ICNC Abstracts, ICNC 2018

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Febrile Infection Related Epilepsy Syndrome (FIRES) – A rare cause of super refractory status epilepticus
Aanchal Pradeep Khemani, Sanjay Bafna, Sagar Lad, Nandan Yardi

Last modified: 2018-09-09

Abstract


INTRODUCTION-FIRES is a catastrophic epilepsy syndrome affecting developmentally normal school children and emerging as an important but rare cause of refractory status epilepticus (SE).

 

CASE REPORT-11 year girl, developmentally normal presented with fever and irrelevant talk since 5 days, altered sensorium since 1 day and multiple convulsions before admission. She was intubated, ventilated and started on ceftriaxone, acyclovir and multiple antiepileptics. She was seizure free for 2 days but from day 11 of illness developed super refractory SE. Newer antiepileptics were added. Coma induction was done sequentially with midazolam, thiopentone and ketamine. Seizures continued for 6 weeks. EEG showed convulsive and nonconvulsive SE. MRI on day 4 of illness was normal but day 19 MRI showed bilateral hippocampal hyperintensities. CSF PCR for pan neurotropic viruses was positive for cytomegalovirus but CSF routine, culture, latex and autoantibody panel were normal. We gave gancyclovir and oral valgancyclovir for total 4 weeks. Serum CMV PCR was negative raising the possibility of false positive CSF CMV result. Considering her stormy clinical course without any etiological clue diagnosis of FIRES was entertained. Intravenous immunoglobulin and pulse methylprednisolone was given. Due to persistent seizures and encephalopathy ketogenic diet was started during 5th week. Gradually seizures subsided from 7th week. She is presently seizure free, following commands and has no focal deficits.

 

CONCLUSION-High suspicion for FIRES is warranted particularly when other etiologies are excluded. Seizures in FIRES need early coma induction to prevent super refractory SE. Early initiation of ketogenic diet is supposed to be beneficial.


Keywords


FIRES; Super refractory status epilepticus; Coma induction; Ketogenic diet

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