Picks disease is a major clinicopathological disease having circumscribed atrophy in the frontotemporal lobe. Demented patients with frontotemporal atrophy are now clinically diagnosed as frontotemporal lobar degeneration (FTLD). Other underlying pathologies in patients with FTLD include FTLD with TDP-43-positive inclusions, corticobasal degeneration, progressive supranuclear palsy, basophilic inclusion body disease, neuronal intermediate filament inclusion disease and argyrophilic grain disease. In this chapter, recent findings regarding the distinct clinical and histopathological features of these pathological disease entities are presented including the discussion on the possibility of future antemortem diagnosis of patients with the disease.In this chapter, recent findings regarding the distinct clinical and histopathological features of these pathological disease entities are presented including the discussion on the possibility of future antemortem diagnosis of patients with the disease.