Achromatopsia with amblyopia
Achromatopsia with amblyopia
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全色盲伴弱视
DOI：
10.1007/bf00149675
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发表时间：
1974
影响因子：
1.4
通讯作者：
S. Merin
中科院分区：
文献类型：
--
作者：
E. Auerbach;S. Merin
对39例先天性全色盲患者进行了包括视网膜电图在内的各种方法的明、暗适应检查。典型表现为畏光、视力低下、眼球震颤和不同程度的屈光不正。9例眼底正常。其他病例大多数表现为各种黄斑或中心凹异常。24例患者表现为色觉完全缺失，在Farnsworth D-15测试中几乎全部显示暗视线，视网膜电图（ERG）在光照下几乎消失。在黑暗中，在大多数患者的ERG显示暗适应机制，但有些ERG显示低于正常的明视成分，无论是在开始或在整个暗适应。通过2个额外的实验验证了在消色差者的ERG中存在明视活性。在一个，恢复的正波的四个消色差者进行了比较，在一个百分比规模与四个正常的主题，并发现是相似的，虽然稍快的过程中消色差者表明少明视活动比正常人。在第二，12个无色者与纯暗视ERG的ERG的正振幅记录在完成暗适应作为增加的刺激强度的函数，所有以上的明视阈值，并与16个正常受试者进行比较。在两组中，振幅随1.2对数强度范围线性增加，尽管消色差者的曲线斜率是正常人的1/4。在另一个实验中，在暗视范围内由超过5个对数单位的光引起的ERG的正波在消色差者中被发现具有与正常人非常相似的形状，这表明暗视活动在两个受试者中是相似的。可以解释为相对不敏感的电学方法加上视网膜明视机制在每个消色差者的亚正常。
Abstract39 patients suffering from congenital achromatopsia were investigated by various methods, including electroretinography, during both light and dark adaptation. This condition was typically expressed by the presence of photophobia, low visual acuity, nystagmus and various degrees of ametropia. The fundus was normal in 9 cases. Most of the other cases showed various macular or foveal abnormalities. 24 patients showed complete absence of color vision, and practically all displayed the ‘scotopic line’ in the Farnsworth Panel D-15 test.The electroretinogram (ERG) was almost always extinct in light. In the dark, in most patients the ERG displayed the scotopic mechanism solely, but some ERGs indicated subnormal photopic components either at the beginning or during all dark adaptation. This presence of photopic activity in the ERG of achromats was verified by 2 additional experiments. In one, the recovery of the positive wave of four achromats was compared on a percentage scale with that of four normal subjects and found to be similar, although the slightly faster course in achromats indicates less photopic activity than in normals. In the second, the positive amplitudes of the ERGs of 12 achromats with purely scotopic ERGs were recorded at completed dark adaptation as function of increasing stimulus intensities, all above the photopic threshold, and compared with those of 16 normal subjects. The amplitudes increased linearly with the 1.2 log intensity range in both groups, though the slope of the curve of the achromats was 1/4 that of the normals. In another experiment, the positive wave of the ERG, as elicited by light over 5 log units in the scotopic range, was found in an achromat to be of very similar shape as that of a normal, indicating scotopic acitivity to be similar in both subjects.The fact that, nevertheless, photopic components were not demonstrable in most ERGs, despite present photopic activity, can be explained by the relatively insensitive electrical method coupled with the subnormality of the retinal photopic mechanism in every achromat.