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.
Highlights
Epilepsy is a common neurological disease defined by recurrent seizures [1]
The typical cause of death associated with epilepsy is sudden unexpected death in epilepsy (SUDEP) [2]
There are several risk factors associated with SUDEP including generalized tonic-clonic (GTC) seizure frequency, postictal generalized electroencephlogram suppression, lower intelligence quotient, cardiac arrhythmias, respiratory dysfunction, gender, early age at seizure onset, and polytherapy with antiseizure drugs (ASDs) [2, 6,7,8]
Summary
The typical cause of death associated with epilepsy is sudden unexpected death in epilepsy (SUDEP) [2]. Recent studies demonstrate that genes are associated with SUDEP. It includes KCN (A1, Q1, and H2), SCN (1A, 2A, 5A, and 8A), DEPDC5, HCN2, RYR3, and HTR2C. They are potential risk factors for SUDEP [8,9,10]. The high-fat, low-carbohydrate/protein ketogenic diet (KD) is recognized as an effective, non-surgical treatments for refractory epilepsy [11]. KD treatment for epilepsy has been recorded since the fifth century and reported in scientific literatures since the early 1900s. Patients with epilepsy are strongly accept the KD therapy [11]
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