Based on the morphology and cortical distribution of the accumulation, the two main groups (FTLD-tau and FTLD-TDP) have been subdivided; Pick’s disease (PiD), corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), argyrophilic grain disease (AGD), globular glial tauopathy (GGT) and FTD caused by microtubule association protein tau (MAPT) for FTLD-tau [15–18] and the subtypes A, B, C, D and E for FTLD-TDP [19]. Here, MAPT is linked to argyrophilic grain disease.