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Chapter 32 - Corticobasal Degeneration and Corticobasal Syndrome

from Section 2: - Hypokinetic Movement Disorders

Published online by Cambridge University Press:  07 January 2025

By
Gerard Saranza ,
Melissa J. Armstrong  and
Anthony E. Lang
Edited by
Erik Ch. Wolters  and
Christian R. Baumann
Erik Ch. Wolters
Affiliation:
Universität Zürich
Christian R. Baumann
Affiliation:
Universität Zürich
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Summary

Corticobasal degeneration (CBD) is a neurodegenerative disease characterized by abnormal aggregation of hyperphosphorylated 4R-tau in cortical and subcortical areas of the brain. It is associated with various clinical phenotypes, such as the characteristic clinical phenotype corticobasal syndrome (CBS), which manifests with asymmetric akinetic–rigid, poorly levodopa-responsive parkinsonism, and cerebral cortical dysfunction. Other associated phenotypes are progressive supranuclear palsy (PSP) syndrome, frontotemporal dementia, Alzheimer’s disease (AD)-like dementia, and non-fluent/agrammatic variant of primary progressive aphasia. Precise use of terminology is critical for a common understanding in discussions of clinical phenotype, attempted clinical diagnosis of CBD with its many presenting phenotypes, and accurate pathologic diagnosis (which can only be made neuropathologically). Diagnosis of probable or possible CBS and the other CBD-associated syndromes is based on the presence of certain clinical features. Pathologic and neuroimaging findings and currently available biological markers are discussed. Treatment for CBD and CBS is symptomatic and supportive at present.

Keywords

atypical parkinsonismcorticobasal degenerationCBDcorticobasal syndromeCBStauopathy4R-tauopathy
Type
Chapter
Information
International Compendium of Movement Disorders , pp. 390 - 402
Publisher: Cambridge University Press
Print publication year: 2025

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