SELF-LIMITED (FAMILIAL) INFANTILE EPILEPSY (SeLIE)

	Mandatory	Alerts	Exclusionary
Seizures	Focal seizures, with behavioral arrest, impaired awareness, automatisms, and motor features; usually brief (<3 min)	Prolonged seizures > 10 minutes	Epileptic spasms Myoclonic seizures Sequential seizures Tonic seizures
EEG		Mild background slowing	Persistent focal slowing Moderate or greater widespread slowing Hypsarrhythmia
Age at onset		18-36 months	<1 month, >36 months
Development at onset		Mild developmental impairment	Moderate or greater developmental impairment
Neurological exam		Neurological examination abnormality
Imaging			Structural cause for the epilepsy
Other tests		Lack of pathogenic variant in PRRT2, SCN2A, SCN8A, KCNQ2/3	Lack of family history suggesting autosomal dominant inheritance with incomplete penetrance
Course of illness		Lack of remission of epilepsy by late childhood	Neurocognitive regression with myoclonic seizures, spasticity, ataxia (consider CLN2)
Are MRI or ictal EEG required for diagnosis? An MRI is required for diagnosis to exclude other causes. An ictal EEG is not required.
Syndrome without laboratory confirmation: in resource-limited regions, this syndrome can be diagnosed without EEG or MRI in an infant with a family history suggestive of familial SeLIE who meets all other mandatory and exclusionary clinical criteria and has no alerts. However, the clinical history of affected family members should be consistent with the expected course for SeLIE, and careful follow-up of the patient is required to ensure their course is also consistent with this syndrome.

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