The largest consecutive bvFTD autopsy study published to date showed there is high confidence of a clinical diagnosis of bvFTD; characteristic pathology is present in 94% of cases, with 84% having pathological aggregation of tau protein (27%) or transactive response DNA binding protein 43 (TDP-43; 57%) [23]. This evidence concerns the gene TARDBP and behavioral variant of frontotemporal dementia.