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Erschienen in: Current Neurology and Neuroscience Reports 5/2017

01.05.2017 | Movement Disorders (S Fox, Section Editor)

Multiple System Atrophy - State of the Art

verfasst von: Brice Laurens, Sylvain Vergnet, Miguel Cuina Lopez, Alexandra Foubert-Samier, François Tison, Pierre-Olivier Fernagut, Wassilios G. Meissner

Erschienen in: Current Neurology and Neuroscience Reports | Ausgabe 5/2017

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Abstract

Multiple system atrophy (MSA) is a rare and fatal neurodegenerative disorder that is characterized by a variable combination of parkinsonism, cerebellar impairment, and autonomic dysfunction. Some symptomatic treatments are available while neuroprotection or disease-modification remain unmet treatment needs. The pathologic hallmark is the accumulation of aggregated alpha-synuclein (α-syn) in oligodendrocytes forming glial cytoplasmic inclusions, which qualifies MSA as synucleinopathy together with Parkinson’s disease and dementia with Lewy bodies. Despite progress in our understanding of the pathogenesis of MSA, the origin of α-syn aggregates in oligodendrocytes is still a matter of an ongoing debate. We critically review here studies published in the field over the past 5 years dealing with pathogenesis, genetics, clinical signs, biomarker for improving diagnostic accuracy, and treatment development.
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Metadaten
Titel
Multiple System Atrophy - State of the Art
verfasst von
Brice Laurens
Sylvain Vergnet
Miguel Cuina Lopez
Alexandra Foubert-Samier
François Tison
Pierre-Olivier Fernagut
Wassilios G. Meissner
Publikationsdatum
01.05.2017
Verlag
Springer US
Erschienen in
Current Neurology and Neuroscience Reports / Ausgabe 5/2017
Print ISSN: 1528-4042
Elektronische ISSN: 1534-6293
DOI
https://doi.org/10.1007/s11910-017-0751-0

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