra initiation was associated with shorter duration of mechanical ventilation, ICU and hospital length of stay. Our retrospective data lay the groundwork for prospective consensus‐driven cohort studies of anakinra in FIRES.

Febrile infection‐related epilepsy syndrome (FIRES) is a rare catastrophic epileptic encephalopathy that presents suddenly in otherwise normal children and young adults causing significant neurological disability, chronic epilepsy, and high rates of mortality. To suggest a therapy protocol to improve outcome of FIRES, workshops were held in conjunction with American Epilepsy Society annual meeting between 2017 and 2019. An international group of pediatric epileptologists, pediatric neurointensivists, rheumatologists and basic scientists with interest and expertise in FIRES convened to propose an algorithm for a standardized approach to the diagnosis and treatment of FIRES. The broad differential for refractory status epilepticus (RSE) should include FIRES, to allow empiric therapies to be started early in the clinical course. FIRES should be considered in all previously healthy patients older than two years of age who present with explosive onset of seizures rapidly progressing to RSE, following a febrile illness in the preceding two weeks. Once FIRES is suspected, early administrations of ketogenic diet and anakinra (the IL‐1 receptor antagonist that blocks biologic activity of IL‐1β) are recommended.

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Abstract:Objective:To examine if telemedicine remains safe and of high quality despite rapid expansion of services by comparing telemedicine encounters prior to and during COVID 19 pandemic.Methods:Pre-post study investigating 2999 telemedicine encounters: 2/1/2020 - 5/15/2020. 2919 completed visits before and after strict social distancing implementation were analyzed for patient and provider characteristics, encounter characteristics (history, physical exam, etc) and quality and safety metrics (phone calls <=7 days post visit, visit- cause specific hospital admission or mortality <=30 days after visit). Stratified analysis of 3 groups for outcomes (young age, neuromuscular diagnosis and new encounters) was performed.Results:Patients ranging from 1 month old to 33 years were seen. Rural patients were less likely to be seen during pandemic compared to urban patients(8 % vs 90% p< 0.0001); teaching clinic and specialty clinic encounters increased significantly during pandemic ( 8% vs 3% ; p=0.005), documentation of at least two systems on exam was noted significantly more frequently during pandemic ( 13% vs 7%; p=0.009). No deaths were reported. There were no differences pre/ during pandemic for safety or telemedicine failure metrics within entire group and high-risk subgroupsConclusions:Despite a markedly and rapidly expanded scope of ambulatory telemedicine care during the COVID19 pandemic, telemedicine remained a safe and high-quality option for pediatric neurology patients. Additionally, populations perceived as high risk for telemedicine (the very young, new patients and those with neuromuscular diagnoses) can benefit from telemedicine visits, particularly when access to in-person care is limited.