EPILEPSY RESEARCH UK
ENDEAVOUR PROJECT GRANT
£145,591 over 24 months
Awarded in 2022

SCIENTIFIC TITLE

Can we extinguish the seizures in FIRES?

CO-INVESTIGATORS

Dr Sukhvir Wright

CO-INVESTIGATORS

Dr Marios Kaliakatsos (GOSH), Prof Gavin Woodhall (Aston University), Dr Suresh Pujar (GOSH), Dr Kimberley Gilmour (GOSH), Dr Dimitrios Champsas (UCL ION)

INSTITUTION

Aston University

FIRES is a devastating epilepsy syndrome with life-changing consequences for patients and their caregivers. We hope this “bench-to-bedside” research will increase our understanding of FIRES from the whole brain to the single brain cell level, and help provide urgently needed insights into the most effective treatments to improve patient outcomes.
Dr Sukhvir Wright

BACKGROUND

“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, or SRSE). It occurs mostly in children, but affects adults too; most people who experience FIRES have no history of epilepsy and are previously healthy. The initial flurry of acute back-to-back seizures (100’s per day) are followed by chronic epilepsy, no less difficult to treat, associated with severe developmental problems. Although FIRES is rare, 12% of children and 30% of adults affected die; and there are no specific treatments that are universally effective.

THE STUDY

In children, most FIRES cases have no identifiable cause but their cerebro-spinal fluid (CSF), the fluid that bathes the brain, often reveals high levels of molecules associated with inflammation. Some of these molecules are known to cause seizures in laboratory models. FIRES patients’ CSF trialled in Dr Wright’s laboratory has been found to cause epileptic activity, so the team are confident and excited this method will provide a world-first accurate disease model of FIRES. These CSF samples will allow them to test newer and standard treatments. Additionally, Dr Wright’s team will systematically identify UK paediatric cases and collect clinical information to learn about current treatments and outcomes. This will enable them to improve future patient care by formulating specific guidelines for FIRES management. The team will also work alongside collaborators in the US to conduct this research.

SIGNIFICANCE

This innovative study with international expert backing has the potential to transform the lives of FIRES patients worldwide and the results will be applicable to people with epilepsy within the next 5 to 10 years. In the first instance, publication of the surveillance study data will give crucial information about the clinical syndrome, treatments given and subsequent outcomes of affected patients. This data will then be used to create specific guidelines on management to improve patient care for future affected children and families, transferring the new knowledge gained from bench to bedside.