In addition to AD and DLB, TDP-43 or phospho-TDP-43 pathology can be found co-occurring with tau pathology in cases of CBD, PSP, PiD and argyrophilic grain disease (Higashi et al., 2007; Uryu et al., 2008; Arai et al., 2009; Fujishiro et al., 2009; Rohn and Kokoulina, 2009; Yokota et al., 2010). Here, TARDBP is linked to Alzheimer disease.