McAnany J Jason, Park Jason C, Collison Frederick T, Fishman Gerald A, Stone Edwin M
Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, 1855 W. Taylor St., Chicago, IL, 60612, USA.
Department of Psychology, University of Illinois at Chicago, 1007 W. Harrison St., Chicago, IL, 60612, USA.
Doc Ophthalmol. 2016 Aug;133(1):61-70. doi: 10.1007/s10633-016-9551-0. Epub 2016 Jul 1.
To evaluate rod-isolated, cone-isolated, and combined rod and cone flicker electroretinograms (ERGs) as a possible means to identify electrophysiological abnormalities in carriers of X-linked retinoschisis (XLRS).
Full-field ERGs were recorded from six carriers of XLRS (aged 34-66 years) and eight normally sighted subjects (aged 27-59 years) under rod-isolated (ERGR), cone-isolated (ERGC), and combined rod and cone (ERGR+C) conditions. ERGs were obtained using a four-primary LED-based ganzfeld photostimulator and standard recording techniques. The four primaries were modulated sinusoidally in phase to achieve combined rod and cone activation (ERGR+C) or in different phases to achieve ERGR and ERGC by means of triple silent substitution. After 30 min of dark adaptation, 8- and 15-Hz ERGR, ERGC, and ERGR+C responses were obtained at a mean luminance level of 24 scot. cd/m(2). Standard ISCEV ERGs were also obtained from each subject.
The ISCEV and 15-Hz flicker ERGs were generally within the normal range for the carriers. The 8-Hz ERGR, ERGC, and ERGR+C amplitudes were also generally normal. In contrast, the carriers had ERGR, ERGC, and ERGR+C timing abnormalities, with phase advances beyond the range of normal for the ERGR (four carriers), ERGC (four carriers), and ERGR+C (three carriers). Only one carrier had normal 8-Hz responses under all conditions.
The 8-Hz ERG timing abnormalities in five of six carriers indicate that retinal function is not necessarily normal in carriers of XLRS. The 8-Hz flicker ERG may be useful for studying retinal function in these individuals.
评估视杆细胞孤立、视锥细胞孤立以及视杆细胞与视锥细胞联合的闪烁视网膜电图（ERG），以此作为识别X连锁视网膜劈裂症（XLRS）携带者电生理异常的一种可能方法。
在视杆细胞孤立（ERGR）、视锥细胞孤立（ERGC）以及视杆细胞与视锥细胞联合（ERGR+C）条件下，对6名XLRS携带者（年龄34 - 66岁）和8名视力正常的受试者（年龄27 - 59岁）进行全视野ERG记录。使用基于四原色发光二极管的全视野光刺激器和标准记录技术获取ERG。通过三重消隐替代，使四原色以同相进行正弦调制以实现视杆细胞与视锥细胞联合激活（ERGR+C），或以不同相位实现ERGR和ERGC。在暗适应30分钟后，于平均亮度水平24 scot.cd/m²下获取8赫兹和15赫兹的ERGR、ERGC及ERGR+C反应。同时也从每位受试者获取标准的国际临床视觉电生理学会（ISCEV）ERG。
ISCEV和15赫兹闪烁ERG在携带者中总体处于正常范围。8赫兹的ERGR、ERGC及ERGR+C振幅总体也正常。相比之下，携带者存在ERGR、ERGC及ERGR+C的时间异常，ERGR（4名携带者）、ERGC（4名携带者）和ERGR+C（3名携带者）的相位提前超出正常范围。只有一名携带者在所有条件下8赫兹反应正常。
6名携带者中有5名出现8赫兹ERG时间异常，这表明XLRS携带者的视网膜功能不一定正常。8赫兹闪烁ERG可能有助于研究这些个体的视网膜功能。
