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 April 2024
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Febrile infection-related epilepsy syndrome?
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Advocacy Organizations
COMBINEDBrain Inc
COMBINEDBrain is a consortium for outcome measures and biomarkers for neurodevelopmental disorders. We are collaborating to cure rare, non-verbal brain disorders.
Encephalitis Society
Our vision A world without death and disability from encephalitis. Our mission Rebuilding futures around the world by saving lives, accelerating awareness and driving research.
Genetic Epilepsy Team Australia
Collaboration of research and care
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.
Moonshots for Unicorns
Curing single-gene disorders
Project CASK
To fund research for treatment and/or a cure for CASK Gene Disorder
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
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