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Corticobasal degeneration: a pathologically distinct 4R tauopathy

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Kouri, Naomi, Whitwell, Jennifer L., Josephs, Keith A., Rademakers, Rosa, Dickson, Dennis W.·2011·Nature Reviews Neurology
DOI 10.1038/nrneurol.2011.43PMC PMC10006729
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Abstract

Corticobasal degeneration (CBD) is a rare, progressive neurodegenerative disorder with onset in the 5(th) to 7(th) decade of life. It is associated with heterogeneous motor, sensory, behavioral and cognitive symptoms, which make its diagnosis difficult in a living patient. The etiology of CBD is unknown; however, neuropathological and genetic evidence supports a pathogenetic role for microtubule-associated protein tau. CBD pathology is characterized by circumscribed cortical atrophy with spongiosis and ballooned neurons; the distribution of these changes dictates the patient's clinical presentation. Neuronal and glial tau pathology is extensive in gray and white matter of the cortex, basal ganglia, diencephalon and rostral brainstem. Abnormal tau accumulation within astrocytes forms pathognomonic astrocytic plaques. The classic clinical presentation, termed corticobasal syndrome (CBS), comprises asymmetric progressive rigidity and apraxia with limb dystonia and myoclonus. CBS also occurs in conjunction with other diseases, including Alzheimer disease and progressive supranuclear palsy. Moreover, the pathology of CBD is associated with clinical presentations other than CBS, including Richardson syndrome, behavioral variant frontotemporal dementia, primary progressive aphasia and posterior cortical syndrome. Progress in biomarker development to differentiate CBD from other disorders has been slow, but is essential in improving diagnosis and in development of disease-modifying therapies.

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  • Fewer than 50% of patients with corticobasal syndrome have CBD at autopsy.

    “This phenotypic variability results in <50% of patients with CBS having CBD at autopsy567.”

    From Genome-wide association study of corticobasal degeneration identifies risk variants shared with progressive supranuclear palsy (2015) · cites this paper as [5]

    The Introduction says autopsy studies show considerable variation in the pathology of patients with CBS, supporting the point that CBS does not always indicate CBD. The Corticobasal syndrome section gives approximately 50% for the reverse group—autopsy-confirmed CBD cases presenting with CBS—not the percentage of CBS patients who have CBD at autopsy. Our copy therefore does not establish the claimed <50% figure. (LLM, conf=0.86)

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Cited by

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Authors
Kouri, N., Whitwell, J. L., Josephs, K. A., Rademakers, R., & Dickson, D. W.
Affiliations
Mayo Clinic (US)
Corresponding author
Dickson, Dennis W.
ORCID
Venue
Nature Reviews Neurology
Publisher
Springer Science and Business Media LLC
Published
2011-04-12
Volume
7
Issue
5
Pages
263-272
ISSN
  • 1759-4758 (ISSN-L)
  • 1759-4766
DOI
10.1038/nrneurol.2011.43
PMC
PMC10006729
OpenAlex
W2071751509
Cited by
246
References
8
Citations by year
2026: 17 · 2025: 12 · 2024: 9 · 2023: 16 · 2022: 13 · 2021: 27 · 2020: 13 · 2019: 16 · 2018: 10 · 2017: 24 · 2016: 14 · 2015: 22 · 2014: 18 · 2013: 19 · 2012: 10
FWCI
1.84
Type
journal article
Language
en
Open access
Licence
Locations
  • Nature Reviews Neurology (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
Funders
  • Mayo Foundation for Medical Education and Research (AG17216, AG16574)
  • CurePSP
  • National Institutes of Health (R21-AG38736, AG16574, AG17216)
  • National Institute on Aging (R21-AG38736, P50‐AG16574, P01-AG-17216, R21AG038736, R01‐AG37491, R01AG037491, P01AG017216, L30 AG051249, P50AG016574)
  • National Institute on Deafness and Other Communication Disorders (R01-DC10367, R01DC010367)
  • National Institute of Neurological Disorders and Stroke (P50-NS72187, P50NS072187)
Topics
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
Keywords
corticobasal degeneration, corticobasal syndrome, tauopathy, 4R tau, tau protein, cortical atrophy, spongiosis, ballooned neurons, Richardson syndrome, behavioral variant frontotemporal dementia, primary progressive aphasia, neuroimaging biomarkers, genetic susceptibility, disease-modifying therapy, differential diagnosis
MeSH terms
Humans, Tauopathies

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