The neuropathology nomenclature of FTD can be categorized into four major patterns depending on the dominant protein accumulation: (1) tau-positive inclusion pathology (FTLD-tau), (2) Tar DNA binding protein 43 (TDP-43) positive inclusion pathology (FTLD-TDP), (3) FET protein family pathology (FTLD-FET), including fused in sarcoma (FUS) protein, (4) immunoreactive for ubiquitin and negative for tau, TDP-43, and FET inclusion pathology (FTLD-UPS) [4]. This evidence concerns the gene FUS and frontotemporal dementia.