Recent progress in diagnostic criteria of non-Alzheimer degenerative dementias is reviewed. These dementias comprise frontotemporal dementias (including hereditary dementias), primary progressive aphasia and anarthria, corticobasal degeneration, progressive supranuclear palsy and dementia with Lewy bodies. The approach of studying these diseases has changed considerably with genetic and biochemical analyses. A molecular classification is suggested and the clinical significance of this classification is discussed.