Acronym for Febrile - Infection - Related - Epilepsy - Syndrome.

Very rare. Rare cause of status epilepticus. Classic clinical description: a few days after a common febrile infection (usually in a child aged from 4 to 9 years), onset of seizures (partial or that generalize secondarily) that evolve to status epilepticus in the following 24 hours. This status epilepticus is difficult to control and often requires intubation and artificial ventilation. No infectious agent can be identified, CSF shows nothing special and MRI shows nonspecific signs. The treatment is ineffective; some teams use a prolonged barbiturate coma. A ketogenic diet introduced early and administration of immunomodulators (corticosteroids or IV immunoglobulins) are recommended.

The prognosis is poor: up to 30 % of deaths; epilepsy or mental retardation; cases of healing without sequelae are very rare.

The pathogenesis is unknown: the current hypothesis is a proinflammatory process that could modify the proper functioning of certain ion channels and so increase neuronal excitability.

Anesthetic implications: 

complex epilepsy.

References : 

-         Kramer U, Chi C-S, Specchio N, Sahin M, Olson H, Nabbout R, Kluger G, Lin J-J, van Baalen A.
Febrile-infection-related epilepsy syndrome (FIRES) : pathogenesis, treatment, and outcome. A multicenter study on 77 children. 
Epilepsia 2011; 52: 1956-65.

Updated: April 2019