The effects of ketogenic diet treatment in KCNA1-null mouse, a model of sudden unexpected death in epilepsy

Abstract

Sudden unexpected death in epilepsy (SUDEP) is a leading cause of abrupt death in patient with epilepsy. It represents 5-30% of all rapid deaths in individuals with epilepsy. Ketogenic diet (KD) has been used in clinic for treatment of epilepsy for many decades. However, the cellular and molecular mechanisms underlying the SUDEP and the relationship between KD and SUDEP remain uncertain. Kcna1-null (Kcna1−/−) mouse, an animal model of SUDEP, is frequently used to study mechanisms underlying SUDEP. The current mini-review focus on risk factors for SUDEP and their relationship with KD treatment in Kcna1−/− mice. Emerging data suggest that factors including seizure frequency, longevity, rest, age, and gender both in Kcna1−/− mice and KD treated Kcna1−/−mice are involved in SUDEP. This provides valuable prediction for clinical application of KD for treatment of SUDEP.