SELF-LIMITED FAMILIAL NEONATAL-INFANTILE EPILEPSY (SeLFNIE)

	Mandatory	Alerts	Exclusionary
Seizures	Focal tonic seizures with head and eye deviation, followed by other tonic and clonic features	Sequential seizures	Epileptic spasms Myoclonic seizures
EEG		Mild background slowing	Persistent focal slowing Moderate or greater widespread slowing
Age at onset	1 day to 23 months
Development at onset		A history of acute provoked seizures	Encephalopathy
Neurological exam		Abnormal exam
Imaging			Structural cause for the epilepsy
Other tests		Lack of an identified genetic cause (usually SCN2A)	Lack of family history of both neonatal and infantile onset focal seizures
Course of illness		Mild developmental impairment Lack of remission of epilepsy by 2 years of age Drug-resistant epilepsy	Moderate or greater developmental impairment
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 a neonate or infant with a family history suggestive of SeLFNIE 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 SeLFNIE, 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