Although ALS, FTD–ALS and roughly half of bvFTD cases are characterized by intracellular protein inclusions of TAR DNA-binding protein (TDP-43)2, PSP, CBD and approximately 40% of bvFTD cases have been linked to tau pathology at autopsy (frontotemporal lobar degeneration, FTLD-tau)3. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.