Corticobasal degeneration

Gerard M. Saranza, Jennifer L. Whitwell, Gabor G. Kovacs, Anthony E. Lang

Research output: Chapter in Book/Report/Conference proceedingChapter

2 Scopus citations

Abstract

Corticobasal degeneration (CBD) is a rare neurodegenerative disease characterized by the predominance of pathological 4 repeat tau deposition in various cell types and anatomical regions. Corticobasal syndrome (CBS) is one of the clinical phenotypes associated with CBD pathology, manifesting as a progressive asymmetric akinetic-rigid, poorly levodopa-responsive parkinsonism, with cerebral cortical dysfunction. CBD can manifest as several clinical phenotypes, and similarly, CBS can also have a pathologic diagnosis other than CBD. This chapter discusses the clinical manifestations of pathologically confirmed CBD cases, the current diagnostic criteria, as well as the pathologic and neuroimaging findings of CBD/CBS. At present, therapeutic options for CBD remain symptomatic. Further research is needed to improve the clinical diagnosis of CBD, as well as studies on disease-modifying therapies for this relentlessly progressive neurodegenerative disorder.

Original languageEnglish (US)
Title of host publicationInternational Review of Neurobiology
EditorsMaria Stamelou, Günter U. Höglinger
PublisherAcademic Press Inc
Pages87-136
Number of pages50
ISBN (Print)9780128177303
DOIs
StatePublished - 2019

Publication series

NameInternational Review of Neurobiology
Volume149
ISSN (Print)0074-7742
ISSN (Electronic)2162-5514

Keywords

  • 4R-tauopathy
  • Atypical parkinsonism
  • Corticobasal degeneration
  • Corticobasal syndrome
  • Tauopathy

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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  • Cite this

    Saranza, G. M., Whitwell, J. L., Kovacs, G. G., & Lang, A. E. (2019). Corticobasal degeneration. In M. Stamelou, & G. U. Höglinger (Eds.), International Review of Neurobiology (pp. 87-136). (International Review of Neurobiology; Vol. 149). Academic Press Inc. https://doi.org/10.1016/bs.irn.2019.10.014