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Updated: Feb 8, 2026
Multifocal Electroretinograms
Published on: December 4, 2011
Febrile Infection-Related Epilepsy Syndrome (FIRES) with Multifocal Subcortical Infarcts, A New Imaging Phenotype
1Department of Diagnostic Imaging, National University Health System, Singapore.
Insights
Febrile infection-related epilepsy syndrome (FIRES) is a rare neurological disorder in children. This case highlights a new imaging pattern of extensive subcortical infarcts, potentially aiding earlier diagnosis.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimmunology
Background:
- Febrile infection-related epilepsy syndrome (FIRES) is a severe, unexplained epileptic encephalopathy primarily affecting school-aged children after a febrile illness.
- Its exact cause is unknown, but it's suspected to involve immune system overactivation.
- FIRES is often a diagnosis of exclusion due to a lack of specific diagnostic criteria or tests.
Observation:
- This report details a rare case of FIRES presenting with extensive subcortical infarcts, particularly in the temporo-occipital regions.
- This specific imaging presentation has not been previously documented in FIRES literature.
Findings:
- The observed extensive subcortical infarcts may represent a novel imaging phenotype of FIRES.
- Understanding diverse imaging patterns is vital for prompt recognition and management of FIRES.
Implications:
- Identifying new imaging phenotypes like this can improve the timely diagnosis of FIRES.
- This finding may prompt further research into the underlying mechanisms and potential treatments for FIRES.
Abstract:
Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic encephalopathy of unknown etiology which occurs predominantly in school-aged children, following a febrile illness. The term FIRES was first proposed in 2010 by van Baalen et al. The etiology of FIRES remains elusive, although most believed that it is likely related to inappropriate activation of the innate immune system. It is often a diagnosis of exclusion as it lacks specific clinical criteria and/or confirmatory tests. Familiarity with the range of imaging phenotypes associated with FIRES is crucial as this will assist timely recognition and institution of appropriate treatment plan. With this in mind, the author would like to present a rare case of FIRES with extensive subcortical infarcts, predominantly in the temporo-occipital lobes. This has never been reported before and may represent a new imaging phenotype of FIRES. A detailed literature review, focusing on the various pattern of imaging phenotypes, in relation to patients' age and clinical outcome, will also be included.
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