Intracranial aneurysm associated with relapsing polychondritis

Abstract

We describe a 50-year-old man with relapsing polychondritis (RP) involving auricular cartilage, uveitis and hearing loss, who had an aneurysm of the anterior cerebral artery. Intracranial aneurysm is a rare manifestation of RP.

“Prominent ear sign” on diffusion-weighted magnetic resonance imaging in relapsing polychondritis

“…Previous reports of CNS involvement have included cases of vasculitis or vasculitic meningoencephalitis. 4,10,11 Coumbaras et al 12 reported a RP case with intracranial aneurysm, and they considered the possibility that this aneurysm resulted from underlying systemic vasculitis. Limbic encephalitis with RP mimicking herpes simplex virus encephalitis has also been reported.…”

Meningeal plasma cell granuloma with relapsing polychondritis

“…16,20,26 The association of an intracranial lesion with RP has occasionally been described. 1,3,5,6,11,14,19,[23][24][25]27 To our knowledge, however, this is the first reported occurrence of an intracranial mass lesion, a PCG, in a patient with RP. Although the relationship between RP and meningeal PCG is uncertain, an autoimmune origin might be considered in cases of aseptic meningitis associated with RP.…”

Relapsing Polychondritis With Encephalitis

“…This patient differs from other reported cases in how he presented only auricular chondritis before his encephalitis developed. [2][3][4][5][6][7][8][9] The diagnosis of RP with encephalitis was established by his clinical manifestations, CSF examinations, and MRI findings. Encephalitis can lead to severe sequelae if treatment is not initiated immediately.…”

“…Cranial nerve disorders, aseptic meningitis, meningoencephalitis, encephalitis, and cerebral aneurysm have been reported. [2][3][4][5][6][7][8][9][10] The cause of CNS involvement is believed to be associated with vasculitis evidenced by 1 autopsy case. 2 Such vasculitis of the CNS is not always associated with systemic vasculitis.…”