Multiple System Atrophy: Recent Developments and Future Perspectives
Mov Disord. 2019-11-06; :
DOI: 10.1002/mds.27894
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Meissner WG(1)(2)(3)(4), Fernagut PO(2)(3)(5)(6), Dehay B(2)(3), Péran P(7), Traon AP(8), Foubert-Samier A(1)(2)(9), Lopez Cuina M(2)(3), Bezard E(2)(3), Tison F(1)(2)(3), Rascol O(10).
Author information:
(1)CRMR Atrophie Multisystématisée, CHU Bordeaux, Service de Neurologie, Bordeaux, France.
(2)Institut des Maladies Neurodégénératives, Univ. de Bordeaux, Bordeaux, France.
(3)CNRS, Institut des Maladies Neurodégénératives, Bordeaux, France.
(4)Dept. of Medicine, University of Otago, Christchurch, New Zealand Brain Research Institute, Christchurch, New Zealand.
(5)Laboratoire de Neurosciences Expérimentales et Cliniques, Université de Poitiers, Poitiers, France.
(6)INSERM, Laboratoire de Neurosciences Expérimentales et Cliniques, Poitiers, France.
(7)ToNIC, Toulouse NeuroImaging Center, Université de Toulouse, Toulouse, France.
(8)Services de Neurologie, CRMR Atrophie Multisystématisée, Toulouse, Institut des Maladies Métaboliques et Cardiovasculaires, Toulouse, France.
(9)Inserm, Bordeaux Population Health Research Center, Bordeaux University, Bordeaux, France.
(10)Services de Neurologie et de Pharmacologie Clinique, Centre de Reference AMS, Centre d’Investigation Clinique, Réseau NS-Park/FCRIN et Centre of Excellence for Neurodegenerative Disorders (COEN) de Toulouse, CHU de Toulouse, Toulouse 3 University, Toulouse, France.
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.
© 2019 International Parkinson and Movement Disorder Society.