Frontotemporal dementias

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By Andrew Kertesz MD

Frontotemporal dementia (FTD) is also know as or subsumes Pick disease, Behavioral variety fronto temporal dementia, Primary progressive aphasia, and Semantic dementia. -ed.

Frontotemporal dementia, also known Pick disease is much underdiagnosed but relatively common. Initial presentations include the behavioral variety (bvFTD), aphasia (PPA), semantic dementia (SD), and corticobasal degeneration syndrome/progressive supranuclear palsy (CBDS/PSP). Eventually these converge. The clinical pattern predicts pathology such as Pick bodies, corticobasal degeneration, ubiquitinated motor neuron disease-type inclusions incompletely; tau-positive pathology is associated with aphasia and CBDS/PSP and ubiquitin positive pathology with bvFTD and SD, but the overlap is significant. Recent discoveries of tau, and progranulin mutations, the TDP-43 protein abnormality associated with the ubiquitinated inclusions, and complex nosologic issues require practitioners and researchers to update their information.