As the FMR1 gene is located on the X chromosome, the male carriers are mostly affected, however the predominance is incomplete, with the current estimate of 16–20% females and 40–75% males to develop FXTAS (Seltzer et al., 2012; Tassone et al., 2012; Hunter et al., 2014). Here, FMR1 is linked to fragile X-associated tremor/ataxia syndrome.