Genetic models of focal epilepsies

•Several focal epilepsy syndromes are proven to be monogenic disorders.•Mutations in CHRNA4, CHRNB2, CHRNA2 and KCNT1 cause autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE).•Mutations in LGI1 cause autosomal dominant epilepsy with auditory features (ADEAF).•This review provides an update of the mutational spectrum in these genes.•We review cellular and genetic animal models generated for autosomal dominant focal epilepsies.