The tauopathies are one of the families of proteinopathies causing neurodegenerative diseases. They are characterized by a combination of cognitive and motor disorders. In this article, we summarize the clinical features of progressive supranuclear palsy and cortico-basal degeneration, focusing on their cognitive-behavioral impairment profiles, which in some cases allow them to be differentiated from other neurodegenerative entities. Finally, we propose tools for therapeutic management.