Benign neonatal sleep myoclonus: An autosomal dominant form not allelic to KCNQ2 or KCNQ3

Zaid Afawi, Haim Bassan, Sarah Heron, Karen Oliver, Rachel Straussberg, Ingrid Scheffer, Richard Leventer, Amos Korczyn, Samuel Berkovic*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Benign neonatal sleep myoclonus is an uncommon, nonepileptic disorder characterized by myoclonic jerks appearing in the neonatal period that occur predominantly during sleep. Although self-limiting, the disorder is frequently confused with epileptic neonatal seizures. A few familial cases have been reported; however the genetics has not been studied. We ascertained 3 families with 2 or more affected individuals and analyzed the pedigrees. We used microsatellite markers to determine if the disorder was possibly linked to KCNQ2 or KCNQ3, the 2 genes that cause most cases of benign familial neonatal seizures, a disorder that it could be easily confused with. The 3 pedigrees, including one with 4 affected individuals, were suggestive of autosomal dominant inheritance. The loci for KCNQ2 and KCNQ3 were excluded in the 2 larger families. We conclude that benign neonatal sleep myoclonus can show autosomal dominant inheritance and is not allelic with benign familial neonatal seizures.

Original languageEnglish
Pages (from-to)1260-1263
Number of pages4
JournalJournal of Child Neurology
Issue number10
StatePublished - Oct 2012


  • epilepsy semiology
  • genetic linkage
  • neonatal myoclonus
  • neonatal seizures


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