Background: Despite the extensive frontal atrophy and behavioral disinhibition that characterizes behavioral variant frontotemporal dementia (bvFTD), many studies of early bvFTD suggest normal executive functioning (EF). The current study examined cognitive control in patients with bvFTD who otherwise seemed cognitively normal.

Methods: Subjects included 7 patients with bvFTD with normal neuropsychological test scores, 7 patients with bvFTD matched for Mini-Mental State Examination score but with impaired neuropsychological test scores, and 14 normal controls. A flanker paradigm and other measures of EF were administered to participants. A semiautomated parcellation program was used to analyze structural MRI scans.

Results: On the flanker task, multivariate analysis of variance revealed a significant condition X diagnosis interaction. Both bvFTD groups showed a larger congruency effect than normal controls, i.e., they displayed disproportionately reduced speed and accuracy on incongruent trials relative to congruent trials. Imaging data illustrated significant orbitofrontal atrophy in patients with early bvFTD as compared with controls.

Conclusions: Patients with behavioral variant frontotemporal dementia (bvFTD) who performed within normal limits on clinical tests of executive functioning demonstrated a select impairment on an experimental test of cognitive control, suggesting a subtle impairment in inhibiting attention or response to the irrelevant stimuli. Measures of neuropsychological functioning sensitive to the ventromedial prefrontal cortex may be useful in early diagnosis of patients with bvFTD. Our understanding of this syndrome may be increased by considering the efficiency of selective inhibition, a fundamental component of executive cognitive control.

Abbreviations: ANOVA = analysis of variance; bvFTD = behavioral variant frontotemporal dementia; CDR = Clinical Dementia Rating; EF = executive functioning; FTLD = frontotemporal lobar dementia; MANCOVA = multivariate analysis of covariance; MCI = mild cognitive impairment; MMSE = Mini-Mental State Examination; MP-RAGE = magnetization-prepared rapid gradient echo; MR = magnetic resonance

Address correspondence and reprint requests to Dr. Casey E. Krueger, 350 Parnassus Ave., Suite 905, San Francisco, CA 94143-1207 ckrueger@memory.ucsf.edu