Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia

Katya Rascovsky, John R. Hodges, David Knopman, Mario F. Mendez, Joel H. Kramer, John Neuhaus, John C. Van Swieten, Harro Seelaar, Elise G P Dopper, Chiadi U. Onyike, Argye E. Hillis, Keith A. Josephs, Bradley F. Boeve, Andrew Kertesz, William W. Seeley, Katherine P. Rankin, Julene K. Johnson, Maria Luisa Gorno-Tempini, Howard Rosen, Caroline E. Prioleau-LathamAlbert Lee, Christopher M. Kipps, Patricia Lillo, Olivier Piguet, Jonathan D. Rohrer, Martin N. Rossor, Jason D. Warren, Nick C. Fox, Douglas Galasko, David P. Salmon, Sandra E. Black, Marsel Mesulam, Sandra Weintraub, Brad C. Dickerson, Janine Diehl-Schmid, Florence Pasquier, Vincent Deramecourt, Florence Lebert, Yolande Pijnenburg, Tiffany W. Chow, Facundo Manes, Jordan Grafman, Stefano F. Cappa, Morris Freedman, Murray Grossman, Bruce L. Miller

Research output: Contribution to journalArticlepeer-review


Based on the recent literature and collective experience, an international consortium developed revised guidelines for the diagnosis of behavioural variant frontotemporal dementia. The validation process retrospectively reviewed clinical records and compared the sensitivity of proposed and earlier criteria in a multi-site sample of patients with pathologically verified frontotemporal lobar degeneration. According to the revised criteria, 'possible' behavioural variant frontotemporal dementia requires three of six clinically discriminating features (disinhibition, apathy/inertia, loss of sympathy/empathy, perseverative/compulsive behaviours, hyperorality and dysexecutive neuropsychological profile). 'Probable' behavioural variant frontotemporal dementia adds functional disability and characteristic neuroimaging, while behavioural variant frontotemporal dementia 'with definite frontotemporal lobar degeneration' requires histopathological confirmation or a pathogenic mutation. Sixteen brain banks contributed cases meeting histopathological criteria for frontotemporal lobar degeneration and a clinical diagnosis of behavioural variant frontotemporal dementia, Alzheimer's disease, dementia with Lewy bodies or vascular dementia at presentation. Cases with predominant primary progressive aphasia or extra-pyramidal syndromes were excluded. In these autopsy-confirmed cases, an experienced neurologist or psychiatrist ascertained clinical features necessary for making a diagnosis according to previous and proposed criteria at presentation. Of 137 cases where features were available for both proposed and previously established criteria, 118 (86) met 'possible' criteria, and 104 (76) met criteria for 'probable' behavioural variant frontotemporal dementia. In contrast, 72 cases (53) met previously established criteria for the syndrome (P

Original languageEnglish
Pages (from-to)2456-2477
Number of pages22
Issue number9
Publication statusPublished - 2011


  • behavioural variant frontotemporal dementia
  • diagnostic criteria
  • frontotemporal lobar degeneration
  • FTD
  • pathology

ASJC Scopus subject areas

  • Clinical Neurology


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