Cases were reported of two sisters presenting with basal ganglia calcifications and generalized epilepsy as main phenotype carrying mutations in the SLC20A2 and in the CHRNB2 genes (known to be associated with AD frontal lobe epilepsy) and a young boy with basal ganglia calcification and refractory epilepsy carrying mutations in the SLC20A2 and in the SCN2A genes [68,69]. This evidence concerns the gene SLC20A2 and frontal lobe epilepsy.