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Executive dysfunction in frontotemporal dementia and corticobasal syndrome

From the Cognitive Neuroscience Section (E.D.H., E.N.G., S.P., M.P., F.K., G.Z., M.C.T., E.M.W., J.G.) and Brain Stimulation Unit (E.M.W.), National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD; Litwin-Zucker Research Center for the Study of Alzheimer's Disease and Memory Disorders (E.D.H.), Feinstein Institute for Medical Research, Manhasset, NY; Departimento di Neuroscienze, Oftalmologia e Genetica (M.P.), Università di Genova; and Departimento di Neuroscienze (G.Z.), Università di Modena e Reggio Emilia, Modena, Italy.

Address correspondence and reprint requests to Dr. J. Grafman, Chief, Cognitive Neuroscience Section, NINDS, NIH, Bldg. 10, Room 7D43, MSC 1440, Bethesda, MD 20892-1440 grafmanj{at}ninds.nih.gov

Objective: To determine the pattern of executive dysfunction in frontotemporal dementia (FTD) and corticobasal syndrome (CBS) and to determine the brain areas associated with executive dysfunction in these illnesses.

Method: We administered the Delis-Kaplan Executive Function System (D-KEFS), a collection of standardized executive function tests, to 51 patients with behavioral-variant FTD and 50 patients with CBS. We also performed a discriminant analysis on the D-KEFS to determine which executive function tests best distinguished the clinical diagnoses of FTD and CBS. Finally, we used voxel-based morphometry (VBM) to determine regional gray matter volume loss associated with executive dysfunction.

Results: Patients with FTD and patients with CBS showed executive dysfunction greater than memory dysfunction. Executive function was better preserved in the patients with CBS than the patients with FTD with the exception of tests that required motor, visuospatial ability, or both. In patients with CBS, dorsal frontal and parietal and temporal-parietal cortex was associated with executive function. In FTD, tests with a language component (Verbal Fluency) were associated with left perisylvian cortex, sorting with the left dorsolateral prefrontal cortex, and reasoning (the Twenty Questions task) with the left anterior frontal cortex. The Twenty Questions test best distinguished the clinical diagnoses of CBS and FTD.

Conclusions: The neuroanatomic findings (especially in frontotemporal dementia [FTD]) agree with the previous literature on this topic. Patients with FTD and patients with corticobasal syndrome (CBS) show disparate performance on higher-order executive functions, especially the Twenty Questions test. It may be difficult to distinguish motor and visuospatial ability from executive function in patients with CBS using tests with significant motor and visuospatial demands such as Trail Making.

Abbreviations: bv-FTD = behavioral variant FTD; CBS = corticobasal syndrome; D-KEFS = Delis-Kaplan Executive Function System; FDR = false discovery rate; FTD = frontotemporal dementia; FWE = family wise error; MDRS2 = Mattis Dementia Rating Scale 2; NINDS = National Institute of Neurological Disorders and Stroke; ROI = region of interest; VBM = voxel-based morphometry; WMS-III = Wechsler Memory Scale–third edition.

Supplemental data at www.neurology.org

Supported by the intramural program of the NIH/National Institute of Neurological Disorders and Stroke.

Disclosure: The authors report no disclosures.

Received May 20, 2008. Accepted in final form October 23, 2008.

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