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Curr Neurol Neurosci Rep. 2017 May;17(5):41. doi: 10.1007/s11910-017-0751-0.

Multiple System Atrophy - State of the Art.

Author information

1
Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, 33000, Bordeaux, France.
2
Institut des Maladies Neurodégénératives, Univ. de Bordeaux, UMR 5293, 33000, Bordeaux, France.
3
CNRS, Institut des Maladies Neurodégénératives, UMR 5293, 33000, Bordeaux, France.
4
Centre de Référence Maladie Rare AMS, Hôpital Pellegrin, CHU de Bordeaux, F-33076, Bordeaux, France.
5
Service de Neurologie, Hôpital Pellegrin, CHU de Bordeaux, 33000, Bordeaux, France. wassilios.meissner@chu-bordeaux.fr.
6
Institut des Maladies Neurodégénératives, Univ. de Bordeaux, UMR 5293, 33000, Bordeaux, France. wassilios.meissner@chu-bordeaux.fr.
7
CNRS, Institut des Maladies Neurodégénératives, UMR 5293, 33000, Bordeaux, France. wassilios.meissner@chu-bordeaux.fr.
8
Centre de Référence Maladie Rare AMS, Hôpital Pellegrin, CHU de Bordeaux, F-33076, Bordeaux, France. wassilios.meissner@chu-bordeaux.fr.

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.

KEYWORDS:

Alpha-synuclein; Atypical parkinsonism; MSA; Oligodendrocyte

PMID:
28378233
DOI:
10.1007/s11910-017-0751-0
[Indexed for MEDLINE]

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