Here we describe three cases, including one with a variation in the PGRN gene, with a clinical presentation reminiscent of PSP ("PSP-like syndrome") in patients with FTLD-TDP (with or without MND) that supports the notion of a further, clinically distinguishable, phenotype within the spectrum of TDP-43 proteinopathies. Here, GRN is linked to supranuclear palsy, progressive, 1.