Chapter 8 - Frontotemporal dementia

Abstract

Frontotemporal dementia (FTD) is a collective term for devastating dementias characterized by behavioral and personality changes or language disturbances due to neurodegeneration of the frontal and temporal areas of the brain. Currently no disease-modifying therapies are available. Research into the genetics and disease mechanisms of FTD has pointed toward novel putative drug targets in the different pathogenetic subtypes of FTD, including FTLD–tau caused by mutations in the microtubule-associated protein tau, and FTLD–TDP, caused by mutations in C9orf72, or granulin (GRN). Some targets are currently taken into preclinical research and early stages of clinical trials. In this chapter, we review current symptomatic disease management, as well as promising targets for disease-modifying therapies. Both strategies to target tau, C9orf72, or TDP-43-mediated toxicity, as well as approaches to enhance granulin expression will be discussed.