The RNA toxicity due to the elevated expression levels of the FMR1 mRNA leads to oxidative stress, mitochondrial dysfunction, calcium dysregulation and sequestration of proteins important to neuronal function.3-13 Eosinophilic, tau-negative intranuclear inclusions are present in neurons and astrocytes throughout the CNS and in the peripheral nervous system (PNS).14,15 FXTAS is a late-onset disorder, usually occurring in the 60s, though some patients have an earlier onset, and its signs and symptoms worsen with age. This evidence concerns the gene FMR1 and fragile X-associated tremor/ataxia syndrome.