Brecelj J, Stirn-Kranjc B
Institute of Clinical Neurophysiology, University Medical Centre, Ljubljana, Slovenia.
Eye (Lond). 1999;13 ( Pt 1):47-54. doi: 10.1038/eye.1999.10.
Our prospective clinical and electrophysiological study of children suspected of Leber's congenital amaurosis (LCA) was aimed to follow-up the course of their visual dysfunction.
Electroretinography (ERG) and visual evoked potentials (VEP) to white flash stimulation were simultaneously recorded in 9 children at least twice.
The first flash ERG and flash VEP recordings were performed when children were 3-17 months old (mean age 7.6 months). Flash ERG was not recordable in 8 children; flash VEP to binocular stimulation could not be detected in 3, was delayed in 2, attenuated in 2, both attenuated and delayed in 1, and without evident abnormality in 1 of the 9 children. On the last examination (mean age 33.8 months) flash VEP activity was recordable in all children, while flash ERG was recordable in 1. Electrophysiological follow-up (mean duration 26.2 months) showed no deterioration of flash VEP in 8 children.
In children of LCA simultaneous recording of flash ERG and flash VEP in alert children was helpful to indicate the nature of the visual problem for diagnostic and follow-up purposes.
我们对疑似莱伯先天性黑蒙（LCA）的儿童进行前瞻性临床和电生理研究，旨在追踪其视觉功能障碍的病程。
对9名儿童至少进行了两次同时记录白色闪光刺激下的视网膜电图（ERG）和视觉诱发电位（VEP）。
首次闪光ERG和闪光VEP记录在儿童3 - 17个月大时进行（平均年龄7.6个月）。9名儿童中，8名儿童的闪光ERG无法记录；双眼刺激的闪光VEP在3名儿童中无法检测到，2名儿童延迟，2名儿童波幅衰减，1名儿童波幅衰减且延迟，1名儿童无明显异常。在最后一次检查时（平均年龄33.8个月），所有儿童均可记录到闪光VEP活动，而闪光ERG仅在1名儿童中可记录。电生理随访（平均持续时间26.2个月）显示，8名儿童的闪光VEP无恶化。
对于LCA儿童，在清醒儿童中同时记录闪光ERG和闪光VEP有助于明确视觉问题的性质，以用于诊断和随访。
