Five cases with a clinical diagnosis of bvFTD received a pathological diagnosis: one had frontotemporal lobar degeneration with TDP-43 pathology type 3; one had a tauopathy with features compatible with chronic traumatic encephalopathy; one had Pick’s disease; one had FTLD-TDP Type A; and one had mixed AD, Lewy body pathology, and TDP 43 pathology. Here, TARDBP is linked to tauopathy.