Ruether K, Apfelstedt-Sylla E, Zrenner E
Universitäts-Augenklinik, Abteilung II, Tübingen, Germany.
Ger J Ophthalmol. 1993 Nov;2(6):429-35.
We examined 23 patients (18 families) with congenital stationary night blindness (CSNB) who showed a "negative" electroretinogram of the Schubert-Bornschein type. The goal of the study was to find evidence for the classification proposed by Miyake (complete and incomplete type) based on electroretinograms (ERGs) and dark-adaptation function and to look for additional classification parameters that argue for or against heterogeneity. In all, 13 patients revealed the complete type and 10 the incomplete type. The mean age of our patients was 24.7 years (median, 21 years; SD, 14.5 years). In both groups, almost all patients were myopic (mean, -6.05 D; SD, 3.77 D; median, -6.12 D), and there was a reduction in visual acuity (mean, 0.34; SD, 0.14; median, 0.35) without significant differences between the subgroups. In all, 56.5% of the patients suffered from nystagmus and 52.2% squinted. These results confirm the Miyake classification and suggest that only ERG and dark-adaptation data allow a discrimination between the two subtypes. The ongoing molecular analysis will show whether there are correlates on the molecular level.
我们检查了23例（18个家系）患有先天性静止性夜盲（CSNB）且呈现舒伯特 - 博恩施泰因型“阴性”视网膜电图的患者。本研究的目的是基于视网膜电图（ERG）和暗适应功能，寻找支持三宅（完全型和不完全型）所提出分类的证据，并寻找支持或反对异质性的其他分类参数。总共，13例患者为完全型，10例为不完全型。我们患者的平均年龄为24.7岁（中位数为21岁；标准差为14.5岁）。在两组中，几乎所有患者均为近视（平均为-6.05 D；标准差为3.77 D；中位数为-6.12 D），且视力下降（平均为0.34；标准差为0.14；中位数为0.35），亚组间无显著差异。总共，56.5%的患者患有眼球震颤，52.2%的患者斜视。这些结果证实了三宅分类，并表明只有ERG和暗适应数据能够区分这两种亚型。正在进行的分子分析将表明在分子水平上是否存在相关性。
