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Sudden Unexplained Death In Epilepsy

(DS) is a form of infantile-onset, that is caused by a mutation in the gene encoding a voltage-gated sodium channel, SCN1A. have a 30-fold increased risk of dying from sudden in epilepsy () compared to patients with other forms of pediatric-onset epilepsy.

In this issue of the Journal of Clinical Investigation, and colleagues at the University of Washington characterized SUDEP in a mouse model of DS. Observation using video, electroencephalography, and electrocardiography revealed that a prolonged slowing of the heart beat preceded SUDEP in mice.

Treatment with with drugs that reduce activity in the parasympathetic nervous system reduced the incidence of SUDEP, suggesting that mortality results from seizure-related parasympathetic hyperactivity.

In a companion Attending Physician, Orrin Devinsky and colleagues discuss how these results could relate to SUDEP in human DS patients.


TITLE: Sudden unexpected death in a mouse model of Dravet Syndrome


ACCOMPANYING THE ATTENDING PHYSICIAN TITLE: Sudden death in epilepsy: Of mice and men


Journal of Clinical Investigation