Frontotemporal Dementia

Abstract

Frontotemporal dementia (FTD) is a progressive neurological syndrome with diverse clinical presentations and underlying pathologies. FTD is a common cause of dementia, particularly among individuals younger than age 65 years. The three core FTD syndromes are behavioral variant FTD, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia. Neuropsychiatric symptoms and language difficulties are common in FTD, but the heterogeneity of clinical presentations poses significant challenges that can impact accurate diagnosis and patient care. A comprehensive clinical assessment involving the evaluation of cognition, socioemotional functioning, neuroimaging, and other in vivo biomarkers assists with early diagnosis and treatment planning. As the understanding of genetic risk factors and the molecular basis for FTD improves, promising pharmacological therapies are being developed. This chapter summarizes the clinically relevant findings typically seen in each of the three FTD subtypes. It reviews the spectrum of underlying pathological and genetic substrates, addresses common diagnostic challenges, and summarize best practices associated with management of FTD.