Frontotemporal Lobar Degeneration (FTLD)

Frontotemporal lobar degeneration (FTLD) is a pathologically and clinically heterogeneous syndrome that is characterized by continual decline in language and behavior associated with degeneration of the anterior and frontal temporal lobes. While the initial cases were discovered at the beginning of the 20th century, it has only been recent where FTLD has been labeled as a leading cause of dementia, particularly in patients under 65 years of age. There are three distinct clinical variants of FTLD: 1. behavioral-variant frontotemporal dementia which is characterized by changes in personality and behavior in association with frontal-predominant cortical degeneration; 2. semantic dementia, a syndrome of progressive knowledge loss of words and objects associated with anterior temporal neuronal loss; and 3. progressive nonfluent aphasia, characterized by grammar loss and motor speech deficits in the setting of left perisylvian cortical atrophy.