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

Date

2012

Authors

Afawi, Z.
Bassan, H.
Heron, S.
Oliver, K.
Straussberg, R.
Scheffer, I.
Leventer, R.
Korczyn, A.
Berkovic, S.

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Journal of Child Neurology, 2012; 27(10):1260-1263

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

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Abstract

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.

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© The Author(s) 2012

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