• Lauren Massimo, David J Libon, Keerthi Chandrasekaran, Michael Dreyfuss, Corey T McMillan, Katya Rascovsky, Ashley Boller, and Murray Grossman.
• Department of Neurology, University of Pennsylvania, 3400 Spruce Street, Gates Building, Philadelphia, PA 19104, USA. mgrossma@mail.med.upenn.edu
• J. Neurol. Neurosurg. Psychiatr.. 2013 Feb 1;84(2):148-53.

ObjectivePrevious 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.Methods49 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.ResultsbvFTD 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).ConclusionPoor self-appraisal in bvFTD affects multiple domains, and this multi-domain impairment pattern is associated with frontal disease in the subgenual cingulate.

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