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 September 2023.
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Febrile infection-related epilepsy syndrome?
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Advocacy Organizations
Encephalitis Society
We are an international charity and the only resource of our kind in the world, dedicated to supporting those affected by encephalitis, their families and professionals involved in their care. Our work involves support, information, awareness and research.
Mickie?s Miracles
To help families get into Level IV Pediatric Epilepsy Centers for diagnosis and treatment urgently -- and provide support to families for every season of the pediatric epilepsy journey.
Genetic Epilepsy Team Australia
Collaboration of research and care
Moonshots for Unicorns
Curing single-gene disorders
COMBINEDBrain Inc
COMBINEDBrain is a consortium for outcome measures and biomarkers for neurodevelopmental disorders. We are collaborating to cure rare, non-verbal brain disorders.
Syndromes Without A Name (SWAN) Australia
Provide information, support and advocacy to families caring for a child with an undiagnosed or rare genetic condition.
Clinical Trials
For a list of clinical trials in this disease area, please click here.