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Super-refractory Status Epilepticus in Febrile Infection-Related Epilepsy Syndrome Triggered by Influenza A: A
1Intensive Care Unit, Cliniques Universitaires Saint-Luc, Brussel, BEL.
Insights
Febrile infection-related epilepsy syndrome (FIRES) is a rare, severe condition. This case shows a pediatric patient with Influenza A developing super-refractory status epilepticus, highlighting neuroinflammation
Area of Science:
- Neurology
- Pediatrics
- Infectious Diseases
Background:
- Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe neurological disorder.
- It is characterized by new-onset refractory status epilepticus following a febrile illness.
Abstract:
Febrile infection-related epilepsy syndrome (FIRES) is a rare cause of new-onset refractory status epilepticus following a febrile illness. We report the case of a previously healthy three-year-old boy who developed focal seizures in the context of a confirmed Influenza A infection, rapidly progressing to super-refractory status epilepticus. Initial investigations, including cerebrospinal fluid analysis and neuroimaging, were unremarkable. Despite early multimodal therapy with multiple antiseizure medications, continuous anesthetic infusions, immunomodulatory treatments, and a ketogenic diet, seizure control remained transient. Continuous EEG confirmed persistent super-refractory status epilepticus, while cytokine analysis demonstrated elevated interleukin-6 and interleukin-8 levels. Follow-up MRI showed bilateral claustrum and hippocampal involvement, later progressing to diffuse cerebral atrophy. Despite escalation of therapy, including intrathecal corticosteroids and vagal nerve stimulation, the patient showed no sustained improvement. Care was ultimately redirected toward palliation, and the patient died after 50 days of intensive care. This case highlights the fulminant and refractory nature of FIRES, supports a potential role of cytokine-driven neuroinflammation, and suggests a possible association with a preceding viral infection.
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