Febrile infection-related epilepsy syndrome

MONDO:0015584

A rare, potentially fatal, epileptic encephalopathy characterized by explosive-onset of recurrent multifocal and bilateral tonic-clonic seizures following an unspecific febrile illness. The syndrome develops without a clear acute structural, toxic or metabolic cause, in a patient without previous epilepsy. FIRES is a subgroup of new-onset refractory status epilepticus (NORSE), and requires a preceding febrile infection as a mandatory feature.

Also known as: AERRPS, DESC syndrome, FIRES, Febrile Infection-Related Epilepsy Syndrome (FIRES), acute encephalitis with refractory repetitive partial seizures, acute non-herpetic encephalitis with severe refractory status epilepticus, devastating epileptic encephalopathy in school-aged children, fever-induced refractory epileptic encephalopathy in school-aged children

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