성인형 스틸씨병에서 동반된 무균성 뇌수막염 1예

• Published in: The Korean journal of medicine Vol. 74; no. 2; pp. 220 - 224
• Main Authors: 우종신, Jong Shin Woo, 윤나리, Na Ri Yun, 이연아, Yeon Ah Lee, 이상훈, Sang Hoon Lee, 양형인, Hyung In Yang, 홍승재, Seung Jae Hong
• Format: Journal Article
• Language: Korean
• Published: 대한내과학회 01.02.2008
• Subjects: Adult onset Still`s disease; Meningoencephailtis; 내과학; 뇌수막염; 성인형 스틸씨 병
• ISSN: 1738-9364; 2289-0769

Adult-onset Still`s disease (AOSD)에서 신경학적 이상이 동반되는 경우는 전세계적으로 드문 경우로 최근 저자들은 고열과 관절통, 피부 발진을 주소로 내원하여 AOSD로 진단된 환자에서 입원 치료도중 경련을 동반한 뇌수막염이 발생하여 AOSD의 신경학적 합병증으로 생각되는 환자 1예를 경험하였다. 환자는 보존적인 치료로 호전되었으며 별다른 후유증없이 회복하였다. 이는 국내에서 AOSD의 신경학적 이상으로 인한 뇌수막염에 대한 최초의 보고이다. 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. (Korean J Med 74:220-224, 2008)