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Multiple System Atrophy: Recent Developments and Future Perspectives

Wassilios Meissner 1, 2, 3, * Pierre‐olivier Fernagut 2, 4 Benjamin Dehay 2 Patrice Péran 5 Anne Pavy‐le Traon 6 Alexandra Foubert‐samier 2, 1, 7 Miguel Lopez Cuina 2 Erwan Bézard 2 François Tison 8, 2 Olivier Rascol 9, 10, 11
* Corresponding author
1 Service de Neurologie - Centre de référence des maladies rares - Atrophie Multisystématisée [CHU Bordeaux]
2 IMN - Institut des Maladies Neurodégénératives [Bordeaux]
3 Department of Medicine [Christchurch, New Zealand]
4 LNEC - Laboratoire de neurosciences expérimentales et cliniques
5 ToNIC - Toulouse Neuro Imaging Center
6 Services de Neurologie - Centre de Référence des Maladies Rares - Atrophie Multisystématisée [Toulouse]
7 BPH - Bordeaux population health
8 Service de neurologie [Bordeaux]
9 CIC CHU (toulouse)/inserm
10 AMS - Centre de référence de l'atrophie multisystématisée [Toulouse]
11 NeuroToul - Centre d’Excellence en Maladies Neurodégénératives
Abstract : 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.
Keywords : MSA diagnosis prognosis treatment α-synuclein
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https://www.hal.inserm.fr/inserm-02439518
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Wassilios Meissner, Pierre‐olivier Fernagut, Benjamin Dehay, Patrice Péran, Anne Pavy‐le Traon, et al.. Multiple System Atrophy: Recent Developments and Future Perspectives. Movement Disorders, Wiley, 2019, 34 (11), pp.1629-1642. ⟨10.1002/mds.27894⟩. ⟨inserm-02439518⟩

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