Goldmann-Favre syndrome in a four-year-old-girl

We report a suspected early stage of Goldmann-Favre syndrome in a four-year-old girl complaining of poor visual acuity in both eyesOphthalmological examinations were performed including fundus examination, fluorescein angiography, and electrophysiological studies. However, dark adaptation and visual field testing were not performed because of her age.Ophthalmoscopic examinations revealed peripheral retinoschisis in the superior temporal retina, diffusely abnormal retinal pigment epithelium, and lack of macular and foveal reflexes in both eyes. Foveal retinoschisis was not observed and the vitreous did not show severe degeneration. Fluorescein angiography showed many leaking points posterior to the retinoschisis. Scotopic electroretinogram (ERG) was almost non-recordable. Photopic electroretinogram showed a marked reduction of the b-wave with a negative shape and delayed peak time. Flash visual evoked potentials were within normal range.It is very difficult to distinguish this case from X-linked juvenile retinoschisis by ophthalmological findings. However, Goldmann-Favre syndrome is most suspected from the fact that the patient is female and her parents and brothers are normal.     

低温对眼电生理的影响

正常有色兔30只,分为37℃、0℃、-5℃三组,经封闭式玻璃体切除术后,采用三种不同温度灌注1小时。于术前及术后1周、2周、1月分别作各眼明暗适应,不同强度闪光视网膜电图(ERG),所得a、b波振幅数据输入电子计算机进行差异性分析,显示-5℃在术后1周ERG的a波振幅与术前有显著性差异。提示过低温度会损害视网膜功能,在眼低温玻璃体手术中不宜采用。     

Absence of ganglion cell subcomponents in multifocal luminance electroretinograms

Multifocal flash electroretinograms (ERG) were recorded binocularly (n= 18). Areas were equal or scaled with excentricity. The latter were expected to increase the total amplitude if ganglion cell subcomponents were involved. Amplitudes were intercorrelated and the factor structure was established. Scaling had no influence on amplitudes or on factors. Reliability and sensitivity were high. The second kernel first slice from the nine hexagons across the midline showed differences near the macula but no component increasing in latency with distance from the disc. Thus, multifocal flash ERG have linear spatial summation and no ganglion cell subcomponents.     

The multifocal electroretinogram in retinal detachment

We evaluated the retinal electrophysiologic function in both the detached and attached areas of eyes with retinal detachment, and assessed the functional recovery of these areas after surgery by quantifying the results, obtained from multifocal electroretinograms. Multifocal electroretinographic recordings and central 0°, to 30°, visual field tests were performed preoperatively and 2 weeks 1, 3 and 6 months postoperatively in 12 patients with unilateral retinal detachment. Each patient's response to the multifocal electroretinogram and the visual field test was classified into two groups: group A, the response from the attached area; and group B, that from the detached retinal area. Individual mean deviation and percentage mean deviation were calculated for each group. All retinal detachments were successfully reattached by the conventional scleral buckling method. The retinal sensitivity in the visual field test of all the patients in group B greatly improved. However, the percentage mean deviation in the response density of the multifocal electroretinogram in group B was −81% preoperatively and −63% at 6 months postoperatively. Thus, the improvement was confined within narrow limits. The response density of the multifocal electroretinogram in group A was very low, and never improved beyond −50% of percentage mean deviation. In the eyes with retinal detachment, electroretinogram response in both the attached and detached areas was more disturbed, than predicted by means of the visual field test during the course of this study.     

LED刺激器多焦视网膜电图一阶和二阶反应波形研究

目的研究二极管发光器(LED)的多焦视网膜电图(mfERG)的一阶及二阶波形特点。设计前瞻性、非对照干预研究。研究对象18例(18眼)正常人。方法18眼随机分为两组分别作阴极射线管(CRT)及LED的mfERG检查。LED刺激时间分为1/10(1．7ms)、3/10(5ms)、5/10(8．3ms)、7/10(11．7ms)、10/10(16．7ms)5种。主要指标mfERG总和反应波形特点,P1、N1及N2波形态、振幅密度及潜伏期。结果CRT及LED刺激器mfERG一阶反应波形基本相似；CRT及LED刺激器的mfERG二阶反应波形不同,前者的P1波呈尖峰状,后者的P1波较宽,呈方形,且伴有一个较深的N2波。随着刺激时间的延长,LED刺激器的mfERG一阶及二阶反应中的P1波及N1波的振幅密度增加,潜伏期延长。结论CRT及LED刺激器的mfERG一阶反应波形基本相同,但LED的二阶反应波形更丰富,其可能包涵了更多视网膜内层信息。(眼科,2006,15：351-355)     

Electrophysiologic findings in optic nerve dysfunction associated with multiple evanescent white-dot syndrome

Electrophysiologic findings in a case of multiple evanescent white-dot syndrome were studied. A 37-year-old woman presented with multiple white dots, granularity of the macula, and optic disc swelling in her left fundus. The electroretinogram and electro-oculogram revealed abnormal findings indicative of changes in the retinal pigment epithelium and photoreceptors. In addition, relative afferent pupillary defect, enlargement of the blind spot, a decrease in the critical fusion frequency, staining of the optic disc on fluorescein angiography and prolonged latency of the P100 component with decreased amplitude in pattern-reversal visual evoked cortical potential strongly suggested dysfunction of the optic nerve in this patient.Abbreviations MEWDS multiple evanescent white-dot syndrome     

Visual Functions and TraceElement Metabolism in Tobacco-toxic Optic Neuropathy

Visual functions and nutrition metabolic characteristics werestudied in 8 subjects(16 eyes)with tobacco-toxic optic neuropathy(TTON).Their visual functions tested by psychophysical and electrophysiologicmethods showed that:1.central vision diminished in 16 eyes,2.dyschromatopsias were found in 14 tested eyes,3.bilateral symmetricalcentral or cecocentral scotomas were the visual field characteristics in allcases,4.PVEP were severe abnormal in 3 spatial frequencies in all cases and56.3% of 15‘ checkboard ...     

Relationships between the electroretinogram a-wave,b-wave and oscillatory potentials and their application to clinical diagnosis

The electroretinogram is the electrical response of the retina to a light stimulus. The amplitude and temporal pattern of its components, the a-wave, the b-wave and the oscillatory potentials, depend on the functional integrity of the retina, on the intensity of test flash reaching the retina and on the ambient illumination. The latter contributions to the normal variability in the electroretinogram can be circumvented by constructing the relationships between the different electroretinogram waves. The electroretinogram responses were recorded from 18 dark-adapted subjects with normal vision. The slope of the a-wave and the amplitude of the b-waves were measured in the time domain. The oscillatory potentials were isolated by a digital filter and were transformed to the frequency domain for quantitative measurement. The relationship between each pair of variables could be fitted by linear segments. Our findings suggest that this mode of electroretinogram analysis can be useful in localizing the site of action of retinal disorders and that the relationship between the a-wave slope and the power density of the oscillatory potentials is a useful index for identifying disorders of the inner retina.     

Vitreal and intraretinal responses to contrast reversing patterns in the pigeon eye

The pattern electroretinogram (PERG) has been recorded vitreally and intraretinally in the pigeon eye. The amplitude of the PERG increases monotonically as pattern contrast is increased, with saturation at high levels. The PERG of the central yellow field has band-pass spatial tuning, with a high frequency cut-off at at 8 c/deg. Time-to-peak is shortest at low spatial frequencies. Both PERG and local b-wave are small and positive-going close to the retinal surface, and large and negative-going in the inner nuclear layer. The PERG and b-wave show qualitatively similar depth profiles.     

Temporal aspects of the electroretinogram in cone-rod dystrophy

Twelve patients with cone-rod dystrophy were studied in terms of the temporal aspects of electroretinography (ERG).The peak time of scotopic b-wave was within normal limits in all patients, while the amplitude was reduced in nine patients. The normal peak time of the scotopic-b wave may help explain rod involvement with normal or only slightly elevated final rod thresholds of subjective dark adaptation.The peak time of photopic b-wave and 30-Hz flicker response was normal in four patients and significantly delayed or nonrecordable in eight patients. Such variations of peak time in photopic and 30-Hz flicker ERG may reflect the stage of the disease or may be caused by the different hereditary mode.