앞허혈시신경병증으로 발견된 모야모야병 1예
A Case of Moyamoya Disease Initially Presenting as Anterior Ischemic Optic Neuropathy
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목적: 시력저하를 증상으로 내원하여 앞허혈시신경병증으로 진단된 환자에게서 진행된 모야모야병을 발견하여 그 증례를 보고하고자한다. 증례요약: 46세 여자 환자가 3일 전부터 시작된 두통과 시력저하를 주소로 내원하였다. 내원 당시 교정시력은 우안 20/25, 좌안 20/70이었고 좌안의 동공은 확장되어 있었으며 상대구심성동공장애를 보였다. 안저검사상 좌측 시신경유두 부종이 관찰되었다. 입원 후고용량 부신피질호르몬 주사요법을 시행하면서 자기공명 뇌혈관촬영을 시행하였는데 그 결과 모야모야병이 의심되었고 뇌혈관조영술로 확진하였다. 고용량 부신피질호르몬 주사요법을 시행 후 두통과 시력저하는 호전되었으며 신경외과와 안과에서 경과관찰 중이다. 결론: 앞허혈시신경병증을 첫 증상으로 하여 발견된 진행된 모야모야병을 경험하여 이를 보고한다.
Abstract
Purpose: To present a case of a patient with decreased visual acuity and anterior ischemic optic neuropathy diagnosed with advanced Moyamoya disease. Case summary: A 46-year-old woman presented sudden episodes of headache and decreased visual acuity. On her first visit, her best corrected visual acuity was 20/25 in the right eye and 20/70 in the left eye. The left eye pupil was dilated with a relative afferent papillary defect. Fundus examinations revealed disc swelling in the left eye. After being admitted, steroid pulse therapy was started and Magnetic Resonance Imaging (MRI) studies revealed Moyamoya disease. The diagnosis was confirmed via Magnetic Resonance Angiography (MRA). After steroid pulse therapy, the headaches and visual acuity improved and the patient is continuing follow-up visits at neurosurgery and ophthalmology clinics. Conclusions: Patients with Moyamoya disease may initially present symptoms and signs of anterior ischemic optic neuropathy.
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