Febrile infection-related epilepsy syndrome - FIRES synonyms

Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic form of epilepsy characterized by the sudden onset of refractory status epilepticus following a febrile illness. The condition is frequently referred to by several synonyms, most notably New-Onset Refractory Status Epilepticus (NORSE), though FIRES is specifically reserved for cases preceded by a febrile infection.

What are the common synonyms and historical names for FIRES?

Because the understanding of Febrile infection-related epilepsy syndrome (FIRES) has evolved significantly, older medical literature may use various terms that describe the clinical presentation rather than a specific disease entity. Common synonyms and related historical terms include:

• NORSE: New-Onset Refractory Status Epilepticus (a broader category that includes FIRES)


• DESC: Devastating Epileptic Encephalopathy in School-age children


• AERRPS: Acute Encephalitis with Refractory Repetitive Partial Seizures


• EIRES: Epilepsy with infection-related electrical status epilepticus

Why does Febrile infection-related epilepsy syndrome (FIRES) have multiple names?

The nomenclature for Febrile infection-related epilepsy syndrome (FIRES) has evolved due to its complex, heterogeneous nature. Historically, clinicians named the condition based on the specific constellation of symptoms they observed, such as "acute encephalitis." In 2018, an international consensus group defined NORSE as the overarching clinical presentation, while Febrile infection-related epilepsy syndrome (FIRES) was established as the specific sub-category for patients where a fever occurs between 24 hours and 2 weeks prior to the onset of seizures.

How is this condition classified in medical systems?

Standardized classification helps clinicians track Febrile infection-related epilepsy syndrome (FIRES) across global health systems. While it does not always have a unique ICD-10 code, it is increasingly recognized in research databases:

1. Orphanet: Listed under ORPHA:284381.


2. OMIM: Frequently discussed within the context of epilepsy-related encephalopathies.


3. ICD-11: Generally coded under "Status Epilepticus" with supplementary codes for the underlying inflammatory or infectious etiology.

Which name is preferred by medical professionals?

Today, Febrile infection-related epilepsy syndrome (FIRES) is the preferred term in clinical and research settings when a preceding fever is identified. Using this specific name helps differentiate the condition from other forms of NORSE that may have different triggers or genetic associations. Our DiseaseMaps.org community of 65 members currently uses this terminology to share experiences and navigate diagnostic pathways.

Next steps

• Consult with a board-certified epileptologist or pediatric neurologist specializing in neuro-immunology.


• Connect with the 65 members of the DiseaseMaps.org community to share lived experiences.


• Review the latest consensus definitions for NORSE and Febrile infection-related epilepsy syndrome (FIRES) on the NIH GARD website.

Medical disclaimer: This content is for informational purposes only and does not constitute professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding a medical condition.

References

• NIH Genetic and Rare Diseases Information Center (GARD)


• Orphanet (ORPHA:284381)


• The NORSE Institute (Clinical Consensus Guidelines)


• International League Against Epilepsy (ILAE) - EpilepsyDiagnosis.org