Thus far, six different intronic loci with the pathogenic pentanucleotide RE combination have been described in FAME patients [SAMD1 [8], STARD7 [29], MARCH6 [30], YEATS2 [31], TNRC6A [8] and RAPGEF2 [8] (Table 1)]. Here, RAPGEF2 is linked to benign adult familial myoclonic epilepsy.