Clinicopathological correlations have revealed that certain clinical subgroups are associated predominantly with either Tau or TDP-43 neuropathology, with all individuals with FTD-MND having TDP-43 immunopositive inclusions and most individuals with CBS, PSPS and PNFA having Tau inclusions at autopsy (Hodges et al., 2004; Schofield et al., 2012). This evidence concerns the gene TARDBP and frontotemporal dementia.