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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
Acute encephalitis with refractory repetitive partial seizures
FIRES - fever-induced refractory epileptic encephalopathy in school-aged children
DESC (devastating epileptic encephalopathy in school-aged children) syndrome
This syndrome 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. Usually presents in 3-15 year olds that have previously been healthy and developmentally normal. It always comes after a simple febrile illness. Manifestations include the sudden onset of convulsive and recurrent focal seizures. This is followed by refractory focal epilepsy along with a decline in memory and cognition. Psychiatric disorders and occasionally motor disability can be present in some cases. In serious cases, the disease progression can lead to a vegetative or semi-conscious state or even death. There may be a genetic cause for the disease, as seen in Dravet syndrome, but as yet no causative genes have been identified.
Id725413002
StatusPrimitive
Associated morphologyacute ontsteking
Finding sitestructuur van cerebrum
Occurrencekinderleeftijd
referentieset met complexe 'mapping' naar ICD-10
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