Frontotemporal lobar degenerations (FTLDs) comprise a spectrum of complex and heterogeneous neurodegenerative disorders characterized by degeneration of the frontal and anterior temporal lobes involving multiple clinical phenotypes, different protein aggregates (tau, TDP-43, fused in sarcoma protein-FUS) in tissue pathology and many genetic loci explaining up to 40% of familial FTLD [140]. This evidence concerns the gene TARDBP and frontotemporal dementia.