SELF-LIMITED (FAMILIAL) NEONATAL EPILEPSY (SeLNE)

OVERVIEW

Self-limited neonatal epilepsy (SeLNE) and self-limited familial neonatal epilepsy (SeLFNE) have the same genetic causes, but de novo pathogenic variants are responsible when there is no family history. Focal motor seizures begin between days 2-7 of age. Despite frequent seizures, the neonate behaves normally between seizures and seizures remit, without impact on development.

Clinical context

Seizures begin in the neonate between days 2-7 from 40 weeks postmenstrual age. Both sexes are affected equally. Pregnancy and birth history are unremarkable. Head size and neurological examination are normal. Development is normal, although a minority of individuals may have learning difficulties or mild motor impairment with time. Some neonates with specific pathogenic variants may have myokymia (continuous muscle activity causing stiffness and subtle twitching), which may present in later infancy. Seizures usually remit by 6 months of age, the majority remit by 6 weeks of age. Up to one third of individuals have seizures in later life, which may include febrile seizures.

NOTE Postmenstrual age is gestational age plus chronological age (in completed weeks), the neonatal period is from birth up to 44 weeks postmenstrual age.