Pathologically, FTD is characterized by the accumulation of Tau, TDP-43 or FUS protein which leads to atrophy of the frontal and temporal regions of the brain referred to as frontotemporal lobar degeneration (FTLD) (2) Mutations in progranulin (GRN) account for 5–20% of familial FTLD-TDP and 1–5% of sporadic cases. Here, FUS is linked to frontotemporal dementia.