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Multiple System Atrophy (MSA)

  • Reference work entry
Handbook of the Cerebellum and Cerebellar Disorders

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Abstract

Multiple system atrophy (MSA) is a sporadic, rapidly progressive neurodegenerative disorder characterized clinically by a combination of cerebellar dysfunction or parkinsonism and autonomic failure. The histopathological hallmark of MSA is the presence of glial cytoplasmic inclusions composed predominantly of α-synuclein within multiple systems of the brain and the spinal cord accompanied by gliosis and neuronal loss. In the past few years there has been substantial progress in the understanding of the pathogenesis of this fatal neurodegenerative disease. The most recent studies indicate that this is a primary oligodendroglial disorder with synucleinopathy leading to subsequent neurodegeneration.

The authors review the epidemiology, clinical features, diagnostic criteria, neuropathology, pathogenesis, management and therapeutic approaches to this disease.

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Author information

Authors and Affiliations

  1. Division of Clinical Neurobiology, Department of Neurology, Medical University, Anichstrasse 35, A-6020, Innsbruck, Austria

    Gregor K. Wenning MD., Ph.D., MSc & Dr. Florian Krismer MD

  2. Department of Neurology, University of Michigan, 300 N. Ingalls 3D15, 48109-0489, Ann Arbor, MI, USA

    Dr. Sid Gilman MD., FRCP

Authors
  1. Gregor K. Wenning MD., Ph.D., MSc
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  2. Dr. Florian Krismer MD
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  3. Dr. Sid Gilman MD., FRCP
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Corresponding author

Correspondence to Gregor K. Wenning MD., Ph.D., MSc .

Editor information

Editors and Affiliations

  1. Unité d'Etude du Mouvement (UEM), FNRS, Neurologie ULB Erasme, Bruxelles, Belgium

    Mario Manto

  2. Ataxia Unit, Cognitive and Behavioral Neurology Unit, Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA

    Jeremy D. Schmahmann

  3. Neuroscience Institute of the Cavalieri-Ottolenghi Foundation (NICO), University of Turin, Orbassano, Turin, Italy

    Ferdinando Rossi

  4. Molecular and Integrative Neuroscience Department (MIND), The Scripps Research Institute, California, CA, USA

    Donna L. Gruol

  5. Department of Integrative Physiolgy, Gunma University Graduate School of Medicine, Maebashi, Gunma, Japan

    Noriyuki Koibuchi

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© 2013 Springer Science+Business Media Dordrecht

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Wenning, G.K., Krismer, F., Gilman, S. (2013). Multiple System Atrophy (MSA). In: Manto, M., Schmahmann, J.D., Rossi, F., Gruol, D.L., Koibuchi, N. (eds) Handbook of the Cerebellum and Cerebellar Disorders. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-1333-8_97

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