Abstract
Febrile Infection-Related Epilepsy Syndrome (FIRES) is a catastrophic, extremely rare epileptic encephalopathy. It strikes previously healthy school-aged children and is usually cryptogenic. Its dramatic onset with refractory status epilepticus is always preceded by a nonspecific febrile illness. The seizure activity in FIRES may last for several weeks with little to no response to antiepileptic treatment, usually resulting in the usage of anaesthetics. This acute phase is followed by a chronic, refractory epilepsy and cognitive deficit, that persist for the rest of the patient’s life. Still to this day no definite cause has been described. In this study we review the current finding in FIRES and describe a case of a 4-year-old patient with a dramatic course of the acute phase in FIRES and unusual presentation of the chronic phase, which is dominated by extrapyramidal symptoms such as dystonia. This case highlights that the clinical presentation of FIRES may differ from those frequently described in literature.
Highlights
Febrile Infection-Related Epilepsy Syndrome (FIRES) is a catastrophic epileptic encephalopathy affecting school-aged children
FIRES has been later described with many names, such as DESC [2], AERRPS [3], idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status [4]
FIRES has been characterised by the following EEG findings: (1) beta-delta complexes resembling extreme delta brush (EDB), (2) gradual increase in seizure burden, (3) seizure onset with prolonged focal fast activity, followed by the gradual appearance of well-formed rhythmic spike or spike-and-wave complexes, and (4) shifting ictal activity [32]
Summary
Febrile Infection-Related Epilepsy Syndrome (FIRES) is a catastrophic epileptic encephalopathy affecting school-aged children. Its occurrence is extremely rare, but often fatal It is all the more devastating given that FIRES develops in previously healthy individuals. FIRES has been later described with many names, such as DESC (devastating epileptic encephalopathy in school-aged children) [2], AERRPS (acute encephalitis with refractory, repetitive partial seizures) [3], idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable status [4]. Nowadays FIRES is considered to be a sub-category of New. Onset Refractory Status Epilepticus (NORSE), which is described as a clinical presentation of refractory status epilepticus that occurs in patients without active epilepsy nor with any signs of structural, toxic or metabolic damage to the brain, that can be ruled out in the first 72 h. The patient did not respond to any implemented treatment and developed severe encephalopathy
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