febrile infection-related epilepsy syndrome true severe refractory status epilepticus owing to presumed encephalitis AERRPS acute encephalitis with refractory repetitive partial seizures ICD10:G40.5 acute non-herpetic encephalitis with severe refractory status epilepticus Febrile infection-related epilepsy syndrome (FIRES) describes an explosive-onset, potentially fatal acute epileptic encephalopathy that develops in previously healthy children and adolescents following the onset of a non-specific febrile illness. MONDO:0015584 idiopathic catastrophic epileptic encephalopathy FIRES DESC syndrome devastating epileptic encephalopathy in school-aged children Orphanet:163703 GARD:0011005 UMLS:CN199955 Febrile infection related epilepsy syndrome (FIRES) has previously also been known as fever induced refractory epilepsy in school-aged children, devastating epileptic encephalopathy in school aged children (DESC) and acute encephalitis with refractory repetitive partial seizures (AERRPS). It is a severe post-infectious neurological disorder that presents with intractable status epilepticus in a previously normal child (or less commonly adult) after a febrile illness. If the patient survives, they have intellectual and motor impairment and ongoing intractable seizures. fever-induced refractory epileptic encephalopathy in school-aged children SCTID:725413002 status epilepticus owing to presumed encephalitis unknown etiology