febrile infection-related epilepsy syndrome
Findings
No curated finding names febrile infection-related epilepsy syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
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.
Definition from the Mondo Disease Ontology (MONDO:0015584), read 2026-09-29. CC BY 4.0.
Features
12 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Developmental regressionHPOHP:0002376
- Very frequent (80% to 99% of cases)
- EEG abnormalityHPOHP:0002353
- Very frequent (80% to 99% of cases)
- Focal-onset seizureHPOHP:0007359
- Very frequent (80% to 99% of cases)
- LethargyHPOHP:0001254
- Very frequent (80% to 99% of cases)
- Atypical behaviorHPOHP:0000708
- Frequent (30% to 79% of cases)
- CoughHPOHP:0012735
- Frequent (30% to 79% of cases)
- Fever
Where it sits
Other names
10 names
Resolves to: febrile infection-related epilepsy syndrome
- Also called
- acute encephalitis with refractory repetitive partial seizuresacute non-herpetic encephalitis with severe refractory status epilepticusAERRPSDESC syndromedevastating epileptic encephalopathy in school-aged childrenFebrile Infection-Related Epilepsy Syndrome (FIRES)fever-induced refractory epileptic encephalopathy in school-aged childrenFIRESidiopathic catastrophic epileptic encephalopathysevere refractory status epilepticus owing to presumed encephalitis