Behavioral Variant Frontotemporal Dementia with Corticobasal Degeneration Pathology: Phenotypic Comparison to bvFTD with Pick’s Disease
- 367 Downloads
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.
KeywordsCorticobasal degeneration Frontotemporal dementia Behavior Neuropsychiatry Neuropsychology Neuropathology
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.
- Cairns NJ, Bigio EH, Mackenzie IR, Neumann M, Lee VM, Hatanpaa KJ, Consortium For Frontotemporal Lobar Degeneration (2007) Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the consortium for frontotemporal lobar degeneration. Acta Neuropathol 114(1):5–22. doi: 10.1007/S00401-007-0237-2 PubMedCrossRefGoogle Scholar
- Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, Dearmond SJ, Huang EJ, Trojanowski JQ, Growdon ME, Jang JY, Sidhu M, See TM, Karydas AM, Gorno-Tempini ML, Boxer AL, Weiner MW, Geschwind MD, Rankin KP, Miller BL. (2011) Clinicopathological correlations in corticobasal syndrome. Ann Neurol. 70(2):327–40Google Scholar
- Ludolph AC, Kassubek J, Landwehrmeyer BG, Mandelkow E, Mandelkow EM, Burn DJ, Reisensburg Working Group For Tauopathies with Parkinsonism (2009) Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options. Eur J Neurol 16(3):297–309. doi: 10.1111/J.1468-1331.2008.02513.X PubMedCrossRefGoogle Scholar
- Reibiz JJ, Kolodny EH, Richardson EP (1968) Corticodentoniagral degeneration with neuronal achromasia. Archives of Neurology 18:20–33Google Scholar
- Rousseaux M, Godefroy O, Cabaret M, Bernati T (1996) Dysexecutive syndrome and disorders of motor control in prefrontal mediobasal and cingulate lesions. Rev Neurol (Paris) 152(8–9):517–527Google Scholar
- Seeley WW, Menon V, Schatzberg AF, Keller J, Glover GH, Kenna H, Reiss AL, Greicius MD (2007) Dissociable intrinsic connectivity networks for salience processing and executive control. J Neurosci 27(9):2349–2356Google Scholar