Can we extinguish the seizures in FIRES? Version 1
Research type
Research Study
Full title
Can we extinguish the seizures in Febrile Infection Related Epilepsy Syndrome(FIRES)? A clinical surveillance study
IRAS ID
311065
Contact name
Sukhvir /K Wright
Contact email
Sponsor organisation
Aston University
Duration of Study in the UK
6 years, 0 months, 1 days
Research summary
"FIRES” or febrile infection-related epilepsy syndrome is exactly what it sounds like, an explosive onset of the most difficult to treat relentless seizures (super-refractory status epilepticus, SRSE). It occurs mostly in children, but affects adults too; most sufferers have no history of epilepsy and are previously healthy. The initial flurry of acute back-to-back seizures (100’s/day) are followed by chronic epilepsy, no less difficult to treat, associated with severe developmental problems. Although FIRES is rare (~1:1000000 incidence), 12% of children and 30% of adults affected die; there are no specific treatments that are universally effective.
In children, the majority of cases have no identifiable cause but their cerebrospinal fluid (CSF), the fluid that bathes the brain, often reveals high levels of molecules associated with inflammation.We will systematically identify UK paediatric cases through the British Paediatic Neurological Surveillance Unit (BPNSU), using the recent expert definition of the disorder to determine exact incidence and prevalence in order to establish a database (clinical data, MRI, EEG). Our aim is to learn about current treatments/outcomes and improve future patient care by formulating specific guidelines for FIRES management. Some patients will be approached to donate cerebrospinal fluid (CSF) samples. We will only use samples that have been taken during routine clinical care, no additional tests are needed due to the very small amount required for our experiments (0.5 ml of CSF could be enough for up to 500 experiments).
We will collect these CSF samples for future experimental use to help us understand why FIRES seizures are so difficult to treat and to test new and established treatments.REC name
Wales REC 5
REC reference
22/WA/0288
Date of REC Opinion
1 Dec 2022
REC opinion
Further Information Favourable Opinion