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Journal of Pediatric Epilepsy 2019; 08(03): 083-092
DOI: 10.1055/s-0039-1701035
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Febrile Infection-Related Epilepsy Syndrome

Mandeep Rana
1   Division of Pediatric Neurology, Department of Pediatrics, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts, United States
,
Alcy R. Torres
1   Division of Pediatric Neurology, Department of Pediatrics, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts, United States
,
Kam Lun Hon
2   Department of Paediatrics and Adolescent Medicine, The Hong Kong Children's Hospital, Hong Kong, Hong Kong
3   Department of Paediatrics, The Chinese University of Hong Kong, Hong Kong, Hong Kong
,
Alexander K.C. Leung
4   Department of Pediatrics, The University of Calgary and The Alberta Children's Hospital, Calgary, Alberta, Canada
,
Rinat Jonas
1   Division of Pediatric Neurology, Department of Pediatrics, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts, United States
› Author Affiliations
Further Information

Publication History

30 October 2019

04 November 2019

Publication Date:
24 January 2020 (online)

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Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a subset or variant of new-onset refractory status epilepticus in children. FIRES is characterized by the occurrence of a febrile episode between 24 hours and 2 weeks before the onset of refractory status epilepticus. A infectious cause is rarely identified in FIRES and an inflammatory or autoimmune etiology is implied. Seizures in FIRES are very difficult to control, and treatments include antiepileptic drugs, ketogenic diet, intravenous immunoglobulin, plasmapheresis, and corticosteroid therapy. The prognosis for patients with FIRES is poor, and most children are left with refractory epilepsy and cognitive impairment. The new consensus guidelines on the terminology of FIRES and recent interest in new treatment approaches have been welcome developments for clinicians who face the challenge of diagnosing and managing status epilepticus in a previously healthy child that occurs following a minor febrile episode. This review aims to provide clinicians with an update on the current hypotheses for the etiology, pathogenesis, clinical evaluation, management, and future directions in the diagnosis and treatment of FIRES.

Keywords

FIRES - epilepticus - refractory - epilepsy
 

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