Frontotemporal dementia (FTD) encompasses a group of behavioral and movement disorders. FTD is the third most common cause of early-onset dementia after Alzheimer disease and vascular dementia. Mutations in seven genes cause FTD, with those in tau (MAPT), chromosome 9 open reading frame 72 (C9ORF72), and progranulin (GRN) being the most common. Frontotemporal dementia (FTD) is a neuropathologically and clinically heterogeneous disorder characterized by focal degeneration of the frontal and/or temporal lobes.
The primary initial clinical manifestations include changes in personality and social behavior or language. Approximately 10% of cases of FTD are inherited in an autosomal-dominant manner while 40% of patients with FTD have a family history. The symptoms correlate better with specific patterns of brain atrophy than with the underlying neuropathology. Genetics of frontotemporal…
