Miyake Y, Yagasaki K, Horiguchi M, Kawase Y
Jpn J Ophthalmol. 1987;31(1):81-7.
The on- and off-responses of photopic electroretinogram (ERG) were recorded by rectangular light stimuli in 13 patients with complete type and 9 patients with incomplete type of congenital stationary night blindness. The positive on-response (b-wave) was significantly smaller in both types than in normal subjects (P less than 0.001). The rapid off-response was normal in complete type patients, but was significantly smaller in incomplete type patients than in normal subjects (P less than 0.001). Since the rapid off-response mainly reflects the decay of the late receptor potential of the cones, incomplete type patients may have a visual disturbance in the cone itself. Normal rapid off-response with abnormal on-response in complete type patients suggests the existence of a photopic visual disturbance in the middle retinal layer. These results strongly suggest that these two types of congenital stationary night blindness have a different pathogenesis in the photopic visual pathway.
采用矩形光刺激记录了13例完全型和9例不完全型先天性静止性夜盲患者明视视网膜电图（ERG）的开反应和关反应。两种类型患者的正向开反应（b波）均显著小于正常受试者（P<0.001）。完全型患者的快速关反应正常，但不完全型患者的快速关反应显著小于正常受试者（P<0.001）。由于快速关反应主要反映视锥细胞晚期感受器电位的衰减，不完全型患者可能在视锥细胞本身存在视觉障碍。完全型患者快速关反应正常而开反应异常提示视网膜中层存在明视视觉障碍。这些结果强烈提示这两种类型的先天性静止性夜盲在明视视觉通路中具有不同的发病机制。
