면역억제제 치료로 호전 된 급성 대상잠재성 외망막병증 1예
Acute Zonal Occult Outer Retinopathy, Responsive to an Immunosuppressive Agent: A Case Report
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목적: 구후시신경염으로 오진되었던 급성 대상잠재성 외망막병증 환자에서 면역억제제 치료로 호전된 1예를 보고하고자 한다. 증례요약: 28세 여자환자가 좌안 광시증과 시야장애를 주소로 타병원을 방문하였다. 시야검사에서 발견된 좌안 시야장애를 설명할만 한 안저 이상은 없었고, 신경학적 검사와 뇌자기공명영상도 정상으로 구후시신경염 진단 하 스테로이드 대량요법치료를 시행하였다. 증상발현 4개월 후 본원 내원시 교정시력 우안 1.0, 좌안 안전수동, 좌안 구심동공운동장애는 없었고, 전안부 및 안저검사에서 특이 소견없었다. 시유발전위도와 형광안저혈관조영술은 정상이었고, 좌안 시야검사에서 시야결손, 안저자가형광에서 황반부 주변의 과형 광 소견, 망막전위도에서 떨어진 반응소견 보였다. 빛간섭단층촬영에서 안저 주변부의 광수용체 내외층 경계부의 소실이 있었다. 급성 대상잠재성 외망막병증으로 진단 후 면역억제제로 치료하였고 10개월 후 시력 0.3으로 호전되었다. 결론: 광시증이 있는 젊은 여자에서 정상적인 안저 소견과 맞지 않는 급격한 시야장애가 있을 때에는 급성 대상잠재성 외망막병증을 감별하기 위하여 망막전위도와 빛간섭단층촬영등이 필요하고, 면역억제제 치료가 도움이 될 수 있다.
Abstract
Purpose: To report a case of acute zonal occult outer retinopathy (AZOOR), initially misdiagnosed as retrobulbar optic neuritis, which was responsive to an immunosuppressive agent. Case summary: A 28-year-old female with photopsia and a visual field defect in the left eye was referred to a hospital. There were no fundus abnormalities to explain her left visual field defect. Neurologic examination and brain MRI were normal. The patient was diagnosed with retrobulbar optic neuritis and treated with high-dose steroids. Four months after the onset of symptoms, she visited our hospital. Visual acuity was hand motion in the left eye. No relative afferent pupillary defect in the left eye and no anterior segment or fundus abnormalities were observed. A visual field examination of the right eye was normal and revealed field defect in the left eye. No abnormality was noted in the visual evoked potential test or fluorescein angiography. All examinations of the right eye were normal. In the left eye, fundus autofluorescence showed a hyperautofluorescent spot at the posterior pole, there was a decreased response in electroretinography and spectral domain optic coherence tomography showed that the junction between the photoreceptor inner and outer segments (IS/OS) was faintly visible only in the fovea. With the presumptive diagnosis of AZOOR, the patient was treated with an immunosuppressive agent. Visual acuity improved to 20/80 in the left eye at 10 months after the onset of symptoms. Conclusions: Electroretinogaphy is essential to diagnose AZOOR in patients with photopsia, visual field defect and minimal or no fundus changes, especially in a young women. There is currently no proven standard treatment, however immunosuppressive agents may be helpful.
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