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  • Updated 08.28.2023
  • Released 10.18.1993
  • Expires For CME 08.28.2026

Self-limited (familial) neonatal epilepsy



Benign familial neonatal seizures is a syndrome characterized by focal clonic seizures, occasionally with a generalized tonic-clonic component, that occurs in full-term neonates. The seizures usually begin within 2 to 8 days of birth and remit by 16 months. As the name implies, this syndrome is usually familial and self-limited, but a work-up is necessary to rule out other causes of seizures. This syndrome is caused by a mutation in the gene that encodes voltage-gated K+ channel subunits (KCNQ2, KCNQ3). These subunits are responsible for the M-current, which is important in neuronal stability. There is no specific treatment at this time; however, there are several drugs for which the mechanism of action looks promising.

Key points

• Seizures occur in full-term neonates, usually beginning within 2 to 8 days from birth.

• The disorder is familial and benign.

• There is no specific treatment. Antiseizure medications are sometimes used.

• This syndrome is caused by mutations in the gene encoding voltage-gated K+ channels.

Historical note and terminology

Antoni described a hereditary form of neonatal seizures with good outcomes for neurologic development (03). Rett and Teubel were the first to describe the syndrome in a family (50). The term “benign” was not in the original description by Rett but was added 4 years later. Interestingly, a reanalysis of Rett’s original kindred 40 years later revealed that they met current clinical and genetic criteria for benign familial neonatal seizures (68). The first case reported in the English literature appeared in 1968 (06). Since 1989, benign familial neonatal seizures have been included in the classification of epilepsy and epilepsy syndromes.

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