Neuropathologically, FTLD can be classified into different subgroups: FTLD-Tau that includes 3-repeated (Pick’s disease (PiD)) and 4-repeated tau (PSP, CBD, argyrophilic grain disease (AGD)), FTLD with TDP-43 inclusions (type A, B, C and D), FTLD with FET protein accumulation (atypical FTLD-U, basophilic inclusion body disease (BIBD), neuronal intermediate filament inclusion disease (NIFID)) and FTLD ubiquitin/proteasome system (FTLD-UPS) [7]. The gene discussed is HMBS; the disease is argyrophilic grain disease.