Overall, PRRT2 is reported to be mutated in 83.3%-100% of ICCA families [6,8,18,31], although one study reported that the much lower rate of 37.5% [17].We reviewed the PRRT2 mutation rate in BFIE and ICCA families described in literature and our study (Additional file 3: Table S3), and found that it was higher (91.6%, 87/95) in ICCA families than in BFIE families (74.1%, 126/170). This evidence concerns the gene PRRT2 and infantile convulsions and choreoathetosis.