Early intrathecal dexamethasone (IT-Dexa) associated with faster recovery and good outcome in an adolescent with febrile infection-related epilepsy syndrome (FIRES)

A previously normal adolescent boy presented with a new-onset super refractory status epilepticus of unknown cause after a brief febrile illness-suggestive of febrile infection-related epilepsy syndrome (FIRES). Bedside continuous EEG (cEEG) showed frequent focal electrical seizures. After a poor response to pulse methylprednisolone therapy, intrathecal dexamethasone (IT-Dexa) was initiated on day 9 of the illness. Complete seizure freedom was achieved after two doses of alternate-day IT-Dexa. Later cEEG showed bilateral PLEDs with gradual return to the normal background. His modified Rankin score was 2 at discharge and 1 at 3-month follow-up, with a well-preserved cognition. Various therapies like intravenous immunoglobulin, ketogenic diet, anakinra and tocilizumab have been tried in these patients, with variable responses. Early administration of IT-Dexa in children with FIRES may be a cost-effective and potent option, especially in low-resource countries-as it reduces the duration of critical care and improves seizure and cognitive outcomes.