FEBRILE INFECTION-RELATED EPILEPSY SYNDROME (FIRES)

	Mandatory	Alerts	Exclusionary
Seizures	Febrile illness within 2 weeks of seizure onset Focal/multifocal seizures that often evolve to bilateral tonic-clonic seizures Seizures increase in frequency and severity culminating in super-refractory status epilepticus, typically within 2 weeks of onset		Prior history of epilepsy
EEG	Background slowing Multifocal epileptiform abnormality Frequent focal seizures (EEG-only and clinical)	Unifocal seizures
Age at onset		<2 years	<1 or > 30 years
Development at onset	Acute encephalopathy	Previous intellectual impairment
Neurological exam		Abnormal prior to seizure onset
Imaging			Other structural cause for the epilepsy
Other tests			Testing shows an alternate cause e.g. CNS infection, autoantibody, toxic
Course of illness		Lack of drug resistant focal/multifocal epilepsy Lack of cognitive or intellectual impairment Lack of cerebral atrophy on MRI
Are MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis to exclude other causes An ictal EEG is required to confirm frequency of seizures and multifocal seizure onset
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome cannot be diagnosed without EEG and MRI

NOTE Alert criteria are absent in the vast majority of patients with the syndrome, but rarely can be seen. Their presence should result in caution in diagnosing the syndrome and consideration of other conditions