title: Relapsing polychondritis with features of dementia with Lewy bodies.
abstract: 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.
authors.affiliation: Institute for Brain Aging and Dementia, University of California, Irvine, CA 92697-4540, USA. ehead@uci.edu
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Head
authors.forename: Arnold
authors.initials: A
authors.lastname: Starr
authors.forename: Ronald C
authors.initials: RC
authors.lastname: Kim
authors.forename: Anna
authors.initials: A
authors.lastname: Parachikova
authors.forename: Genaro E
authors.initials: GE
authors.lastname: Lopez
authors.forename: Malcolm
authors.initials: M
authors.lastname: Dick
authors.forename: David H
authors.initials: DH
authors.lastname: Cribbs
mesh_terms: D000368:Aged; D000679:Amygdala / Q000473:pathology; D003937:Diagnosis, Differential; D018728:Entorhinal Cortex / Q000473:pathology; D006624:Hippocampus / Q000473:pathology; D006801:Humans; D020961:Lewy Body Disease / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D011081:Polychondritis, Relapsing / Q000175:diagnosis / Q000473:pathology*