Frontotemporal Dementia

Neurol Clin. 2017 May;35(2):339-374. doi: 10.1016/j.ncl.2017.01.008.


Frontotemporal dementia (FTD) is a heterogeneous disorder with distinct clinical phenotypes associated with multiple neuropathologic entities. Presently, the term FTD encompasses clinical disorders that include changes in behavior, language, executive control, and often motor symptoms. The core FTD spectrum disorders include behavioral variant FTD, nonfluent/agrammatic variant primary progressive aphasia, and semantic variant PPA. Related FTD disorders include frontotemporal dementia with motor neuron disease, progressive supranuclear palsy syndrome, and corticobasal syndrome. In this article, the authors discuss the clinical presentation, diagnostic criteria, neuropathology, genetics, and treatments of these disorders.

Keywords: Corticobasal syndrome (CBS); Frontotemporal dementia (FTD); Motor neuron disease; Nonfluent PPA; Primary progressive aphasia; Progressive supranuclear palsy (PSP); Semantic PPA.

Publication types

  • Historical Article
  • Review

MeSH terms

  • Brain / metabolism
  • Brain / pathology*
  • Frontotemporal Dementia / epidemiology
  • Frontotemporal Dementia / history
  • Frontotemporal Dementia / pathology*
  • Frontotemporal Dementia / physiopathology*
  • History, 20th Century
  • Humans
  • Supranuclear Palsy, Progressive / pathology
  • Supranuclear Palsy, Progressive / physiopathology