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Hippocampal Changes in Febrile Infection-Related Epilepsy Syndrome (FIRES)
Amit Agarwal1, Shyamsunder Sabat1, Krishnamurthy Thamburaj1
1Department of Radiology, Penn State University, Hershey, PA, U.S.A.
Insights
Febrile infection-related epilepsy syndrome (FIRES) is an increasingly recognized condition in children, characterized by severe refractory seizures following an infection. This syndrome progresses through distinct phases, leading to chronic epilepsy and neurological impairments.
Area of Science:
- Neurology
- Pediatric Neurology
- Epileptology
Background:
- Febrile seizures are common in childhood, typically linked to fever.
- A severe, refractory afebrile seizure form post-infection in healthy children is increasingly identified globally.
- Febrile infection-related epilepsy syndrome (FIRES) is a proposed term for this severe, refractory post-infectious epileptic condition.
Observation:
- This case report details MR imaging findings in a 5-year-old child with refractory epilepsy after febrile seizures.
- The report focuses on initial and follow-up imaging to understand the evolving neurological changes.
Findings:
- FIRES represents a group of immune-mediated epileptic encephalopathies in previously healthy children.
- The syndrome typically presents in three phases: initial febrile infection, acute refractory seizures, and a chronic phase.
- Chronic phase is characterized by neuropsychological impairments and persistent seizures.
Implications:
- Increased global recognition of acute post-infectious seizures and FIRES is crucial for timely diagnosis.
- Understanding the phased progression of FIRES aids in managing refractory epilepsy in children.
- Further research into the immune-mediated mechanisms of FIRES is warranted for improved therapeutic strategies.
Background:
Febrile seizures are the most common seizure disorder in childhood, associated with a significant rise in body temperature. However, post-infectious refractory afebrile form of seizures in previously healthy children is being increasingly recognized in around the world, which evolves into a chronic refractory form of epilepsy. The term 'Febrile infection-related epilepsy syndrome' (FIRES) has been proposed for these conditions and represents a refractory severe post-infectious epileptic condition in previously normal children.
Case Report:
We report the initial and follow-up MR imaging findings in a 5year-old with refractory epilepsy post-febrile seizures.
Conclusions:
In summary, acute post-infectious seizures are increasingly being recognized across the globe with the newly coined term 'Febrile infection-related epilepsy syndrome'(FIRES) for this group of immune-mediated epileptic encephalopathy in previously healthy children. This has three phases: episode of simple febrile infection, followed by acute refractory seizures and lastly the chronic phase of neuropsychological impairments and seizures.
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