The behavioral variant of frontotemporal dementia: Linking neuropathology to social cognition

Chiara Cerami, Stefano F. Cappa

Research output: Contribution to journalArticle

Abstract

The behavioral variant of frontotemporal dementia (bvFTD) is one of the most frequent neurodegenerative disorders with a presenile onset. It is characterized by a long phase of subclinical behavioral changes and social conduct disorders, associated with a progressive modification of personality. Recently, an international consortium of experts developed revised guidelines for its clinical diagnosis, which highlight the supportive role of biomarkers in the diagnostic process. According to new criteria, bvFTD can be classified in "possible" (requiring three of six specific clinical features), "probable" (in the presence of functional disability and typical neuroimaging features), and "with definite frontotemporal lobar degeneration" (requiring the presence of a known causal mutation or a histopathological confirmation). Familial aggregation is frequently reported in bvFTD and frontotemporal lobar degeneration in general, with an autosomal dominant transmission in about 10 % cases. The aim of this paper is to review and discuss recent advances in the knowledge of clinical, neuropsychological, and imaging features of bvFTD. We also briefly summarize the available genetic information about the frontotemporal lobar degeneration spectrum.

Original languageEnglish
Pages (from-to)1267-1274
Number of pages8
JournalNeurological Sciences
Volume34
Issue number8
DOIs
Publication statusPublished - Aug 2013

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Keywords

  • [ F]FDG PET imaging
  • Behavioral variant of frontotemporal dementia
  • Frontotemporal lobar degeneration
  • Social cognition disorders
  • Voxel-based morphometry

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology

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