Cibis G W, Fitzgerald K M
Department of Ophthalmology, Children's Mercy Hospital, Kansas City, Missouri.
Doc Ophthalmol. 1994;86(1):11-22. doi: 10.1007/BF01224624.
Abnormal electroretinograms (decreased amplitude and prolonged implicit time > 2 standard deviations) in several patients with optic nerve hypoplasia (ONH) and developmental brain anomalies led us to study the electroretinogram (ERG) in 34 consecutive cases of ONH presenting to our practice. Ages of the subjects were between 7 months and 13 years (mean, 4 years). ERGs were recorded from each eye by means of a contact lens electrode and ganzfeld stimuli. Rod-dominated dark-adapted responses were recorded as well as cone-dominated light-adapted responses. When clinically indicated, brain imaging by either computed tomography (CT) or magnetic resonance imaging (MRI) was performed. The ERG was abnormal in 12 (35%) of the children, including five (42%) with unilateral ONH. Imaging studies of the brain in 12 children with ONH and an abnormal ERG disclosed brain malformations in nine (75%) of them compared to five (23%) in the group with ONH and a normal ERG. An abnormal ERG associated with ONH and brain malformations may represent retinal or transsynaptic degeneration beyond the ganglion cell layer and implies a shared causative mechanism.
数例视神经发育不全（ONH）及发育性脑异常患者出现异常视网膜电图（振幅降低且隐含时间延长>2个标准差），促使我们对在我院就诊的34例连续性ONH患者的视网膜电图（ERG）进行研究。受试者年龄在7个月至13岁之间（平均4岁）。通过接触镜电极和全视野刺激记录每只眼睛的ERG。记录了以视杆细胞为主的暗适应反应以及以视锥细胞为主的明适应反应。根据临床指征，进行了计算机断层扫描（CT）或磁共振成像（MRI）脑部成像检查。12名（35%）儿童的ERG异常，其中5名（42%）为单侧ONH。12名ERG异常的ONH儿童的脑部成像研究显示，其中9名（75%）存在脑畸形，而ERG正常的ONH组中这一比例为5名（23%）。与ONH和脑畸形相关的异常ERG可能代表神经节细胞层以外的视网膜或跨突触变性，并提示存在共同的致病机制。
