SELF-LIMITED (FAMILIAL) NEONATAL EPILEPSY (SeLNE)

	Mandatory	Alerts	Exclusionary
Seizures	Focal motor seizures with tonic or clonic features	Clinical history suggestive of in utero seizures	Epileptic spasms Myoclonic seizures Generalized tonic seizures Generalized tonic-clonic seizures
EEG		Mild background slowing	Persistent focal slowing Moderate or greater widespread slowing Burst suppression pattern hypsarrhythmia Lack of ictal EEG patterns with clinical events
Age at onset			Onset after 44 weeks post-menstrual age
Development at onset			Encephalopathy
Neurological exam		Abnormal exam
Imaging			Structural cause for the seizures
Other tests		Expected genetic cause not found (usually KCNQ2 or KCNQ3 Lack of family history in keeping with autosomal dominant inheritance with incomplete penetrance	Identified cause for acute provoked seizures
Course of illness		Mild developmental impairment Lack of epilepsy remission by 6 months of age Drug-resistant epilepsy	Moderate-severe 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 with a family history suggestive of SeLFNE 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 SeLNE, 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