Brain, Vol. 122, No. 8, 1469-1493,
August 1999
© 1999 Oxford University Press
Specific cognitive deficits in mild frontal variant frontotemporal dementia
1 Departments of Psychiatry and 2 Neurology, 3 MRC Cognition and Brain Sciences Unit, 4 Department of Experimental Psychology and the 5 MRC Centre for Brain Repair, University of Cambridge, Cambridge, UK
Correspondence to:
Dr Barbara J. Sahakian, Department of Psychiatry, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital, Cambridge CB2 2QQ, UK
Eight patients with relatively mild frontal variant frontotemporal dementia (fvFTD) were compared with age- and IQ-matched control volunteers on tests of executive and mnemonic function. Tests of pattern and spatial recognition memory, spatial span, spatial working memory, planning, visual discrimination learning/attentional set-shifting and decision-making were employed. Patients with fvFTD were found to have deficits in the visual discrimination learning paradigm specific to the reversal stages. Furthermore, in the decision-making paradigm, patients were found to show genuine risk-taking behaviour with increased deliberation times rather than merely impulsive behaviour. It was especially notable that these patients demonstrated virtually no deficits in other tests that have also been shown to be sensitive to frontal lobe dysfunction, such as the spatial working memory and planning tasks. These results are discussed in relation to the possible underlying neuropathology, the anatomical connectivity and the hypothesized heterogeneous functions of areas of the prefrontal cortex. In particular, given the nature of the cognitive deficits demonstrated by these patients, we postulate that, relatively early in the course of the disease, the ventromedial (or orbitofrontal) cortex is a major locus of dysfunction and that this may relate to the behavioural presentation of these patients clinically described in the individual case histories.
frontotemporal dementia; decision-making; reversal learning; orbitofrontal prefrontal cortex; ventromedial prefrontal cortex
ANOVA = analysis of variance; CANTAB = Cambridge Neuropsychological Test Automated Battery; FTD = frontotemporal dementia; fvFTD = frontal variant FTD; HMPAO = 99mTc-hexamethyl-propyleneamineoxide; MMSE = Mini-Mental State Examination; NART = National Adult Reading Test; SPECT = single photon emission computed tomography; WAIS = Wechsler Adult Intelligence Scale; WCST = Wisconsin Card Sorting Test
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