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Chapter 4 - Neuropathological Overview of Neurodegenerative Disorders

from Section 1: - Basic Introduction

Published online by Cambridge University Press:  07 January 2025

By
Erik Ch. Wolters
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

Neurodegenerative disorders are complex multisystem disorders mainly characterized by aggregations of misfolded proteins (such as misfolded amyloid-beta protein in Alzheimer’s disease) in select regions in the central, peripheral, and autonomic nervous systems. In this chapter the various proteinopathic neurodegenerative movement disorders will be dealt with: synucleinopathies, tauopathies, frontotemporal lobar degenerations with TAU, TAR DNA binding protein-43 (TDP), and/or fused in sarcoma (FUS) proteinopathies, polyglutamine CAG-repeat disorders, and misfolded prion proteins. Abnormal protein deposits can be visualized post mortem with immunohistochemical methods that define the diseases, allow the staging schemes, and establish correlations between neuropathologic and clinical phenotypes. As neurodegenerative disorders often display comorbidity, immunohistochemistry with antibody panels has to be performed to enable assessment of the specific protein aggregations in various regions.

Keywords

Neurodegenerationneuropathologic hallmarkssynucleinopathiestauopathiespolyglutamine disordersCAG repeat diseasesprion diseases.
Type
Chapter
Information
International Compendium of Movement Disorders , pp. 53 - 73
Publisher: Cambridge University Press
Print publication year: 2025

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