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Multiple System Atrophy with Cerebellar Ataxia

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Multiple system atrophy with predominant cerebellar ataxia (MSA-C) is a sporadic disorder which usually presents as a midline cerebellar disorder which progresses more rapidly than other late-onset sporadic ataxias. The patient typically becomes wheelchair-dependent within 5 years of onset. Diagnostic criteria for probable MSA-C include a sporadic, progressive adult-onset cerebellar syndrome together with additional features of parkinsonism, Babinski signs, and laryngeal stridor. Interestingly, cerebellar ataxia often improves as signs of parkinsonism emerge later in the disease. A recent consensus statement emphasizes neuroimaging features including atrophy of the putamen, middle cerebellar peduncle, or pons; FDG-PET hypometabolism in the putamen; and presynaptic nigrostriatal dopaminergic denervation with SPECT or PET imaging.

Keywords

Multiple System Atrophy Cerebellar Ataxia Spinocerebellar Ataxia Middle Cerebellar Peduncle Babinski Sign 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Clip 1: the patient shows evidence of mild facial masking and parkinsonism, but the cerebellar features are more prominent including gait ataxia and inability to perform tandem gait. Clip 2: at follow-up examination 18 months later, facial masking has become more prominent, and the upper extremities and gait have become more ataxic. She now requires assistance to walk for a few steps and she has become wheelchair-dependent.

MSA-C.mp4 (MP4 31,298KB)

References

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    Silber MH, Levine S. Stridor and death in multiple system atrophy. Mov Disord. 2000;15:699–704.PubMedCrossRefGoogle Scholar
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    Bhidayasiri R, Ling H. Multiple system atrophy. Neurologist. 2008;14:224–37.PubMedCrossRefGoogle Scholar
  3. 3.
    Gilman S, Wenning GK, Low PA, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008;71:670–6.PubMedCentralPubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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