Acta Neuropathologica

, Volume 112, Issue 2, pp 217–225 | Cite as

Relapsing polychondritis with features of dementia with Lewy bodies

  • Elizabeth Head
  • Arnold Starr
  • Ronald C.  Kim
  • Anna Parachikova
  • Genaro E.  Lopez
  • Malcolm Dick
  • David H. Cribbs
Case Report


We describe a 72-year old man with clinical features suggestive of dementia with Lewy bodies (DLB) who proved neuropathologically to have degeneration induced by relapsing polychondritis (RP), an autoimmune inflammatory disorder of cartilaginous tissues. There was lymphocytic infiltration of the leptomeninges, perivascular cuffing, reactive astrocytosis, and activation of microglia in multiple brain areas all consistent with an immunologically mediated process. There was widespread neuronal loss within the hippocampus, entorhinal cortex, and amygdala as well as diffuse myelin pallor of cortical pathways. Elevated levels of complement proteins and endothelial markers of inflammation were observed, which are similar to previous reports in DLB. This study demonstrates that qualitatively similar inflammation-associated neurodegeneration is present in widespread regions of the brain in a RP case presenting clinically as DLB.


Complement Hippocampal cell loss Neuroinflammation White matter degeneration 



Funding supported by ADRC P50 AG16573, P50 AG000658, NIA-AG-20241 and NINDS-NS50895 (DHC). We would also like to extend our gratitude to the patient and his family for their assistance in the study.


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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Elizabeth Head
    • 1
    • 2
  • Arnold Starr
    • 1
    • 2
  • Ronald C.  Kim
    • 1
  • Anna Parachikova
    • 1
  • Genaro E.  Lopez
    • 1
  • Malcolm Dick
    • 1
  • David H. Cribbs
    • 1
    • 2
  1. 1.Institute for Brain Aging and DementiaUniversity of CaliforniaIrvineUSA
  2. 2.Department of Neurology, College of MedicineUniversity of CaliforniaIrvineUSA

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