Relapsing polychondritis with features of dementia with Lewy bodies
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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.
KeywordsComplement 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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