Clinicopathological correlations in corticobasal degeneration

Suzee E. Lee, Gil D. Rabinovici, Mary Catherine Mayo, Stephen M Wilson, William W. Seeley, Stephen J. Dearmond, Eric J. Huang, John Q. Trojanowski, Matthew E. Growdon, Jung Y. Jang, Manu Sidhu, Tricia M. See, Anna M. Karydas, Maria Luisa Gorno-Tempini, Adam L. Boxer, Michael W. Weiner, Michael D. Geschwind, Katherine P. Rankin, Bruce L. Miller

Research output: Contribution to journalArticle

225 Citations (Scopus)

Abstract

Objective: To characterize cognitive and behavioral features, physical findings, and brain atrophy patterns in pathology-proven corticobasal degeneration (CBD) and corticobasal syndrome (CBS) with known histopathology. Methods: We reviewed clinical and magnetic resonance imaging data in all patients evaluated at our center with either an autopsy diagnosis of CBD (n = 18) or clinical CBS at first presentation with known histopathology (n = 40). Atrophy patterns were compared using voxel-based morphometry. Results: CBD was associated with 4 clinical syndromes: progressive nonfluent aphasia (n = 5), behavioral variant frontotemporal dementia (n = 5), executive-motor (n = 7), and posterior cortical atrophy (n = 1). Behavioral or cognitive problems were the initial symptoms in 15 of 18 patients; less than half exhibited early motor findings. Compared to controls, CBD patients showed atrophy in dorsal prefrontal and perirolandic cortex, striatum, and brainstem (p < 0.001 uncorrected). The most common pathologic substrates for clinical CBS were CBD (35%), Alzheimer disease (AD, 23%), progressive supranuclear palsy (13%), and frontotemporal lobar degeneration (FTLD) with TDP inclusions (13%). CBS was associated with perirolandic atrophy irrespective of underlying pathology. In CBS due to FTLD (tau or TDP), atrophy extended into prefrontal cortex, striatum, and brainstem, whereas in CBS due to AD, atrophy extended into temporoparietal cortex and precuneus (p < 0.001 uncorrected). Interpretation: Frontal lobe involvement is characteristic of CBD, and in many patients frontal, not parietal or basal ganglia, symptoms dominate early stage disease. CBS is driven by medial perirolandic dysfunction, but this anatomy is not specific to a single underlying histopathology. Antemortem prediction of CBD will remain challenging until clinical features of CBD are redefined, and sensitive, specific biomarkers are identified.

Original languageEnglish (US)
Pages (from-to)327-340
Number of pages14
JournalAnnals of Neurology
Volume70
Issue number2
DOIs
StatePublished - Aug 2011

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Atrophy
Frontotemporal Lobar Degeneration
Prefrontal Cortex
Brain Stem
Primary Progressive Nonfluent Aphasia
Pathology
Progressive Supranuclear Palsy
Frontotemporal Dementia
Parietal Lobe
Frontal Lobe
Basal Ganglia
Autopsy
Anatomy
Alzheimer Disease
Biomarkers
Magnetic Resonance Imaging
Brain

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Lee, S. E., Rabinovici, G. D., Mayo, M. C., Wilson, S. M., Seeley, W. W., Dearmond, S. J., ... Miller, B. L. (2011). Clinicopathological correlations in corticobasal degeneration. Annals of Neurology, 70(2), 327-340. https://doi.org/10.1002/ana.22424

Clinicopathological correlations in corticobasal degeneration. / Lee, Suzee E.; Rabinovici, Gil D.; Mayo, Mary Catherine; Wilson, Stephen M; Seeley, William W.; Dearmond, Stephen J.; Huang, Eric J.; Trojanowski, John Q.; Growdon, Matthew E.; Jang, Jung Y.; Sidhu, Manu; See, Tricia M.; Karydas, Anna M.; Gorno-Tempini, Maria Luisa; Boxer, Adam L.; Weiner, Michael W.; Geschwind, Michael D.; Rankin, Katherine P.; Miller, Bruce L.

In: Annals of Neurology, Vol. 70, No. 2, 08.2011, p. 327-340.

Research output: Contribution to journalArticle

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 degeneration', Annals of Neurology, vol. 70, no. 2, pp. 327-340. https://doi.org/10.1002/ana.22424
Lee SE, Rabinovici GD, Mayo MC, Wilson SM, Seeley WW, Dearmond SJ et al. Clinicopathological correlations in corticobasal degeneration. Annals of Neurology. 2011 Aug;70(2):327-340. https://doi.org/10.1002/ana.22424
Lee, Suzee E. ; Rabinovici, Gil D. ; Mayo, Mary Catherine ; Wilson, Stephen M ; Seeley, William W. ; Dearmond, Stephen J. ; Huang, Eric J. ; Trojanowski, John Q. ; Growdon, Matthew E. ; Jang, Jung Y. ; Sidhu, Manu ; See, Tricia M. ; Karydas, Anna M. ; Gorno-Tempini, Maria Luisa ; Boxer, Adam L. ; Weiner, Michael W. ; Geschwind, Michael D. ; Rankin, Katherine P. ; Miller, Bruce L. / Clinicopathological correlations in corticobasal degeneration. In: Annals of Neurology. 2011 ; Vol. 70, No. 2. pp. 327-340.
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abstract = "Objective: To characterize cognitive and behavioral features, physical findings, and brain atrophy patterns in pathology-proven corticobasal degeneration (CBD) and corticobasal syndrome (CBS) with known histopathology. Methods: We reviewed clinical and magnetic resonance imaging data in all patients evaluated at our center with either an autopsy diagnosis of CBD (n = 18) or clinical CBS at first presentation with known histopathology (n = 40). Atrophy patterns were compared using voxel-based morphometry. Results: CBD was associated with 4 clinical syndromes: progressive nonfluent aphasia (n = 5), behavioral variant frontotemporal dementia (n = 5), executive-motor (n = 7), and posterior cortical atrophy (n = 1). Behavioral or cognitive problems were the initial symptoms in 15 of 18 patients; less than half exhibited early motor findings. Compared to controls, CBD patients showed atrophy in dorsal prefrontal and perirolandic cortex, striatum, and brainstem (p < 0.001 uncorrected). The most common pathologic substrates for clinical CBS were CBD (35{\%}), Alzheimer disease (AD, 23{\%}), progressive supranuclear palsy (13{\%}), and frontotemporal lobar degeneration (FTLD) with TDP inclusions (13{\%}). CBS was associated with perirolandic atrophy irrespective of underlying pathology. In CBS due to FTLD (tau or TDP), atrophy extended into prefrontal cortex, striatum, and brainstem, whereas in CBS due to AD, atrophy extended into temporoparietal cortex and precuneus (p < 0.001 uncorrected). Interpretation: Frontal lobe involvement is characteristic of CBD, and in many patients frontal, not parietal or basal ganglia, symptoms dominate early stage disease. CBS is driven by medial perirolandic dysfunction, but this anatomy is not specific to a single underlying histopathology. Antemortem prediction of CBD will remain challenging until clinical features of CBD are redefined, and sensitive, specific biomarkers are identified.",
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AU - Dearmond, Stephen J.

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