ADAPTATION OF RESIDUAL ROD SIGNALS CSNB PATIENTS

CSNB 患者残余棒状信号的适应

• 批准号: 3261181
• 负责人: ROCKEFELLER S YOUNG
• 金额: $ 4.66万
• 依托单位: TEXAS TECH UNIVERSITY HEALTH SCIS CENTER
• 依托单位国家: 美国
• 财政年份: 1986
• 资助国家: 美国
• 起止时间: 1986-09-01 至 1989-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/3261181
• 关键词: congenital vision disorder; electroretinography; human subject; light adaptations; neural information processing; night blindness; psychophysics; retinal adaptation; rod cell; stimulus /response; visual feedback; visual photoreceptor; visual photosensitivity; visual stimulus; visual threshold

The loss of scotopic sensitivity in human patients with recessively inherited Congenital Stationary Nightblindness (CSNB) has not been thoroughly investigated in the past. This investigation will examine whether the adaptation (i.e., the regulation of sensitivity to different light levels) of residual rod signals is normal. In one experiment, the response of rod photoreceptors is studied using corneal ERG records. The rod a-wave component is isolated from the cone component by computing a "difference waveform" from the responses elicited by photopically-balanced blue and red flashes. To evaluate adaptation, rod increment threshold curves derived from the "a-wave slope" are compared for normal subjects and CSNB patients. In a second experiment, the perceptual rod signals are measured psychophysically. Rod-mediated thresholds and adaptation effects are identified by spectral and directional sensitivity measurements. Then, adaptation is evaluated by comparing the rod increment threshold curves for normal subjects and patients. Rationale: Although patients with CSNB are nightblind, they have residual rod visual function, albeit with markedly reduced sensitivity. The presence of residual rod vision in patients permits a comparison of their vision with normal subjects. Specifically, the study of adaptation attempts to evaluate whether there is a defect in the patients' visual system to regulate sensitivity at different light levels. Importance: Studies of rare visual disorders, such as CSNB, can provide a unique insight about the human visual pathways and their modes of failure. The studies can improve the understanding of how the retina adapts to different levels of light and of the measurable electrical signals generated by the retina. Advancement of the knowledge in these areas, in turn, has practical significance ot diagnostic procedures and to the development of noninvasive tests of retina function.

人类幕后患者的SCOTOPIC灵敏度丧失 继承的先天性固定夜总会（CSNB）尚未 过去彻底调查。 这项调查将检查 是否适应（即调节对不同的敏感性 残留杆信号的光级别是正常的。 在一个实验中， 使用角膜ERG记录研究了杆光感受器的响应。 这 通过计算A型杆A波分量从锥体组件中分离出来 来自光平衡引起的响应的“差异波形” 蓝色和红色闪光。 为了评估适应性，杆增加阈值 比较了正常受试者的“ A波坡”的曲线，并且 CSNB患者。 在第二个实验中，感知杆信号为 从心理物理进行测量。 杆介导的阈值和适应效果 通过光谱和方向灵敏度测量来识别。 然后， 通过比较杆的增量阈值曲线来评估适应 正常受试者和患者。 理由：尽管CSNB患者是夜间的，但它们有残留 杆的视觉功能，尽管敏感性显着降低。 这 患者存在残留的杆视力允许对其进行比较 正常受试者的视力。 具体而言，适应研究 试图评估患者视觉的缺陷 在不同光级别调节灵敏度的系统。 重要性：对CSNB等罕见视觉疾病的研究可以提供 关于人类视觉途径及其失败方式的独特见解。 这些研究可以提高对视网膜如何适应的理解 不同级别的光和可测量的电信号 由视网膜生成。 在这些领域的知识进步 转弯，具有诊断程序的实际意义， 开发视网膜功能的无创测试。