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KMID : 0360220120530071041
Journal of the Korean Ophthalmological Society
2012 Volume.53 No. 7 p.1041 ~ p.1045
Specific Spectral Domain Optical Coherence Tomographic Findings of Oguchi Disease
Hyun Joo

Park Tae-Kwann
Chang Jee-Ho
Ohn Young-Hoon
Abstract
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.
KEYWORD
Dark adaptation, Electroretinography, Mizuo-Nakamura phenomenon, Oguchi disease, Optical coherence tomography
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