成人発症Still's病の神経学的症状の症例レビュー

Neurological manifestations of adult-onset Still's disease-case-based review.

• Mariana Santos
• Daniela Rodrigues
• Helder Santos
• Catarina Parente
• Catarina Carvalho
• Rodrigo Rufino
• Inês Almeida
• Hugo Miranda
• António Cardoso
• Martinho Fernandes

抄録

成人発症型Still病（AOSD）は，その病態生理がまだ十分に解明されていない稀な炎症性疾患である．我々は、発熱、関節痛、全身性炎症性疾患を示唆する臨床検査値異常を呈した66歳男性の症例を報告する。診断トレーニング中に神経症状、すなわち突然の交絡症候群と難聴を発症したが、コルチコステロイド療法で改善した。悪性腫瘍、感染症（神経系感染症）、その他のリウマチ性疾患を除外し、山口基準でAOSDと診断した。しかし，まれに神経症状を呈する症例もあり，臨床家がこれらの症状に気づかない場合には，診断がより困難になることがある．そこで著者らは，AOSDの神経学的症状を呈した患者の症例を紹介する。

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder in which pathophysiology is yet to be fully understood. We report the case of a 66-year-old male that presents with fever, arthralgia, and laboratory abnormalities suggestive of a systemic inflammatory disease. During a diagnostic workout, the patient developed neurological symptoms, namely a sudden confounding syndrome and hearing loss that improved with corticosteroid therapy. After exclusion of malignancy, infection-namely nervous system infection-and other rheumatic diseases, AOSD diagnosis was made using the Yamaguchi criteria. In some rare cases, neurological symptoms are present and the diagnosis may become even more challenging if the clinicians are not aware of these rare manifestations of AOSD. Therefore, the authors present the case of a patient with neurological manifestations of AOSD.