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Acta Neuropathologica

, Volume 137, Issue 1, pp 167–169 | Cite as

“Minimal change” multiple system atrophy with limbic-predominant α-synuclein pathology

  • Shunsuke Koga
  • Dennis W. DicksonEmail author
Correspondence

Multiple system atrophy (MSA) is a progressive neurodegenerative disease characterized by autonomic failure, parkinsonism, and cerebellar ataxia [3]. The striatonigral and olivopontocerebellar systems are most vulnerable regions in MSA. A variable degree of neuronal loss and astrogliosis are observed in addition to glial cytoplasmic inclusions (GCIs). “Minimal change” or “preclinical” MSA has been reported to as an early pathologic form of MSA [2, 4, 5, 6, 7]. These cases have minimal neuronal loss confined to the striatonigral and/or olivopontocerebellar systems [2, 5, 6, 7]. Some have no neuronal loss [4], but GCIs are observed throughout the brain, even in regions without neuronal loss. This finding suggests that the formation of GCI may precede neuronal loss, and both striatonigral and olivopontocerebellar systems are the brain regions affected in the earliest stage of the disease. We herein report a limbic counterpart of “minimal change” MSA with abundant α-synuclein pathology in...

Abbreviations

GCI

Glial cytoplasmic inclusion

MSA

Multiple system atrophy

NCI

Neuronal cytoplasmic inclusion

Notes

Acknowledgements

We would like to thank the patients and their families who donated brains. We also acknowledge Linda Rousseau, Virginia Phillips, Ariston Librero, and Monica Castanedes-Casey for their neuropathologic assistance. This work is supported by Tau Consortium (Rainwater Charitable Foundation), a Jaye F. and Betty F. Dyer Foundation Fellowship in progressive supranuclear palsy research, and CBD Solutions Research Grant.

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References

  1. 1.
    Aoki N, Boyer PJ, Lund C et al (2015) Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein. Acta Neuropathol 130:93–105.  https://doi.org/10.1007/s00401-015-1442-z CrossRefGoogle Scholar
  2. 2.
    Fujishiro H, Ahn TB, Frigerio R et al (2008) Glial cytoplasmic inclusions in neurologically normal elderly: prodromal multiple system atrophy? Acta Neuropathol 116:269–275.  https://doi.org/10.1007/s00401-008-0398-7 CrossRefGoogle Scholar
  3. 3.
    Gilman S, Wenning GK, Low PA et al (2008) Second consensus statement on the diagnosis of multiple system atrophy. Neurology 71:670–676.  https://doi.org/10.1212/01.wnl.0000324625.00404.15 CrossRefGoogle Scholar
  4. 4.
    Kon T, Mori F, Tanji K et al (2013) An autopsy case of preclinical multiple system atrophy (MSA-C). Neuropathology 33:667–672.  https://doi.org/10.1111/neup.12037 CrossRefGoogle Scholar
  5. 5.
    Ling H, Asi YT, Petrovic IN et al (2015) Minimal change multiple system atrophy: an aggressive variant? Mov Disord 30:960–967.  https://doi.org/10.1002/mds.26220 CrossRefGoogle Scholar
  6. 6.
    Wakabayashi K, Mori F, Nishie M et al (2005) An autopsy case of early (“minimal change”) olivopontocerebellar atrophy (multiple system atrophy-cerebellar). Acta Neuropathol 110:185–190.  https://doi.org/10.1007/s00401-005-1029-1 CrossRefGoogle Scholar
  7. 7.
    Wenning GK, Quinn N, Magalhaes M et al (1994) “Minimal change” multiple system atrophy. Mov Disord 9:161–166.  https://doi.org/10.1002/mds.870090206 CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of NeuroscienceMayo ClinicJacksonvilleUSA

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