Neuronal hyperexcitability in a mouse model of SCN8A epileptic encephalopathy

Significance Patients with early infantile epileptic encephalopathy experience severe seizures and cognitive impairment and are at increased risk for sudden unexpected death in epilepsy (SUDEP). Here, we investigated the neuronal phenotype of a mouse model of early infantile epileptic encephalopathy (EIEE) 13 caused by a mutation in the sodium channel gene SCN8A . We found that excitatory and inhibitory neurons from mutant mice had increased...