Contact lens electroretinography in preterm infants from 32 weeks after conception: a development in current methodology. (49/1146)

AIM: To assess the feasibility of using a contact lens electrode to record the electroretinogram (ERG) in preterm infants less than 35 weeks after conception. METHODS: The ERG was recorded from seven very low birthweight preterm infants on a total of 14 occasions using an infant monkey contact lens electrode. Age at recording the first ERG ranged from 23 to 51 days (gestational age 32-34 weeks), and weight ranged upwards from 1100 g. RESULTS: No complications were observed. With advancing age and maturity the dark adapted rod threshold decreased, indicating increased retinal sensitivity. CONCLUSIONS: Contact lens recording of the ERG from extremely small immature preterm infants is a practicable and well tolerated procedure. This method of recording the ERG will enable further evaluation of retinal development in this vulnerable population.  (+info)

Receptive field organization of ganglion cells in the frog retina: contributions from cones, green rods and red rods. (50/1146)

1. The impulse discharge of ganglion cells was recorded with extracellular micro-electrodes in the excised and opened eye of the common frog, Rana temporaria. 2. When a single unit was isolated, the cell type was first determined according to the Maturana, Lettvin, McCulloch & Pitts (1960) classification with the aid of varying moving and stationary stimuli. 3. Class 4 cells respond only to a decrease of light when cones are stimulated but respond to an increase of light when green rods are stimulated. A distinct class of deviating class 4 cells was found that give a brief high frequency burst at 'off' from their small excitatory receptive fields (ERF); unlike typical class 4 cells they possess a purely inhibitory surrounding field (IRF).4. The contributions from the cones and the green and red rods were isolated by measuring the thresholds of the discharges with on-off stimuli of varying wave-lengths against strong yellow backgrounds, or against a very weak background or no background at all. The spatial distribution of the contributions to the ERF was determined by mapping threshold profiles, and additional information about ERF and IRF was obtained from area-threshold curves. 5. The cone-mediated ERFs were found to be 0-06-0-50 mm wide (1-5-12 degrees of visual field), which agrees well with the sizes of the dendritic trees of the ganglion cells. The green rod-mediated ERFs can be 0-5-1-5 mm wide and have less distinct boundaries than the cone-mediated. The green rod-mediated ERF of an individual ganglion cell is always larger than the cone-mediated ERF of the same cell. The red rod-mediated ERFs seem to be somewhat larger than the cone-mediated but smaller than the green rod-mediated. 6. The green rods contribute only to the on thresholds of class 1, 2 and 4 cells, but both to on and off in typical class 3 cells, while the cones contribute to on and off in classes 1-3 and only to off in class 4.7. When the red rods begin to contribute during dark adaptation they seem to enter the cone but not the green rod channels. 8. All three receptor types contribute to the IRF surrounding the ERF of classes 1, 2, 3 and deviating class 4 cells. Normal class 4 cells have no IRF. 9. The organization of the receptive fields is discussed in relation to the anatomy and electrophysiology of the cell types transmitting the signals from the receptors to the ganglion cells.  (+info)

The development of scotopic sensitivity. (51/1146)

PURPOSE. Test the hypothesis that the developmental increases in rod photoreceptor sensitivity and rod-mediated visual sensitivity at 10 degrees, 20 degrees , and 30 degrees eccentric are concurrent. It is known that maturation of the parafoveal (10 degrees eccentric) rod outer segments and visual sensitivity is delayed compared to that at 30 degrees eccentric. METHODS: Rod isolated electroretinographic (ERG) responses to full-field stimuli were obtained from dark-adapted subjects (n = 71), ranging in age from early infancy through middle age. Rod photoreceptor sensitivity was calculated by fitting a model of the activation of phototransduction to the a-wave response. Rod driven b-wave sensitivity was calculated from stimulus-response functions. A logistic growth model was used to summarize the developmental increases in sensitivity of the rod photoreceptors and the b-wave. Previously reported dark-adapted, rod-mediated visual sensitivities at 10 degrees , 20 degrees, and 30 degrees eccentric, obtained using preferential looking procedures, were reanalyzed using the logistic growth model. RESULTS: The logistic growth model accounted for 57% to 85% of the variance of each sensitivity parameter with age in normal subjects. The shape of the growth curve and the age at which sensitivity reaches 50% of the adult value is similar (10.0-13.5 weeks) for the rods, the b-wave, and peripheral visual sensitivity, but is significantly older, 19.5 weeks, for rod-mediated parafoveal visual sensitivity. CONCLUSIONS: Rod photoreceptor sensitivity and peripheral, rod-mediated visual sensitivity develop concurrently. A parsimonious explanation is that rod photoreceptor sensitivity determines dark-adapted, rod-mediated visual sensitivity during development.  (+info)

The electroretinogram in dogs with inherited cone degeneration. (52/1146)

The electroretinogram (ERG) of hemeralopic Alaskan malamute dogs contains only rod components. There is absence of the photopic b-wave which is normally elicited with red light stimuli during dark adaptation and, using flicker stimulation, only the first or rod branch of the flicker fusion response curve is present. At high stimulus intensity levels, the flicker response of hemeralopes is absent. A normal ERG is recorded from affected dogs using blue light stimuli and low intensity white light. In the adult hemeralope, the retina contains no cones.  (+info)

Night blindness and the retinal mechanism of visual adaptation. (53/1146)

An account is given of investigations into the mechanisms of dark-adaptation in the retina of man and of the skate and other fish. Working hypotheses as to the possible sites of abnormal function in the various disorders of which night blindness is a feature are presented.  (+info)

Disease expression of RP1 mutations causing autosomal dominant retinitis pigmentosa. (54/1146)

PURPOSE: To determine the disease expression in heterozygotes for mutations in the RP1 gene, a newly identified cause of autosomal dominant retinitis pigmentosa (adRP). METHODS: Screening strategies were used to detect disease-causing mutations in the RP1 gene, and detailed studies of phenotype were performed in a subset of the detected RP1 heterozygotes using electroretinography (ERG), psychophysics, and optical coherence tomography (OCT). RESULTS: Seventeen adRP families had heterozygous RP1 changes. Thirteen families had the Arg677ter mutation, whereas four others had one of the following: Pro658 (1-bp del), Ser747 (1-bp del), Leu762-763 (5-bp del), and Tyr1053 (1-bp del). In Arg677ter RP1 heterozygotes, there was regional retinal variation in disease, with the far peripheral inferonasal retina being most vulnerable; central and superior temporal retinal regions were better preserved. The earliest manifestation of disease was rod dysfunction, detectable as reduced rod ERG photoresponse maximum amplitude, even in heterozygotes with otherwise normal clinical, functional, and OCT cross-sectional retinal imaging results. At disease stages when cone abnormalities were present, there was greater rod than cone dysfunction. Patients with the RP1 frameshift mutations showed similarities in phenotype to those with the Arg677ter mutation. CONCLUSIONS: Earliest disease expression of RP1 gene mutations causing adRP involves primarily rod photoreceptors, and there is a gradient of vulnerability of retinopathy with more pronounced effects in the inferonasal peripheral retina. At other disease stages, cone function is also affected, and severe retina-wide degeneration can occur. The nonpenetrance or minimal disease expression in some Arg677ter mutation-positive heterozygotes suggests important roles for modifier genes or environmental factors in RP1-related disease.  (+info)

Metabolic stress reversibly activates the Drosophila light-sensitive channels TRP and TRPL in vivo. (55/1146)

Drosophila transient receptor potential (TRP) is a prototypical member of a novel family of channel proteins underlying phosphoinositide-mediated Ca(2+) entry. Although the initial stages of this signaling cascade are well known, downstream events leading to the opening of the TRP channels are still obscure. In the present study we applied patch-clamp whole-cell recordings and measurements of Ca(2+) concentration by ion-selective microelectrodes in eyes of normal and mutant Drosophila to isolate the TRP and TRP-like (TRPL)-dependent currents. We report that anoxia rapidly and reversibly depolarizes the photoreceptors and induces Ca(2+) influx into these cells in the dark. We further show that openings of the light-sensitive channels, which mediate these effects, can be obtained by mitochondrial uncouplers or by depletion of ATP in photoreceptor cells, whereas the effects of illumination and all forms of metabolic stress were additive. Effects similar to those found in wild-type flies were also found in mutants with strong defects in rhodopsin, Gq-protein, or phospholipase C, thus indicating that the metabolic stress operates at a late stage of the phototransduction cascade. Genetic elimination of both TRP and TRPL channels prevented the effects of anoxia, mitochondrial uncouplers, and depletion of ATP, thus demonstrating that the TRP and TRPL channels are specific targets of metabolic stress. These results shed new light on the properties of the TRP and TRPL channels by showing that a constitutive ATP-dependent process is required to keep these channels closed in the dark, a requirement that would make them sensitive to metabolic stress.  (+info)

Scotopic sensitivity during adulthood. (56/1146)

Older adults typically exhibit about a half log unit loss in scotopic sensitivity that cannot be attributed to optical factors and retinal disease suggesting a neural origin. Little is understood about the developmental course of this neural deficit as to whether it first appears in late life or gradually emerges during the course of adulthood. To address this developmental issue, scotopic sensitivity was measured in 94 adults ranging in age from the 20s to the 80s. Thresholds were measured at 27 test loci within a 18 degrees radius field. Analogous measurements were made for photopic sensitivity. Fundus photography and a grading scale were used to characterize macular health in subjects over age 49 in order to control for macular disease. Scotopic sensitivity decreased at a rate of 0.08 log units per decade; this decline was better fit by a single line model, not a bilinear model, implying that the impairment does not suddenly emerge in late life but gradually appears over the course of adulthood. Photopic sensitivity also decreased in a linear fashion at a rate of 0.04 log units per decade. Under these test conditions, the rate of scotopic sensitivity decline during adulthood was about double the rate of photopic sensitivity decline.  (+info)