J Neurol Neurosurg Psychiatry 84:148-153 doi:10.1136/jnnp-2012-303153
  • Cognitive neurology
  • Research paper

Self-appraisal in behavioural variant frontotemporal degeneration

  1. Murray Grossman1
  1. 1Department of Neurology, University of Pennsylvania, Philadelphia, Pennsylvania, USA
  2. 2Department of Neurology, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA
  3. 3Weill Cornell Medical College, Cornell University, Ithaca, New York, USA
  1. Correspondence to Dr Murray Grossman, Department of Neurology, University of Pennsylvania, 3400 Spruce Street, Gates Building, Philadelphia, PA 19104, USA; mgrossma{at}
  1. Contributors LM and MG were responsible for the conceptualisation of the study, analysis and interpretation of the data, and drafting or revising the manuscript. DL was responsible for study design, revising the manuscript, and statistical analysis. KC was responsible for analysis of the data. MD was responsible for analysis of the data. CTM was responsible for analysis and interpretation of the data. KR was responsible for analysis and interpretation of the data. AB was responsible for analysis of data as well as acquisition of data.

  • Received 8 May 2012
  • Revised 11 July 2012
  • Accepted 1 August 2012
  • Published Online First 5 September 2012


Objective Previous work investigating deficits in self-appraisal in behavioural-variant frontotemporal degeneration (bvFTD) has focused on a single domain: social/behavioural processes. We examined whether a domain-specific versus multi-domain model best explains degraded self-appraisal in bvFTD.

Methods 49 patients with bvFTD and 73 patients with Alzheimer's disease (AD) were administered quantitative assessments of episodic memory, naming and grammatical comprehension. Self-appraisal of cognitive test performance was assessed by asking patients to rate their performance immediately after completing each neuropsychological test. A discrepancy score was created to reflect the difference between patient performance on neuropsychological tests and self-appraisal of their test performance. Self-appraisal for each neuropsychological measure was related to grey matter (GM) density in each group using voxel-based morphometry.

Results bvFTD patients were poor at evaluating their own performance on all cognitive tests, with no significant correlations between self-appraisal and actual performance. By contrast, poor self-appraisal in AD was restricted to episodic memory performance. Poor self-appraisal on each task in bvFTD and AD was related to reduced GM density in several ventral and rostral medial prefrontal regions. Crucially, poor self-appraisal for all domains in bvFTD was related to a specific area of reduced GM density in the subgenual cingulate (BA 25).

Conclusion Poor self-appraisal in bvFTD affects multiple domains, and this multi-domain impairment pattern is associated with frontal disease in the subgenual cingulate.


  • Funding This work was supported in part by the John A Hartford Foundation's Building Academic Geriatric Nursing Capacity Award Program, US Public Health Service (F31NR013306, AG17586, AG15116, AG10124, NS53488 and NS44266) and the Wyncote Foundation.

  • Competing interests None.

  • Ethics approval Ethics approval was provided by University of Pennsylvania Institutional Review Board.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement The first author has full access to all of the data; and has the right to publish any and all data, separate and apart from the guidance of any sponsor.

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