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要約 背景:多発性硬化症の特殊形である視神経脊髄炎で,特異的な自己抗体である抗アクアポリン4(AQP4)抗体が血清中に発見された。これにより古典的多発性硬化症と視神経脊髄炎が鑑別できる可能性がある。目的:抗AQP4抗体陽性視神経炎5例の報告。症例:過去1年間に視神経脊髄炎が疑われた16例で血清の抗AQP4抗体を測定し,5例(31%)が陽性であった。全例が女性で,年齢は15~78歳(平均50歳)であった。全5例に3椎体以上に及ぶ脊髄炎が併発していた。視神経炎はすべて片眼性であり,経過中に2例で対側にも発症した。矯正視力は光覚なしから1.2に及び,中心暗点,水平半盲,耳側半盲などの視野異常があった。3例はステロイドパルス療法で軽快し,1例は血漿交換療法の追加で改善した。結論:抗AQP4抗体陽性視神経炎は比較的高齢の女性に好発し,急速に進行し,脊髄炎が併発し,副腎皮質ステロイドなどの治療に反応する。その診断と治療では眼科と神経内科との連携が必要である。
Abstract. Background:Anti-aquaporin 4 antibody(anti-AQP4 antibody)may be present in the serum of patients with myelo-optic neuropathy. There is a possibility that classical multiple sclerosis and myelo-optic neuropathy may be differentiated using anti-AQP4 antibody as a marker. Purpose:To report 5 cases of myelo-optic neuropathy with positive anti-AQP4 antibody. Cases:Sixteen patients were diagnosed with optic neuritis during the past 12 months. Five cases(31%)were positive for anti-AQP4 antibody. All were females aged from 15 to 78 years,average 50 years. All had myelitis involving more than 3 vertebral bones. Optic neuritis was unilateral in all the cases. It became bilateral later in 2 cases. Corrected visual acuity ranged from no light perception to 1.2. Visual field defect included central scotoma,horizontal and temporal hemianopia. Pulsed corticosteroid was effective in 3 cases. Another case improved after additional plasma exchange. Conclusion:Optic neuritis positive for anti-AQP4 antibody is characterized by involvement of females in advanced age,rapid progression,association of myelitis,and improvement after systemic corticosteroid. Coordination between ophthalmologists and neurologists is essential in the diagnosis and treatment.
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