There are few reports of DCTN1 mutations in patients with FDT and MND/ALS (Münch et al., 2005; Newsway et al., 2010), variably associated with parkinsonism, hypoventilation, dysautonomia, weight loss, and behavioral disturbances configuring the recently described Perry syndrome, which is associated with TDP-43 pathology (Farrer et al., 2009; Ohshima et al., 2010). This evidence concerns the gene TARDBP and mild neurocognitive disorder.