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Febrile infection related epilepsy syndrome (disorder)
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
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
Occurrencekindertijd
ICD-10 complex map reference set
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