핵주변항호중구세포질항체와 연관된 경수막염에 동반된 양안 안와첨부증후군
Orbital Apex Syndrome with Perinuclear Anti-neutrophil Cytoplasmic Antibody-associated Pachymeningitis
최용제, 최정윤, 양희경,
소속 상세정보
최용제 ( Choi Yong-Je )
Seoul National University College of Medicine Seoul National University Bundang Hospital Department of Ophthalmology
최정윤 ( Choi Jeong-Yoon )
Seoul National University College of Medicine Seoul National University Bundang Hospital Department of Neurology
양희경 ( Yang Hee-Kyung )
Seoul National University College of Medicine Seoul National University Bundang Hospital Department of Ophthalmology
Abstract
Purpose: To report a rare case of perinuclear anti-neutrophil cytoplasmic antibody-associated pachymeningitis presenting as bilateral orbital apex syndrome.
Case summary: A 79-year-old female presented with a visual acuity decrease and ophthalmoplegia in both eyes from 1 week prior to her visit. Orbital magnetic resonance imaging showed diffuse hypertrophy and enhancement of the dura mater near the optic canal and superior orbital fissure causing orbital apex syndrome. The serum titer of perinuclear anti-neutrophil cytoplasmic antibody was elevated. Based on these findings, she was diagnosed with pachymeningitis related to perinuclear anti-neutrophil cytoplasmic antibody-associated vasculitis. The antibody titers decreased and ophthalmoplegia recovered after treatment with high dose intravenous steroid injection, cyclophosphamide, and long-term maintenance therapy with oral azathioprine and prednisolone. However, her visual acuity did not improve in either eye.
Conclusions: Perinuclear anti-neutrophil cytoplasmic antibody associated vasculitis can present as orbital apex syndrome and should be considered as a differential diagnosis.
키워드
Pachymeningitis; Perinuclear anti-neutrophil cytoplasmic antibody
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