Febrile infection-related epilepsy syndrome

Alternate names

FIRES; Acute encephalitis with refractory repetitive partial seizures; AERRPS; Acute non-herpetic encephalitis with severe refractory status epilepticus; Devastating epileptic encephalopathy in school-aged children; DESC syndrome; Fever-induced refractory epileptic encephalopathy in school-aged children; Idiopathic catastrophic epileptic encephalopathy; Severe refractory status epilepticus owing to presumed encephalitis; Status epilepticus owing to presumed encephalitis; Febrile infection-related epilepsy syndrome

Summary

FIRES (Febrile Infection-Related Epilepsy Syndrome) is a sub-type of cryptogenic new-onset refractory status epilepticus (NORSE). NORSE describes a condition in which a healthy person who has not had seizures before, begins having seizures. Of note, FIRES previously was considered a separate condition that occurred only in children, while NORSE was described only in adults. However, there is now consensus that FIRES and NORSE without a prior fever can both be described in people of any age.

Cause

Some researchers believe cryptogenic NORSE, including FIRES, may be an inflammatory disorder. Others suggest that an unidentified brain infection may cause some cases.

Inheritance

Currently there is no evidence that NORSE runs in families (hereditary); however, studies indicate that changes in certain genes, including the SCN2A and IL1RN gene(s) may increase the risk to develop NORSE.

Signs and symptoms

• FIRES starts with a febrile illness up to two weeks before seizure onset.
• These seizures damage the frontal lobe's cognitive brain function such as memory and sensory abilities.
• This can result in learning disabilities, behavioral disorders, memory issues, sensory changes, and possibly death.
• Children continue to have seizures throughout their lives.

Diagnosis

• FIRES is difficult to diagnose due to its rarity and lack of definitive biomarker.
• It is often diagnosed by ruling out other options such as infectious, toxic, metabolic, and genetic causes.
• FIRES will consist of two phases - acute and chronic.
• The acute phase consists of highly recurrent focal seizures, rapidly evolving into refractory status epilepticus.
• The chronic phase consists of drug-resistant epilepsy with cognitive impairment.

Treatment

Treatment for NORSE (including FIRES) requires being cared for in an intensive care unit, at least until status epilepticus (SE) subsides and consciousness is regained. If an underlying cause of new-onset refractory status epilepticus (NORSE) is identified, treatment will include addressing the cause. There is no standard treatment for cryptogenic NORSE (when the cause cannot be found).

NIH genetic and rare disease info

• NIH info on Febrile infection-related epilepsy syndrome

Febrile infection-related epilepsy syndrome is a rare disease.

Febrile infection-related epilepsy syndrome Resources
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