특발성 비대경수막염에 동반된 급성 양안 시력 소실
Acute Bilateral Visual Loss with Idiopathic Hypertrophic Pachymeningitis
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목적: 특발성 비대경수막염에 동반된 양안의 시신경병증 증례를 경험하여 이를 보고하고자 한다. 증례요약: 66세 여자 환자가 내원 6일 전 발생한 우안의 급격한 시력 감소, 3일 전부터 시작된 좌안의 급격한 시력 감소를 주소로 내원하였다. 시력은 우안 광각 없음, 좌안은 안전 수지 30 cm이었고 우안에 상대구심성동공운동장애가 있었다. 뇌자기공명영상에서 뇌경막이 비후되고 조영증강되고 있었고 혈액검사에서 적혈구침강속도, 세포반응단백질 수치가 증가되었고 골수세포형과산화효소-항호중구세포질항체(myeloperoxidase-antineutrophil cytoplasmic antibody, MPO-ANCA) 양성 소견을 보였다. 다른 신경과적, 내과적 이상은 찾을 수 없었다. 이에 특발성 비대경수막염으로 진단하였고 고용량 메틸프레드니솔론 충격 요법을 시행하였다. 스테로이드 치료2일 후 시력은 우안 광각 있음, 좌안은 0.6으로 호전되었고 3개월 후에는 우안 안전수지 20 cm, 좌안 0.7이었다. 결론: 저자들은 드문 질환인 특발성 비대경수막염과 연관된 시신경병증을 보고하며 시신경병증의 여러 가지 원인 중의 하나로서 비대경수막염도 고려해야 할 것으로 생각한다.
Abstract
Purpose: To report a case of bilateral optic neuropathy related with idiopathic hypertropic pachymeningitis. Case summary: A 66-year old woman presented with acute visual loss that developed 6 days previously in the right eye and 3 days prior in the left eye. During the initial evaluation, her visual acuity was light perception in the right eye and counting fingers at 30 cm in the left eye. A relative afferent papillary defect (RAPD) was noted in the patient’s right eye. On brain MRI, the duramater was thickened and enhanced by contrast. The erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP) level were increased and myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) as positive. Other neurological and medical abnormalities were not found. Under the suspicion of bilateral optic neuropathy due to idiopathic hypertrophic pachymeningitis, the patient was treated with methylprednisolone pulse therapy. Two days after the treatment, the visual acuity was hand movements at 20 cm in the right eye and 0.6 in the left eye. Three months after the treatment, the visual acuity was counting fingers at 20 cm in the right eye, and 0.7 in the left eye. The right eye showed optic disc pallor and diffuse retinal nerve fiber layer (RNFL) defect. The left eye showed a suspicious RNFL defect, and was otherwise normal. Conclusions: The authors report a rare case of optic neuropathy related with idiopathic hypertrophic pachymeningitis. Idiopathic hypertrophic pachymeningitis should be considered as one of the various causes of optic neuropathy
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