Multiple system atrophy
Nat Rev Dis Primers. 2022-08-25; 8(1):
DOI: 10.1038/s41572-022-00382-6
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Poewe W(1), Stankovic I(2), Halliday G(3), Meissner WG(4)(5)(6), Wenning GK(7), Pellecchia MT(8), Seppi K(7), Palma JA(9), Kaufmann H(9).
Author information:
(1)Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria. .
(2)Neurology Clinic, Clinical Center of Serbia, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
(3)Brain and Mind Centre, Faculty of Medicine and Health School of Medical Sciences, University of Sydney, Sydney, New South Wales, Australia.
(4)CHU Bordeaux, Service de Neurologie – Maladies Neurodégénératives, CRMR AMS, IMNc, Bordeaux, France.
(5)CNRS, IMN, UMR 5293, University of Bordeaux, Bordeaux, France.
(6)New Zealand Brain Research Institute, Department of Medicine, University of Otago, Christchurch, New Zealand.
(7)Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
(8)Center for Neurodegenerative Diseases, Department of Medicine, Surgery and Dentistry « Scuola Medica Salernitana », University of Salerno, Salerno, Italy.
(9)Department of Neurology, New York University Grossman School of Medicine, New York, NY, USA.
Multiple system atrophy (MSA) is a rare neurodegenerative disease that is characterized by neuronal loss and gliosis in multiple areas of the central nervous system including striatonigral, olivopontocerebellar and central autonomic structures. Oligodendroglial cytoplasmic inclusions containing misfolded and aggregated α-synuclein are the histopathological hallmark of MSA. A firm clinical diagnosis requires the presence of autonomic dysfunction in combination with parkinsonism that responds poorly to levodopa and/or cerebellar ataxia. Clinical diagnostic accuracy is suboptimal in early disease because of phenotypic overlaps with Parkinson disease or other types of degenerative parkinsonism as well as with other cerebellar disorders. The symptomatic management of MSA requires a complex multimodal approach to compensate for autonomic failure, alleviate parkinsonism and cerebellar ataxia and associated disabilities. None of the available treatments significantly slows the aggressive course of MSA. Despite several failed trials in the past, a robust pipeline of putative disease-modifying agents, along with progress towards early diagnosis and the development of sensitive diagnostic and progression biomarkers for MSA, offer new hope for patients.
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