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Chapter 30 - Multiple System Atrophy

from Section 2: - Hypokinetic Movement Disorders

Published online by Cambridge University Press:  07 January 2025

By
Evdokia Efthymiou
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

Multiple system atrophy (MSA) is an adult-onset, rapidly progressive, rare, neurodegenerative disorder characterized by an abnormal accumulation of α-synuclein (αSYN) in oligodendrocytes associated with multifocal neuronal degeneration. Further synucleionopathies include Parkinson disease (PD) and dementia with Lewy bodies (DLB). Clinically, the disease is characterized by poorly L-dopa-responsive parkinsonism and/or cerebellar ataxia accompanied by autonomic failure. This chapter provides an update of clinical symptomatology, diagnosis and, especially, disease-modifying and symptomatic treatment.

Keywords

atypical parkinsonian disorderParkinson-plus syndromeneurodegenerative disordera-synucleinopathyoligodendrogliopathy
Type
Chapter
Information
International Compendium of Movement Disorders , pp. 368 - 379
Publisher: Cambridge University Press
Print publication year: 2025

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