Purpose: To describe clinical features, treatment modalities and outcome of Febrile infection related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy in Indian children.

Methods: We conducted a retrospective multicenter study at three centres from India. Children with normal development who presented with status epilepticus or encephalopathy with recurrent seizures following febrile illness were included.

Key Findings: From January 2007 to January 2018 around 59 children met the inclusion and exclusion criteria. The age at presentation, ranged from 8 months to 15 years (median 7 years). All children presented with prolonged or recurrent seizures occurring 1 to 14 days (median 4 days) after the onset of fever. CSF analysis was normal in all children. Initial neuroimaging was normal in 36 children.  Treatment modalities included multiple antiepileptic drugs (2-11 AED; median 6 drugs). Midazolam infusion was administered in 36 patients. 19 patients required barbiturate coma to suppress the seizure activity. Duration of barbiturate coma ranged from 2 to 90 days (median four days). Immunotherapy  was given to 54 patients without sustained response. Eleven children died in the acute phase. All children required prolonged hospital stay (Median – 30 days). On follow up 30 children were having ongoing refractory epilepsy. Outcome remained poor with median Glasgow outcome Scale of two on follow up.

Discussion: This is first multi-centre study from India on FIRES, which underlines poor long term developmental and seizure outcome. There is an urgent need for developing new effective therapeutic strategies in this acute catastrophic epileptic syndrome.