背景:明视ON通路缺陷与夜间视力丧失有关。然而,尚未探索检测杆状疾病(杆状体比视锥受影响更大)的ON功能的测量。我们评估了ON/OFF途径的心理物理学评估是否可用于区分视锥细胞为主的疾病和视杆为主的疾病。
方法:在iPad上使用“EyeSpeed”[iOS应用程序]对37例遗传性视网膜疾病患者进行了测试。测试在黑白噪声背景上显示随机数字(1-3)的亮或暗目标。参与者在触摸屏上回答,指示所显示的目标的正确数量。结果变量-反应时间,使用标准的受试者工作特征(ROC)分析评估了对亮和暗目标的准确性和性能指标(速度[1/反应时间]*准确性)的诊断能力。
结果:锥和棒为主组的平均±标准差年龄和视力为25.15±11.74岁,0.80±0.25logMAR和28.3±14.29年,0.48±0.26logMAR,分别。杆状疾病[四分位间距]对光目标的中位反应时间为5.28s[3.17],显着大于患有视锥细胞为主疾病的患者(2.07s[0.93];Mann-WhitneyU检验,p<0.001)。在所有评估的结果变量中,对光标靶的反应时间(≥2.98s的标准)在ROC曲线下表现出最高面积(面积=0.89±0.11;p<0.001),敏感性和特异性分别为82.4%和85%。
结论:使用ON/OFF通路范例对光照目标的反应时间是区分以棒状和视锥为主的视网膜营养不良的有效标记。使用基于片剂的测试测量的ON通路功能可以作为诊断具有挑战性的光感受器特异性遗传性视网膜疾病的补充测试。
The photopic ON pathway defect is associated with nocturnal vision loss. However, the measurement of ON function to detect a rod-dominated disease (rods affected more than cones) has not been explored. We evaluated whether the psychophysical evaluation of ON/OFF pathways can be used to distinguish cone-dominated from rod-dominated diseases.
Thirty-seven patients with inherited retinal diseases were tested using the \'EyeSpeed\' [iOS application] on an iPad. The test displayed a random number (1-3) of light or dark targets on a black-and-white noise background. Participants responded on a touch screen indicating the correct number of targets displayed. The outcome variables-reaction time, accuracy and performance index (speed [1/reaction time] * accuracy) to both light and dark targets were assessed for diagnostic ability using standard receiver-operating characteristic (ROC) analysis.
Mean ± standard deviation age and visual acuity for the cone- and rod-dominated groups were 25.15 ± 11.74 years, 0.80 ± 0.25 logMAR and 28.3 ± 14.29 years, 0.48 ± 0.26 logMAR, respectively. The median reaction time to light targets in rod-dominated disease [interquartile range] was 5.28 s [3.17], significantly greater than for patients with cone-dominated disease (2.07 s [0.93]; Mann-Whitney U test, p < 0.001). Amongst all of the outcome variables evaluated, the reaction time to light targets (criterion of ≥2.98 s) exhibited the highest area under the ROC curve (area = 0.89 ± 0.11; p < 0.001), with a sensitivity and specificity of 82.4% and 85% respectively.
Reaction time to light targets using the ON/OFF pathway paradigm is a valid marker to differentiate between rod- and cone-dominated retinal dystrophies. ON pathway function measured using a tablet-based test could act as a supplemental test in the diagnosis of challenging photoreceptor-specific inherited retinal diseases.