Myotonic dystrophy (DM) is a multi-systemic, progressive disease caused by expanded CTG or CCTG repeats in the 3′ UTR of the dystrophia myotonic protein kinase (DMPK) gene (DM type 1 [DM1]) (Brook et al., 1992) or the first intron of the cellular nucleic acid binding protein (CNBP) gene (DM type 2 [DM2]) (Liquori et al., 2001), respectively. This evidence concerns the gene CNBP and myotonic dystrophy type 2.