What are the signs and symptoms of frontotemporal dementia (FTD)?

Updated: Jun 14, 2018
  • Author: Howard S Kirshner, MD; Chief Editor: Jasvinder Chawla, MD, MBA  more...
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Answer

Answer

In England and Europe, cases of frontal lobe dementia were described with progressive dysfunction of the frontal lobes. In a series of case reports, Neary and Snowden outlined a syndrome with initial symptoms that were suggestive of psychiatric illness. However, the following frontal lobe behavioral abnormalities appeared over time:

  • Disinhibition

  • Impulsivity

  • Impersistence

  • Inertia

  • Loss of social awareness

  • Neglect of personal hygiene

  • Mental rigidity, stereotyped behavior

  • Utilization behavior - Ie, a tendency to pick up and manipulate any object in the environment

These descriptions included language abnormalities such as reduced speech output, mutism, echolalia, and perseveration.

The condition described in the North American literature as primary progressive aphasia and that described in the European literature as frontal dementia have been combined under the term frontotemporal lobe dementia (FTD), or frontotemporal lobar degeneration (FTLD). Within this grouping, the frontal lobe syndrome described by Neary and Snowdon, [8, 9] is referred to as, interchangeably, behavioral variant frontotemporal lobe dementia (bvFTD) or frontal variant frontotemporal lobe dementia (fvFTD). The progressive aphasias have been divided into 3 groups: progressive nonfluent aphasia, semantic dementia, and logopenic progressive aphasia. [10, 11, 12]

In recent years, the term frontotemporal dementia has become an umbrella term referring to clinical syndromes of frontal dementia or progressive aphasia. An alternate term, frontotemporal lobar degeneration, relates to pathologies associated with the frontotemporal lobe dementia syndromes. In this review, the 2 terms are used more or less synonymously.


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