原発性シェーグレン症候群（SjS）に視神経炎と脳脊髄炎を合併し，抗アクアポリン4抗体が陽性であった25歳女性例
作者： Hirotaka Kato , Hiroo Ichikawa , Daigo Hayashi , Takahiro Yamazaki , Youhei Ohnaka
DOI: 10.5692/CLINICALNEUROL.49.576
关键词: Medicine 、 Dermatology
摘要: 症例は8歳時に，耳下腺腫脹と口腔内乾燥症状で発症した原発性シェーグレン症候群（SjS）の25歳女性．10歳時に右片麻痺が出現．MRIにて脳・脊髄病変をみとめ，中枢神経病変をともなうSjSとしてステロイド加療を受けていた．25歳時，左眼視力低下，右上肢のしびれ，両下肢の脱力を主訴に入院．左球後視神経炎とMRIにて皮質下白質領域に多発性病変がみられ，胸髄は萎縮し，頸胸髄の灰白質を中心に縦に長い病変をみとめた．抗アクアポリン4が抗体陽性．ステロイドパルス療法を施行し，症状は軽快した．本例は自己免疫疾患に関連した視神経脊髄炎関連疾患群として捉えることができ，若年で発症しSjSが先行した点が特異であった．
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