Korean Journal of Medicine  2008;74(2):220-204

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

Jong Shin WOO 1 ; Na Ri YUN ; Yeon Ah LEE ; Sang Hoon LEE ; Hyung In YANG ; Seung Jae HONG

Affiliations

+expand

Keywords

Adult onset Still's disease; Meningoencephailtis

Country

Republic of Korea

Language

Korean

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.