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Article

Altered cardiac electrophysiology and SUDEP in a model of Dravet syndrome.

PloS one - 1 Jan 2013

Auerbach David S, Jones Julie, Clawson Brittany C, Offord James, Lenk Guy M, Ogiwara Ikuo, Yamakawa Kazuhiro, Meisler Miriam H, Parent Jack M, Isom Lori L

Abstract excerpt

OBJECTIVE: Dravet syndrome is a severe form of intractable pediatric epilepsy with a high incidence of SUDEP: Sudden Unexpected Death in epilepsy. Cardiac arrhythmias are a proposed cause for some cases of SUDEP, yet the susceptibility and potential mechanism of arrhythmogenesis in Dravet syndrome remain unknown. The majority of Dravet syndrome patients have de novo mutations in SCN1A, resulting in...

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Identifiers

PMID: 24155976
DOI: 10.1371/journal.pone.0077843
PMCID: PMC3796479

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