title: Relapsing polychondritis complicated by cognitive dysfunction: two distinct clinical phenotypes?
abstract: Relapsing polychondritis (RPC) is a rare, immune-mediated condition affecting approximately 3.5 per million population per year. Neurological involvement in RPC is still rarer and is presumed to be the result of a vasculitic process, although this is seldom confirmed in the literature. We present two cases of RPC complicated by cognitive dysfunction with contrasting clinical trajectories. Our findings suggest that there are two clinical phenotypes of cognitive dysfunction in RPC. The first is a fulminant, multisystem presentation with sub-acute cognitive decline mimicking central nervous system vasculitis, and we provide histopathological evidence of this process occurring. The other is an insidious cognitive decline without associated constitutional or systemic symptoms.
authors.affiliation: a Neurology Department , The Walton Centre for Neurology and Neurosurgery , Liverpool , UK.
authors.forename: R J B
authors.initials: RJ
authors.lastname: Ellis
authors.affiliation: a Neurology Department , The Walton Centre for Neurology and Neurosurgery , Liverpool , UK.
authors.forename: G K
authors.initials: GK
authors.lastname: Mbizvo
authors.affiliation: a Neurology Department , The Walton Centre for Neurology and Neurosurgery , Liverpool , UK.
authors.forename: A
authors.initials: A
authors.lastname: Jacob
authors.affiliation: a Neurology Department , The Walton Centre for Neurology and Neurosurgery , Liverpool , UK.
authors.forename: M
authors.initials: M
authors.lastname: Doran
authors.affiliation: a Neurology Department , The Walton Centre for Neurology and Neurosurgery , Liverpool , UK.
authors.forename: A J
authors.initials: AJ
authors.lastname: Larner
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D003072:Cognition Disorders / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000000981:diagnostic imaging / Q000209:etiology*
keywords: CNS histopathology; CNS vasculitis; relapsing polychondritis; reversible dementia