What are the diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD)?

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

Answer

According to revised guidelines from an international consortium, 3 of the following 6 clinically discriminating features indicate possible behavioral variant FTD (bvFTD):

Disinhibition

  • Apathy/inertia

  • Loss of sympathy/empathy

  • Preservative/compulsive behaviors

  • Hyperorality

  • Dysexecutive neuropsychological profile

Functional disability and characteristic neuroimaging should also be considered when diagnosing probable bvFTD. In addition, histopathologic evidence (brain biopsy or autopsy) or proof of a pathogenic mutation is needed for a diagnosis of bvFTD with definite frontotemporal lobar degeneration (FTLD). Commercial laboratories such as Athena now offer genetic testing for the tau, progranulin, and C9ORF72 mutations. The revised criteria improve diagnostic accuracy, particularly in the earliest stages. However, the authors cautioned that future studies are needed to establish reliability and specificity. [60]


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