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Corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, argyrophilic grain disease, and rarer neurodegenerative diseases 

Corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, argyrophilic grain disease, and rarer neurodegenerative diseases
Chapter:
Corticobasal degeneration, progressive supranuclear palsy, multiple system atrophy, argyrophilic grain disease, and rarer neurodegenerative diseases
Author(s):

Elizabeth A. Coon

and Keith A. Josephs

DOI:
10.1093/med/9780199655946.003.0037
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date: 28 May 2020

Neurodegenerative disorders include a variety of cognitive and motor syndromes with varying clinical presentations and pathologic findings. Tauopathies are a distinct subset due to abnormal deposition of the protein tau and include corticobasal degeneration and progressive supranuclear palsy which will be discussed in this chapter, as well as some forms of frontotemporal lobar degeneration. Abnormal accumulation of the protein alpha synuclein leads to another spectrum of parkinsonian disorders including multiple system atrophy which has prominent autonomic dysfunction. Clinical syndromes along with neuropsychology, imaging findings, neuropathologic features, and management options will be discussed for each disorder.

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