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Can progressive and non-progressive behavioural variant frontotemporal dementia be distinguished at presentation?
  1. M Hornberger1,
  2. B P Shelley2,3,
  3. C M Kipps2,4,
  4. O Piguet1,
  5. J R Hodges1,2
  1. 1
    Prince of Wales Medical Research Institute, University of New South Wales, Sydney, Australia
  2. 2
    Department of Clinical Neurosciences, University of Cambridge, UK
  3. 3
    Department of Neurology, Father Muller Medical College, Mangalore, India
  4. 4
    Wessex Neurological Centre, Southampton University NHS Trust, Southampton, UK
  1. Professor J Hodges, Prince of Wales Medical Research Institute, Cnr Barker and Easy Street, Randwick, NSW 2031, Sydney, Australia; j.hodges{at}powmri.edu.au

Abstract

Background: Recent findings suggest that patients with behavioural variant frontotemporal dementia (bv-FTD) differ in their disease progression (progressive vs non-progressive patients). The current study investigates whether the two groups can be discriminated by their clinical features at first presentation.

Methods: Archival clinical data of the Early Onset Dementia Clinic, Cambridge, UK, were analysed for 71 patients with bv-FTD: 45 progressive and 26 non-progressive cases with more than 3 years of follow-up.

Results: The subgroups were largely indistinguishable on the basis of the presenting clinical features but could be distinguished on general cognitive (Addenbrooke’s Cognitive Examination-revised) and selected supportive diagnostic features (distractibility, stereotypic speech, impaired activities of daily living (ADLs) and current depression).

Conclusions: Progressive and non-progressive patients are difficult to differentiate on the basis of current clinical diagnostic criteria for FTD but a combination of general cognitive, executive dysfunction and impaired ADL measures appear to be the most promising discriminators.

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Footnotes

  • Funding: This research was funded in part by an MRC program grant to JRH. JRH is supported by an Australian Research Council Federation Fellowship (FF0776229). OP is supported by a National Health and Medical Research Council of Australia Clinical Career Development Award Fellowship (#510184).

  • Competing interests: None.

  • Ethics approval: The study was approved by Addenbrooke’s Hospital Local Research Ethics Committee, Cambridge, UK.