Chapter 7
Corticobasal degeneration and progressive supranuclear palsy
Suzee E. Lee,
Bruce L. Miller,
Suzee E. Lee
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorBruce L. Miller
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorSuzee E. Lee,
Bruce L. Miller,
Suzee E. Lee
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorBruce L. Miller
University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorBook Editor(s):Michael D. Geschwind MD PhD,
Caroline Racine Belkoura PhD,
Michael D. Geschwind MD PhD
Memory and Aging Center, Department of Neurology, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorCaroline Racine Belkoura PhD
Department of Neurological Surgery, University of California, San Francisco, San Francisco, CA, USA
Search for more papers by this authorFirst published: 22 January 2016
Summary
This chapter reviews corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), with a strong emphasis on clinicopathological correlations. First, clinically defined corticobasal syndrome (CBS) and PSP syndrome are discussed in light of clinicopathological series, which reveal the extent to which clinical features can predict pathology. Pathologically defined CBD manifests as three main clinical syndromes, including an executive–motor syndrome, nonfluent variant primary progressive aphasia (nfvPPA), and behavioral variant frontotemporal dementia (bvFTD); detailed case studies outline these clinical syndromes most commonly associated with CBD. Pathologically defined PSP usually presents as PSP syndrome during life, although PSP may present clinically as Parkinson's disease (PD), CBS, and multiple system atrophy (MSA). This chapter also reviews CBD and PSP pathological features and explores genetic associations. We discuss biomarkers and neuroimaging for the diagnosis of CBS and PSP syndrome, which will prove crucial for evaluating emerging therapies.
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