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Minimal change multiple system atrophy: an aggressive variant?

Helen Ling1,2, Yasmine T Asi1, Igor N Petrovic2,3

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|April 10, 2015
PubMed
Summary
This summary is machine-generated.

Minimal change multiple system atrophy (MC-MSA) shows more neuronal inclusions but similar glial pathology compared to typical MSA. Some MC-MSA cases exhibit aggressive disease progression and early symptoms before significant neuronal loss.

Keywords:
Minimal changealpha-synucleinmultiple system atrophysudden unexpected death

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Movement Disorders

Background:

  • Multiple system atrophy (MSA) is characterized by glial cytoplasmic inclusions (GCIs) containing alpha-synuclein.
  • Minimal change MSA (MC-MSA) is a rare subtype with neuronal loss primarily in the substantia nigra and locus coeruleus.

Purpose of the Study:

  • To investigate the neuropathological differences between MC-MSA and typical MSA.
  • To characterize the clinical and pathological progression in MC-MSA.

Main Methods:

  • Immunohistochemistry and semiquantitative assessment of brain regions from six MC-MSA and eight MSA control cases.
  • Comparison of neuronal cytoplasmic inclusions (NCIs) and GCIs load between groups.

Main Results:

  • MC-MSA cases showed significantly more NCIs in the caudate and substantia nigra compared to MSA controls.
  • No significant difference in GCI load was observed between MC-MSA and MSA controls in any region.
  • MC-MSA cases with sudden unexpected death presented with earlier onset and rapid progression, suggesting an aggressive variant. Other MC-MSA cases showed interrupted pathological progression.
  • Significant respiratory dysfunction and early orthostatic hypotension were present in all MC-MSA cases.

Conclusions:

  • Alpha-synuclein-associated oligodendroglial pathology in MSA may cause neuronal dysfunction preceding overt neuronal loss.
  • MC-MSA represents a distinct clinicopathological entity within the spectrum of MSA.