Tan Xue, Aoki Aya, Yanagi Yasuo
Department of Ophthalmology, University of Tokyo School of Medicine, Hongo, Bunkyo-ku, Tokyo, Japan.
Clin Ophthalmol. 2013;7:1587-90. doi: 10.2147/OPTH.S49496. Epub 2013 Aug 7.
Patients with the complete form of congenital stationary night blindness (CSNB) often have reduced visual acuity, myopia, impaired night vision, and sometimes nystagmus and strabismus, however, they seldom complain of color vision abnormality. A 17-year-old male who was at technical school showed abnormalities in the color perception test for employment, and was referred to our hospital for a detailed examination. He had no family history of color vision deficiency and no other symptoms. During the initial examination, his best-corrected visual acuity was 1.2 in both eyes. His fundus showed no abnormalities except for somewhat yellowish reflex in the fovea of both eyes. Electroretinogram (ERG) showed a good response in cone ERG and 30 Hz flicker ERG, however, the bright flash, mixed rod and cone ERG showed a negative type with a reduced b-wave (positive deflection). There was no response in the rod ERG, either. From the findings of the typical ERG, the patient was diagnosed with complete congenital stationary night blindness. This case underscores the importance of ERG in order to diagnose the cause of a color vision anomaly.
完全型先天性静止性夜盲（CSNB）患者通常视力下降、近视、夜视受损，有时还伴有眼球震颤和斜视，然而，他们很少主诉色觉异常。一名17岁的男性技校学生在就业色觉测试中表现异常，遂被转诊至我院进行详细检查。他无色觉缺陷家族史，也无其他症状。初诊时，他双眼最佳矫正视力均为1.2。除双眼黄斑区有稍发黄的反光外，眼底未见异常。视网膜电图（ERG）显示，视锥细胞ERG和30Hz闪烁ERG反应良好，然而，明闪光、混合视杆-视锥细胞ERG呈b波降低的阴性型（正性偏转）。视杆细胞ERG也无反应。根据典型的ERG检查结果，该患者被诊断为完全型先天性静止性夜盲。该病例强调了ERG在诊断色觉异常病因方面的重要性。
