In summary, our findings reveal several relationships across motor, cognitive executive, and psychiatric symptom domains in male carriers of FMR1 premutation alleles diagnosed with FXTAS, which, alongside features of cerebellar ataxia and the core peduncular damage, is reminiscent of impairments commonly seen in other disorders involving disruption to cerebro-cerebellar pathways. This evidence concerns the gene FMR1 and fragile X-associated tremor/ataxia syndrome.