인격변화 및 인지장애로 발현된 재발성 다발연골염 1례

Abstract

Relapsing polychondritis is a rare, multisystem autoimmune disease. It is characterized by recurrent inflammation of the cartilage and connective tissues in the body. In this paper, we described a case of relapsing polychondritis initially presented symptoms of cognitive dysfunction and personality changes. A 63-year-old male reprented fever, cognitive impairment and personality changes. Brain magnetic resonance imaging revealed leptomeningeal and periauricular hyperintensities. A cerebrospinal fluid examination showed aseptic meningitis. As he reprented hearing difficulties, audiometry showed the sensory neural hearing loss. On physical examination, erythematous swollen auricles were noted. Auricle biopsy consisted with inflammation with perichondritis. He was diagnosed with relapsing polychondritis accompanied by leptomeningeal meningitis, and treated with methylprednisolone (62.5 mg/day for 3 days) followed by prednisolone 60 mg/day and methotrexate 7.5 mg/week. Fever and painful swellings on the both ears subsided. He showed improvement in cognitive function and personality. Although relapsing polychondritis is rare, it should be considered to be a possible cause of leptomeningeal meningitis.