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It is neuropathologically defined by widespread and abundant central nervous system \u03b1\u2010synuclein\u2013positive glial cytoplasmic inclusions and striatonigral and\/or olivopontocerebellar neurodegeneration. There are two clinical subtypes of MSA distinguished by the predominant motor features: the parkinsonian variant (MSA\u2010P) and the cerebellar variant (MSA\u2010C). Despite recent progress in understanding the pathobiology of MSA, investigations into the symptomatology and natural history of the cerebellar variant of the disease have been limited. MSA\u2010C presents a unique challenge to both clinicians and researchers alike. A key question is how to distinguish early in the disease course between MSA\u2010C and other causes of adult\u2010onset cerebellar ataxia. This is a particularly difficult question, because the clinical framework for conceptualizing and studying sporadic adult\u2010onset ataxias continues to undergo flux. To date, several investigations have attempted to identify clinical features, imaging, and other biomarkers that may be predictive of MSA\u2010C. This review presents a clinically oriented overview of our current understanding of MSA\u2010C with a focus on evidence for distinguishing MSA\u2010C from other sporadic, adult\u2010onset ataxias. \u00a9 2014 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.25847","type":"journal-article","created":{"date-parts":[[2014,2,24]],"date-time":"2014-02-24T13:51:52Z","timestamp":1393249912000},"page":"294-304","source":"Crossref","is-referenced-by-count":62,"title":["Multiple system atrophy of the cerebellar type: Clinical state of the art"],"prefix":"10.1002","volume":"29","author":[{"given":"David J.","family":"Lin","sequence":"first","affiliation":[{"name":"Ataxia Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine L.","family":"Hermann","sequence":"additional","affiliation":[{"name":"Ataxia Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy D.","family":"Schmahmann","sequence":"additional","affiliation":[{"name":"Ataxia Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology Massachusetts General Hospital Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2014,2,24]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000324625.00404.15"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.52.Suppl.78"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-009-5173-8"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.49.5.1284"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(99)04137-9"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(00)82050-4"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.33.321"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21839"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(89)90219-0"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(98)00407-8"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410440324"},{"key":"e_1_2_4_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0304-3940(98)00504-7"},{"key":"e_1_2_4_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00702-005-0328-y"},{"key":"e_1_2_4_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.21734"},{"key":"e_1_2_4_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00702-005-0381-6"},{"key":"e_1_2_4_17_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009258"},{"key":"e_1_2_4_18_1","first-page":"330","article-title":"Thomas A. 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