Febrile infection-related epilepsy syndrome

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Febrile infection-related epilepsy syndrome

Synonyms: AERRPS | Acute encephalitis with refractory repetitive partial seizures | Acute non-herpetic encephalitis with severe refractory status epilepticus | DESC syndrome | Devastating epileptic encephalopathy in school-aged children | FIRES | Fever-induced refractory epileptic encephalopathy in school-aged children | Idiopathic catastrophic epileptic encephalopathy | Severe refractory status epilepticus owing to presumed encephalitis

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.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version May 2024

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