[4] Complications often include intellectual disability, behavioral problems, and ongoing seizures.
These seizures damage the frontal lobe's cognitive brain function such as memory and sensory abilities.
This can result in learning disabilities,[6] behavioral disorders, memory issues, sensory changes, and possibly death.
There are theories of an immunological source, a genetic predisposition, and an inflammation-mediated process, but the definite cause is unknown.
The acute phase consists of highly recurrent focal seizures, rapidly evolving into refractory status epilepticus.
[6][11] On a 10-20 scalp electrode EEG, the ictal activity commonly begins temporally and spreads hemispherically and/or bilaterally.
[13] A recent study of 12 FIRES patients demonstrated diffuse delta-theta background slowing interictally in all 12 cases.
[5][18] Previous names include AERRPS (acute encephalitis with refractory, repetitive partial seizures), DESC (Devastating Epilepsy in School-aged Children),[19] and NORSE (New-Onset Refractory Status Epilepticus).