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Behavioral Variant Frontotemporal Dementia with Corticobasal Degeneration Pathology: Phenotypic Comparison to bvFTD with Pick’s Disease

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Abstract

Patients with corticobasal degeneration (CBD) pathology present with diverse clinical syndromes also associated with other neuropathologies, including corticobasal syndrome, progressive nonfluent aphasia, and an Alzheimer’s-type dementia. Some present with behavioral variant frontotemporal dementia (bvFTD), though this subtype still requires more detailed clinical characterization. All patients with CBD pathology and clinical assessment were reviewed (N = 17) and selected if they initially met criteria for bvFTD [bvFTD(CBD), N = 5]. Available bvFTD patients with Pick’s [bvFTD(Pick’s), N = 5] were selected as controls. Patients were also compared to healthy older controls [N = 53] on neuropsychological and neuroimaging measures. At initial presentation, bvFTD(CBD) showed few neuropsychological or motor differences from bvFTD(Pick’s). Neuropsychiatrically, they were predominantly apathetic with less florid social disinhibition and eating disturbances, and were more anxious than bvFTD(Pick’s) patients. Voxel-based morphometry revealed similar patterns of predominantly frontal atrophy between bvFTD groups, though overall degree of atrophy was less severe in bvFTD(CBD), who also showed comparative preservation of the frontoinsular rim, with dorsal > ventral frontal atrophy, and sparing of temporal and parietal structures relative to bvFTD(Pick’s) patients. Despite a remarkable overlap between the two patient types, bvFTD patients with underlying CBD pathology show subtle clinical features that may distinguish them from patients with Pick’s disease neuropathology.

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Acknowledgments

This study was supported by the following grants: NIA-PPG P01-AG1972403; GCRC-M01-RR00079; AG19724-01A1; ARCC 01-154-20; and T32AG23481, P50AG023501, State of California DHS 04–33516.

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  1. University of California San Francisco, San Francisco, CA, USA

    Katherine P. Rankin, Mary Catherine Mayo, William W. Seeley, Suzee Lee, Gil Rabinovici, Maria Luisa Gorno-Tempini, Adam L. Boxer, Michael W. Weiner, Stephen J. DeArmond & Bruce L. Miller

  2. San Francisco Veterans Administration Hospital, San Francisco, CA, USA

    Michael W. Weiner

  3. University of Pennsylvania, Philadelphia, PA, USA

    John Q. Trojanowski

  4. UCSF Department of Neurology, Memory and Aging Center, 350 Parnassus Avenue, Suite 905, San Francisco, CA, 94143-1207, USA

    Katherine P. Rankin

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  1. Katherine P. Rankin
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Correspondence to Katherine P. Rankin.

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Katherine P. Rankin and Mary Catherine Mayo are co-first authors.

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Rankin, K.P., Mayo, M.C., Seeley, W.W. et al. Behavioral Variant Frontotemporal Dementia with Corticobasal Degeneration Pathology: Phenotypic Comparison to bvFTD with Pick’s Disease. J Mol Neurosci 45, 594–608 (2011). https://doi.org/10.1007/s12031-011-9615-2

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