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Diffuse putaminal degeneration without iron deposition in multiple system atrophy.

Shin-Ichi Ueno1, Taiji Tsunemi1, Daisuke Taniguchi1

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Multiple system atrophy with parkinsonism (MSA-P) typically shows dark putamen on MRI. This case revealed a bright putamen, indicating iron-independent damage in advanced MSA.

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Area of Science:

  • Neurology
  • Neuroimaging
  • Pathology

Background:

  • Multiple system atrophy (MSA) is a progressive neurodegenerative disease.
  • The parkinsonian variant (MSA-P) often presents with putaminal hypointensities on T2 MRI.
  • This is typically attributed to iron accumulation.

Observation:

  • A 57-year-old woman with MSA-P exhibited atypical serial brain MRI findings.
  • Progressive T2 hyperintensity was observed in the putamen, contrasting with typical hypointensity.
  • Clinical symptoms included parkinsonism, orthostatic hypotension, and autonomic dysfunction.

Findings:

  • Neuropathological examination confirmed MSA with severe putaminal neuronal loss, gliosis, and glial cytoplasmic inclusions.
  • Prussian blue staining revealed only mild iron deposition in the affected putamen.
  • The progressive T2 hyperintensity correlated with severe neurodegeneration, not significant iron accumulation.

Implications:

  • Advanced MSA-related putaminal damage can manifest as T2 hyperintensity on MRI.
  • This suggests that iron-independent mechanisms play a role in MSA pathophysiology.
  • Revises understanding of MRI signal changes in MSA and their underlying pathological basis.