Overview
Frontotemporal lobar degeneration (FTLD) is the second most common form of early-onset dementia after Alzheimer's disease (AD), accounting for 5-10 percent of all dementia patients and 10-20 percent of patients with an onset of dementia before 65 years. Up to 50 percent of FTLD patients report a family history of dementia, suggesting a strong genetic component to the disease. FTLD usually affects patients in midlife, but onset of symptoms can vary between the age of 35 and 75 years. The most common clinical manifestation of FTLD is frontotemporal dementia (FTD), whereby patients present with an early alteration in personality and social conduct, characterized by inertia, loss of volition, and disinhibition, followed by more general cognitive decline, eventually leading to dementia. Sometimes, FTD is accompanied by signs of parkinsonism or by amyotrophic lateral sclerosis (FTD-ALS). The neuropathology associated with most FTD patients is characterized by atrophy of the frontal and temporal lobes and the presence of abnormal intracellular protein aggregates of the tau protein (FTLD-tau) or the TAR DNA-binding protein 43 (FTLD-TDP).
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