Ann Child Neurol.  2020 Jan;28(1):8-15. 10.26815/acn.2019.00283.

Febrile Infection-Related Epilepsy Syndrome: Refractory Status Epilepticus and Management Strategies

Affiliations
  • 1Department of Pediatrics, Pusan National University Children’s Hospital, Pusan National University School of Medicine, Yangsan, Korea
  • 2Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, Korea

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic epileptic syndrome that strikes previously healthy children. Its pathogenesis is unknown, it has few treatments, and it is typically refractory. In FIRES, refractory status epilepticus or a cluster of seizures starts a few days after the onset of an acute febrile illness, and it may continue as drug-resistant epilepsy, with neuropsychological impairments occurring without latency. Clinical knowledge and guidelines on FIRES are limited because it is sporadic and extremely rare. To date, the absence of specific biomarkers poses a significant diagnostic challenge; nevertheless, early diagnosis is very important for optimal management. Despite treatment with multiple immunotherapies and anti-seizure medications, the majority of patients with FIRES are left with significant cognitive disabilities and refractory epilepsy. This review aims to highlight the most recent insights into the clinical features, terminology, epidemiology, pathogenesis, diagnostic challenges, and therapeutic options associated with FIRES.

Keyword

Seizures, febrile; Drug resistant epilepsy; Epileptic syndromes; Status epilepticus
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