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febriele-infectiegerelateerd epilepsiesyndroom (aandoening)
febriele-infectiegerelateerd epilepsiesyndroom
febrile infection related epilepsy syndrome
'devastating epileptic encephalopathy in school-aged children'-syndroom
DESC
FIRES
Febrile infection related epilepsy syndrome
FIRES - fever-induced refractory epileptic encephalopathy in school-aged children
DESC (devastating epileptic encephalopathy in school-aged children) syndrome
Acute encephalitis with refractory repetitive partial seizures
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.
Id725413002
StatusPrimitive
Associated morphologyacute ontsteking
Finding sitestructuur van cerebrum
Occurrencekinderleeftijd
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetG40.5
TermSpeciale epileptische syndromen
SNOMED CT to Orphanet simple map163703
SNOMED CT to ICD-10 extended map
TargetG40.5
RuleTRUE
AdviceALWAYS G40.5 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified