A case of aseptic meningoencephalitis in adult-onset Still's disease / 대한내과학회지

ABSTRACT

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology, affecting mainly young adults,characterized by a high spiking quotidian fever, arthralgia or arthritis, evanescent salmon-colored maculopapular rash. It affects almost all organs, but neurological manifestations of AOSD are rare and could be responsible for a delay in diagnosis. We describe a case of AOSD who developed aseptic meningoencephalitis without any infectious cause. A 23-year-old woman was admitted because of high quotidian fever, arthralgia, maculopalpular rashes, leukocytosis with increased ferritin. During treatment by prednisolone, she suddenly developed status epilepticus. The brain image, electroencephalogram, and cerebrospinal fluid analysis did not show any abnormality including evidence of infection. After methyprednisolne pulse therapy, her clinical symptoms and laboratory tests including ferritin dramatically improved, and she could be discharged. To our knowledge, this is the first reported case of aseptic meningoencephalitis in a patient with AOSD in Korea.