ABSTRACT
Frontotemporal dementias (FTDs) are a group of heterogenous clinical syndromes marked by changes in behavior, personality, cognition, and mood. Clinical syndromes are characterized based on early signs and symptoms, including changes in behavior (behavioral variant FTD; bvFTD), language (nonfluent variant FTD, nfvFTD; semantic variant FTD, svFTD), and motor function (FTD-Motor Neuron Disease; Progressive Supranuclear Palsy, PSP; Corticobasal Syndrome; CBS), with significant overlap amongst syndromes. FTD typically presents at an earlier age of onset than other neurodegenerative disorders, most commonly between the ages of 45 and 64 years old. Progressive frontal and temporal lobe neurodegeneration drives symptom progression, with considerable variability based on the underlying protein pathology. Although FTD is typically sporadic, between 10% and 40% of cases are associated with an autosomal dominant pattern of genetic inheritance. There are no approved treatments that effectively target disease pathology; however, behavioral and environmental modifications, family and caregiver education and support, and symptom management can be effective.
