Multiple System Atrophy: Recent Developments and Future Perspectives

Multiple system atrophy (MSA) is a rare and fatal neurodegenerative disorder characterized by a variable combination of parkinsonism, cerebellar impairment, and autonomic dysfunction. The pathologic hallmark is the accumulation of aggregated α‐synuclein in oligodendrocytes, forming glial cytoplasmic...

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Published in:Movement disorders 2019-11, Vol.34 (11), p.1629-1642
Main Authors: Meissner, Wassilios G., Fernagut, Pierre‐Olivier, Dehay, Benjamin, Péran, Patrice, Traon, Anne Pavy‐Le, Foubert‐Samier, Alexandra, Lopez Cuina, Miguel, Bezard, Erwan, Tison, François, Rascol, Olivier
Format: Article
Language:English
Subjects:
Atrophy
Autonomic nervous system
Basal ganglia
Central nervous system diseases
Cerebellum
Dementia disorders
diagnosis
Inclusion bodies
Lewy bodies
Life Sciences
Medical diagnosis
Medical prognosis
Movement disorders
MSA
Neurodegenerative diseases
Neuroprotection
Oligodendrocytes
Parkinson's disease
Pathogenesis
prognosis
Synuclein
treatment
α‐synuclein
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Summary:Multiple system atrophy (MSA) is a rare and fatal neurodegenerative disorder characterized by a variable combination of parkinsonism, cerebellar impairment, and autonomic dysfunction. The pathologic hallmark is the accumulation of aggregated α‐synuclein in oligodendrocytes, forming glial cytoplasmic inclusions, which qualifies MSA as a synucleinopathy together with Parkinson's disease and dementia with Lewy bodies. The underlying pathogenesis is still not well understood. Some symptomatic treatments are available, whereas neuroprotection remains an urgent unmet treatment need. In this review, we critically appraise significant developments of the past decade with emphasis on pathogenesis, diagnosis, prognosis, and treatment development. We further discuss unsolved questions and highlight some perspectives. © 2019 International Parkinson and Movement Disorder Society
ISSN:0885-3185
1531-8257
DOI:10.1002/mds.27894