Johns Hopkins Psychiatry Guide

Frontotemporal Dementia

Tamela McClam, M.D., Chiadi Onyike‎, M.D.
Frontotemporal Dementia is a topic covered in the Johns Hopkins Psychiatry Guide.

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DEFINITION

  • Frontotemporal dementia (FTD) is a clinically and histopathologically heterogeneous neurodegenerative disorder that presents as a focal disorder of conduct, temperament, judgment, and/or speech and language.
  • Three canonical syndromes are recognized:
    • Behavior variant FTD (bvFTD): the commonest; characterized by a focal decline in conduct, temperament, and judgment
    • Primary progressive aphasia (PPA) variants: primary non-fluent aphasia features halting, telegraphic speech with agrammatism and phonological errors
    • Semantic dementia: manifests progressive anomia, with focal loss of word and object knowledge, and fluent and vacuous speech with semantic errors
  • In time, the illness includes a wider range of cognitive deficits.
  • Frontotemporal lobar degeneration refers to the histopathological state that underlies the clinical syndromes.
  • Cognitive impairment due to frontotemporal dementia is classified under the neurocognitive disorders (NCDs) section of the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5).[1]

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Last updated: March 1, 2017