P-EP010. Extinguishing fires using Tocilizumab: Malaysia’s first

Abstract

Introduction. Febrile infection related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with antecedent febrile infection preceding the onset of refractory status epilepticus (RSE), without the evidence of structural, toxic or metabolic cause. FIRES often manifest initially with a febrile infection preceding the onset of the acute phase with prolonged duration of RSE, usually lasting days to months, followed by the chronic phase with refractory epilepsy and neurological impairment without a silent period between the two phases. The pathophysiology of FIRES is largely unknown but has been hypothesized to be due to excessive cytokine release perpetuating the underlying seizure. This is supported by the fact that children with FIRES exhibit high levels of cytokines, including interleukin-6, in the serum, even more so in the CSF. Conventional immunotherapies such as steroid, IVIG, plasma exchange, rituximab have previously been considered for the treatment of FIRES but has been disappointing so far. Following a small retrospective observational case series showing dramatic cessation of RSE in six out of seven adults with new onset RSE, the therapeutic potential with Tocilizumab certainly needs to be further investigated. We report a successful therapeutic encounter using Tocilizumab, a humanized monoclonal antibody against interleukin-6 receptor to treat RSE in a 14-year-old girl with FIRES following dismal outcome using conventional immunosuppressive strategies. Results . The administration of IV Tocilizumab at a single dose of 4mg/kg has successfully terminated RSE within 72 hours. Exhaustive workup to exclude metabolic, structural, paraneoplastic, autoimmune and infectious causes has been unrevealing. The electro-clinical features, brain imaging and outcome for this patient are illustrated. Conclusion . The overproduction of cytokines and the poor performance of immunotherapies implicate a putative role of inflammation in FIRES. Our case showed that complete termination of RSE was achieved after Tocilizumab administration suggesting it may be a life-saving drug for this catastrophic condition.