title: Self-appraisal in behavioural variant frontotemporal degeneration.
abstract: 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).
  
  
  CONCLUSIONS
  Poor self-appraisal in bvFTD affects multiple domains, and this multi-domain impairment pattern is associated with frontal disease in the subgenual cingulate.
authors.affiliation: Department of Neurology, University of Pennsylvania, 3400 Spruce Street, Gates Building, Philadelphia, PA 19104, USA. mgrossma@mail.med.upenn.edu
authors.forename: Lauren
authors.initials: L
authors.lastname: Massimo
authors.forename: David J
authors.initials: DJ
authors.lastname: Libon
authors.forename: Keerthi
authors.initials: K
authors.lastname: Chandrasekaran
authors.forename: Michael
authors.initials: M
authors.lastname: Dreyfuss
authors.forename: Corey T
authors.initials: CT
authors.lastname: McMillan
authors.forename: Katya
authors.initials: K
authors.lastname: Rascovsky
authors.forename: Ashley
authors.initials: A
authors.lastname: Boller
authors.forename: Murray
authors.initials: M
authors.lastname: Grossman
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000544:Alzheimer Disease / Q000473:pathology / Q000523:psychology*; D001284:Atrophy; D059026:Diagnostic Self Evaluation; D057180:Frontotemporal Dementia / Q000473:pathology* / Q000523:psychology*; D006801:Humans; D008875:Middle Aged; D036421:Nerve Fibers, Unmyelinated / Q000473:pathology; D009483:Neuropsychological Tests / Q000706:statistics & numerical data