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要約 目的:瞳孔記録計による評価が有用であった小児の抗ミエリンオリゴデンドロサイト糖蛋白(MOG)抗体陽性視神経周囲炎の症例を報告する。
症例:左眼の急激な暗黒感,視力低下を呈した8歳の女児。矯正視力は右1.2,左50cm手動弁で,左相対的瞳孔求心路障害(RAPD)陽性であった。中心フリッカ値(CFF)は右45Hz,左測定不能であった。検眼鏡的に眼底および視神経に明らかな異常所見はなかった。瞳孔記録計(RAPDx®)にて,左眼のRAPD amplitude scoreは5.12と高値を示した。頭部MRIでは脂肪抑制画像で左視神経周囲炎を示唆する左視神経周囲の高信号を認めたが,視神経に異常信号はなかった。T2強調画像にて右側頭葉白質,右後頭葉白質と脊髄中心管の灰白質に高信号を認めた。抗MOG抗体は血清・髄液ともに陽性であった。左視神経周囲炎,右側頭葉・後頭葉白質病変,脊髄病変を有する抗MOG抗体関連疾患と診断し,ステロイドパルス療法を施行した。
結果:第15病日には左矯正視力0.6,CFF 20Hz,RAPD amplitude score 0.42と視機能の改善を認めた。ステロイド内服を継続し,第115病日に左矯正視力1.5,CFF 50Hz,RAPD amplitude score 0.2まで改善した。
結論:小児の視神経周囲炎において,瞳孔記録計は診断だけでなく治療経過の指標にも有用であった。
Abstract Purpose:To report a case of anti-myelin-origodendrocyte glycoprotein(MOG)antibody-positive pediatric optic perineuritis in which pupillary recorder was useful.
Case:An 8-year-old girl presented with a sudden dark sensation in the left eye. Best corrected visual acuity was 20/16 in the right eye and 50 cm hand motion in the left eye, with a positive left relative afferent pupillary defect(RAPD). The central flicker value(CFF)was 45 Hz on the right and unmeasurable on the left eye. There were no obvious abnormal findings in the fundus or optic nerve. The pupillary recorder(RAPDx®, Konan Medical, Inc.)showed a high RAPD amplitude score of 5.12 in the left eye. MRI of the head showed a high signal in the left peripapillary optic nerve area suggestive of left peripapillary inflammation on fat-suppressed images, but no abnormal signal in the optic nerve. Anti-MOG antibody was positive in both serum and spinal fluid. This case was diagnosed as anti-MOG antibody-related disease with left peripapillary optic neuritis, right temporal and occipital lobe white matter lesions, and spinal cord lesions. Steroid pulse therapy was administered.
Result:On the 15th day, visual function improved with corrected visual acuity of 20/32 in the left eye, CFF of 20 Hz, and RAPD amplitude score of 0.42. The patient was continued on oral steroids. On the 115th day, visual acuity was improved to 20/13, CFF was 50 Hz, and RAPD amplitude score was 0.2 in the left eye.
Conclusion:The pupillary recorder was useful for diagnosis and as an indicator of treatment course in children with optic perineuritis.
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