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Specific Spectral Domain Optical Coherence Tomographic Findings of Oguchi Disease

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ÇöÁÖ ( Hyun Joo ) - ¼øÃµÇâ´ëÇб³ Àǰú´ëÇÐ ¾È°úÇб³½Ç

¹ÚŰü ( Park Tae-Kwann ) - ¼øÃµÇâ´ëÇб³ Àǰú´ëÇÐ ¾È°úÇб³½Ç
ÀåÁöÈ£ ( Chang Jee-Ho ) - ¼øÃµÇâ´ëÇб³ Àǰú´ëÇÐ ¾È°úÇб³½Ç
¿Â¿µÈÆ ( Ohn Young-Hoon ) - ¼øÃµÇâ´ëÇб³ Àǰú´ëÇÐ ¾È°úÇб³½Ç

Abstract

¸ñÀû: ¾ÈÀú°Ë»ç¿Í ¸Á¸·ÀüÀ§µµ °Ë»ç¸¦ ÅëÇØ ¿À±¸Ä¡º´À¸·Î Áø´ÜµÈ ȯÀÚÀÇ Æ¯Â¡ÀûÀÎ ºû°£¼·´ÜÃþÃÔ¿µ ¼Ò°ßÀ» °æÇèÇÏ¿´±â¿¡ À̸¦ º¸°íÇϰí ÀÚ ÇÑ´Ù.

Áõ·Ê¿ä¾à: 14¼¼ ³²ÀÚ°¡ 10³â ÀüºÎÅÍ Áö¼ÓµÇ´Â ¾ß¸ÍÁõÀ» ÁÖ¼Ò·Î ³»¿øÇÏ¿´´Ù. ȯÀÚÀÇ ¾ÈÀú´Â Ư¡ÀûÀΠȲ±Ýºû »ö¼Ò¹Ý»ç¸¦ º¸¿´°í 3½Ã°£ ÀÇ ¾Ï¼øÀÀ ÈÄ ÀÌ »ö¼Ò¹Ý»ç´Â Á¤»óÀ¸·Î µ¹¾Æ¿Ô´Ù(Mizuo-Nakamura phenomenon). Àüü¸Á¸·ÀüÀ§µµ¿¡¼­ ¸·´ë¼¼Æ÷¹ÝÀÀ Áß bÆÄ°¡ ¼Ò½ÇµÇ ¾î ÀÖ¾ú´Ù. Ãִ뺹ÇÕ ¹ÝÀÀ¿¡¼­ aÆÄ´Â 3½Ã°£ÀÇ ¾Ï¼øÀÀ ÈÄ ÁøÆøÀÌ Áõ°¡ÇÏ´Â ¼Ò°ßÀ» º¸ÀÌ´Â ¹Ý¸é, bÆÄ´Â ¾Ï¼øÀÀ ÈÄ ÁøÆøÀÇ Áõ°¡°¡ Å©Áö ¾Ê¾Æ À½¼º ÆÄÇüÀÌ ¶Ñ·ÇÇØÁö´Â ¼Ò°ßÀ» º¸¿´´Ù. ºû°£¼·´ÜÃþÃÔ¿µ»ó ¾Ï¼øÀÀ Àü¿¡´Â Á߽ɿÀ¸ñÁÖÀ§ ¸Á¸·ÀÇ ³»Àý/¿ÜÀý°ú ¸Á¸·»ö¼Ò»óÇÇÀÇ °£ °ÝÀÌ ¼Ò½ÇµÇ¾î µÎ ÃþÀÌ ÇϳªÀÇ ½ÅÈ£°­µµ·Î ³ªÅ¸³µÀ¸³ª, 3½Ã°£ÀÇ ¾Ï¼øÀÀ ÈÄ¿¡´Â µÎ ÃþÀ¸·Î ±¸º°µÇ´Â ½ÅÈ£°­µµ¸¦ º¸¿´´Ù.

°á·Ð: ¾ÈÀú°Ë»ç¿Í Àüü¸Á¸·ÀüÀ§µµ ¿Ü¿¡µµ ¾Ï¼øÀÀ ÀüÈÄÀÇ Æ¯Â¡ÀûÀÎ ºû°£¼·´ÜÃþÃÔ¿µ ¼Ò°ßÀÌ ¿À±¸Ä¡º´ÀÇ Áø´Ü¿¡ Áß¿äÇÑ ¿ªÇÒÀ» ÇÒ °ÍÀ¸ ·Î »ý°¢ÇÑ´Ù.
Purpose: To report specific spectral domain OCT findings of Oguchi disease diagnosed with fundoscopic examination and electrophysiological study.

Case summary: A 14-year-old patient visited our clinic with a complaint of night blindness for ten years. Fundoscopic examination showed a golden-yellow fundus reflex. After three hours of dark adaptation, the fundus color returned to normal (Mizuo-Nakamura phenomenon). In full-field ERG, rod b-wave was not detectable. The a-wave amplitude in maximal combined response increased after three hours of dark adaptation, although the b-wave amplitude was similar to the amplitude before dark adaptation, demonstrating a negative waveform. In the spectral domain OCT images of the perifoveal area, no gap between the retinal pigment epithelium and the inner segment/outer segment (IS/OS) junction was detected before prolonged dark adaptation, and a highly reflective band was shown. However, the gap appeared after three hours of dark adaptation, and two highly reflective bands were detected in the OCT images.

Conclusions: The characteristic OCT finding in addition to the specific fundoscopic finding and full-field ERG results may be useful to diagnose Oguchi disease.

Ű¿öµå

Dark adaptation; Electroretinography; Mizuo-Nakamura phenomenon; Oguchi disease; Optical coherence tomography
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