An autopsy case of perivasculitic meningoencephalitis associated with relapsing polychondritis presenting with central nervous system manifestation

Abstract

A 76-year-old woman presented with decreased spontaneous speech and voluntary behavior. She developed impaired consciousness, as well as swelling of her ears, with subsequent gradual worsening of such symptoms. Prolonged fever, nuchal rigidity, hearing loss, and elevated plasma CRP and erythrocyte sedimentation rate were observed. CSF showed elevation of protein and IgG, and pleocytosis. Cranial MRI showed progressive brain atrophy and multiple high intensity lesions in the white matter on T2-weighted imaging and FLAIR imaging. The histopathology of biopsy sample from auricular cartilage was consistent with the relapsing polychondritis (RP). Accordingly, the diagnosis of meningoencephalitis associated with RP was made. Impaired consciousness was transiently improved by steroid pulse therapy, but subsequently worsened in spite of intensive immunosuppressive therapy with prednisolone, methotrexate and cyclosporine. Histological examination of the autopsied brain revealed perivascular lymphocyte infiltration in the pia mater and the cerebrum white matter, and inflammatory destruction of the myelin sheath. Meningoencephalitis of this case was thought to be caused by RP-associated perivascular inflammation.