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Chlorogenic Acid Supplementation Improves Multifocal Electroretinography in Patients with Retinitis Pigmentosa
摘要： To evaluate the effect of chlorogenic acid supplementation in patients with retinitis pigmentosa, we evaluated objective change in visual function with multifocal electroretinography, along with visual acuity, visual field, standard electroretinography, and contrast sensitivity. Eighteen patients diagnosed with retinitis pigmentosa were enrolled in this prospective, non-comparative, single-arm study. Multifocal electroretinography, best-corrected visual acuity in Early Treatment Diabetic Retinopathy Study letters, total point score on visual field examination with Humphrey Field Analyzer II, electroretinography, and contrast sensitivity were measured and repeated after 3 months supplementation with chlorogenic acid. The amplitude of ring 5 was significantly higher on multifocal electroretinography after 3 months of chlorogenic acid supplementation (7.2 ± 9.5 vs 8.3 ± 10.8 nV/deg2, mean ± standard deviation, P = 0.022). There were no significant changes in the best-corrected visual acuity, total point score on Humphrey Field Analyzer, 30 Hz flicker amplitude on standard electroretinography, or contrast sensitivity. Chlorogenic acid may have a beneficial effect on the peripheral area at the margins of retinal degeneration, and should be considered as an anti-oxidant for the management of retinitis pigmentosa.
关键词： Retinitis Pigmentosa,Oxidative Stress,Chlorogenic Acid,Retinal Degeneration,Antioxidants
更新于2025-09-23 15:22:29
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[Essentials in Ophthalmology] Advances in Vision Research, Volume II (Genetic Eye Research in Asia and the Pacific) || Genetics and Susceptibility of Retinal Eye Diseases in India
摘要： As per the World Health Organization, genetic eye disorders are one of the top ten major causes of global ocular health burden. AMD and DR take major share of the adult eye diseases component that particularly affects the neurovascular retina. More than 100 genes are known to cause Mendelian types of retinal degenerations including syndromic and non-syndromic RP, and it is presumed that this constitutes only 60% of all the genes known so far, and the remaining are yet to be identified. The burden of genetic disorders in India is significant, and very many significant genes like RPE65 have been identified with consanguineous autosomal recessive pedigrees obtained from this region.
关键词： Visual cycle,Phototransduction pathway,Stargardt disease,Retinitis pigmentosa,Leber congenital amaurosis
更新于2025-09-23 15:21:21
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Outer Retinal Changes Including the Ellipsoid Zone Band in Usher Syndrome 1B due to <i>MYO7A</i> Mutations
摘要： PURPOSE. To study transition zones from normal to abnormal retina in Usher syndrome IB (USH1B) caused by myosin 7A (MYO7A) mutations. METHODS. Optical coherence tomography (OCT) scattering layers in outer retina were segmented in patients (n ? 16, ages 2–42; eight patients had serial data, average interval 4.5 years) to quantify outer nuclear layer (ONL) and outer segments (OS) as well as the locus of EZ (ellipsoid zone) edge and its extent from the fovea. Static perimetry was measured under dark-adapted (DA) and light-adapted (LA) conditions. RESULTS. Ellipsoid zone edge in USH1B-MYO7A could be located up to 238 from the fovea. Ellipsoid zone extent constricted at a rate of 0.518/year with slower rates at smaller eccentricities. A well-de?ned EZ line could be associated with normal or abnormal ONL and/or OS thickness; detectable ONL extended well beyond EZ edge. At the EZ edge, the local slope of LA sensitivity loss was 2.6 (61.7) dB/deg for central transition zones. At greater eccentricities, the local slope of cone sensitivity loss was shallower (1.1 6 0.4 dB/deg for LA) than that of rod sensitivity loss (2.8 6 1.2 dB/deg for DA). CONCLUSIONS. In USH1B-MYO7A, constriction rate of EZ extent depends on the initial eccentricity of the transition. Ellipsoid zone edges in the macula correspond to large local changes in cone vision, but extramacular EZ edges show more pronounced losses on rod-based vision tests. It is advisable to use not only the EZ line but also other structural and functional parameters for estimating natural history of disease and possible therapeutic effects in future clinical trials of USH1B-MYO7A.
关键词： myosin 7A,retinitis pigmentosa,optical coherence tomography,photoreceptors
更新于2025-09-23 15:21:21
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Management of cystoid macular edema secondary to retinitis pigmentosa via subliminal micropulse yellow laser
摘要： To investigate the effects of subliminal micropulse yellow laser application on the central macular thickness and best-corrected visual acuity in cystoid macular edema secondary to retinitis pigmentosa patients. This prospective open-label clinical trial, conducted between January 2018 and October 2019, included 32 eyes of 29 patients who had cystoid macular edema secondary to retinitis pigmentosa. Patients were treated by subliminal micropulse yellow laser for one session. Central macular thickness and best-corrected visual acuity changes were investigated just before the treatment and 1 year later after the one session of the treatment. The mean central macular thickness was 651.3 μm before the treatment and 247.7 μm at 12 months after the treatment. The decrease in mean central macular thickness was statistically significant (p = 0.01). Median best-corrected visual acuity was 66.8 ETDRS letters before the treatment and 70.0 letters at 12 months after the treatment. The increase in best-corrected visual acuity was not statistically significant (p = 0.18). Eighty-six percent of the patients stated that the quality of central vision increased and that color vision, contrast sensitivity, and distortion improved. We did not encounter any serious adverse events related to the application of subliminal micropulse yellow laser. The subliminal micropulse yellow laser seems to be a therapeutic, effective, and safe option for the treatment of non-inflammatory and resistant cystoid macular edema secondary to retinitis pigmentosa patients. ClinicalTrials.gov ID: NCT04234438, January 17, 2020.
关键词： Intraretinal cysts,Retinitis pigmentosa,Subliminal micropulse laser,Cystoid macular edema
更新于2025-09-23 15:21:01
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Clinically Relevant Outcome Measures for the I307N Rhodopsin Mouse: A Model of Inducible Autosomal Dominant Retinitis Pigmentosa
摘要： PURPOSE. The I307N rhodopsin (Rho) mouse is a light-inducible model of autosomal dominant retinitis pigmentosa (adRP) that may be useful in testing therapies. We investigated the time-course of retinal changes of the I307N Rho mouse with spectral-domain optical coherence tomography (SD-OCT). METHODS. SD-OCT was performed up to day 30 after light damage; electroretinography (ERG) was employed to evaluate photoreceptor function. We utilized ImageJ to analyze reflectivity of the retina. We used light and electron microscopy to assess retinal organization. We stained synaptophysin and zonula occludins-1 with immunohistochemistry to determine injury to the plexiform layers and retinal pigment epithelium (RPE). We performed lectin staining to evaluate retinal blood vessels. RESULTS. Retinal degeneration increased with longer exposures to light. An increase in retinal thickness was detected by SD-OCT on day 1 after light challenge followed by loss of the outer nuclear layer (ONL) by day 8. Degeneration was most severe in the nasal and inferior retina. Hyper-reflectivity on SD-OCT developed as early as 1 day after light exposure. Disorganization of the ONL, condensation of photoreceptor chromatin, disruption of the outer limiting membrane, and disarray of outer segments were associated with the hyper-reflectivity. Retraction of the outer plexiform synapses and resorption of the subretinal detachment contributed to retinal thinning. The RPE remained intact, whereas atrophied major retinal vessels were evident after light damage. CONCLUSIONS. Our time-course analysis of retinal degeneration in the I307N Rho mouse with SD-OCT and other outcome measures should enable the use of the mouse model in preclinical efficacy studies and mechanistic studies.
关键词： SD-OCT,rhodopsin,electroretinography,retinitis pigmentosa,hyper-reflectivity
更新于2025-09-23 15:21:01
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Mechanisms Underlying the Visual Benefit of Cell Transplantation for the Treatment of Retinal Degenerations
摘要： The transplantation of retinal cells has been studied in animals to establish proof of its potential benefit for the treatment of blinding diseases. Photoreceptor precursors have been grafted in animal models of Mendelian-inherited retinal degenerations, and retinal pigmented epithelial cells have been used to restore visual function in animal models of age-related macular degeneration (AMD) and recently in patients. Cell therapy over corrective gene therapy in inherited retinal degeneration can overcome the genetic heterogeneity by providing one treatment for all genetic forms of the diseases. In AMD, the existence of multiple risk alleles precludes a priori the use of corrective gene therapy. Mechanistically, the experiments of photoreceptor precursor transplantation reveal the importance of cytoplasmic material exchange between the grafted cells and the host cells for functional rescue, an unsuspected mechanism and novel concept. For transplantation of retinal pigmented epithelial cells, the mechanisms behind the therapeutic benefit are only partially understood, and clinical trials are ongoing. The fascinating studies that describe the development of methodologies to produce cells to be grafted and demonstrate the functional benefit for vision are reviewed.
关键词： retinitis pigmentosa,photoreceptors,retinal pigmented epithelium,age-related macular degeneration,cytoplasmic material transfer,induced-pluripotent stem cells
更新于2025-09-19 17:15:36
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Progesterone anti-inflammatory properties in hereditary retinal degeneration
摘要： The interactions between steroid gonadal hormones and the retina (a part of the visual system and the central nervous system (CNS)) have received limited attention and beneficial effects of these hormones in retinal diseases is controversial. Retinitis pigmentosa (RP) is the most common cause of retinal hereditary blindness and to date no treatment is available. However, results regarding the effects of progesterone on the progression of RP are promising. With the idea of demonstrating if the progesterone retinal protection in RP is related to its possible anti-inflammatory properties, we have administered orally progesterone to rd10 mice, an animal model of RP. We observed that progesterone decreased photoreceptors cell death, reactive gliosis and the increase in microglial cells caused by RP. We also examined the expression of neuronal and inducible nitric oxide synthase (nNOS and iNOS), the enzyme responsible for NO production. The results demonstrated a decrease in nNOS expression only in control mice treated with progesterone. Inflammation has been related with an increase in lipid peroxidation. Noticeably progesterone administration was able to diminish retinal malondialdehyde (MDA, a lipid peroxidation product) concentrations in rd10 mice. Altogether, we can conclude that progesterone could be a good therapeutic option not only in RP but also for other retinal diseases that have been associated with inflammation and lipid peroxidation.
关键词： retinitis pigmentosa,glia,malondialdehyde,inflammation,progesterone
更新于2025-09-19 17:15:36
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Electrical activation of degenerated photoreceptors in blind mouse retina elicited network-mediated responses in different types of ganglion cells
摘要： Electrical (e-) stimulation is explored in schemes to rescue the vision of blind people, e.g. those affected by Retinitis Pigmentosa (RP). We e-activated subretinally the surviving degenerated photoreceptors (d-Phrs) of the rd1 mouse (RP model) and evoked visual responses in the blind retina. The e-stimulation was applied with a single platinum/iridium electrode. The d-Phrs (calcium-imaging) and ganglion cells (GC) activity (MEA-recording) were recorded in simultaneous multilayer recordings. The findings of this study confirm that the d-Phrs responded to e-stimulation and modulated the retinal network-activity. The application of blockers revealed that the synaptic interactions were dependent on voltage-gated calcium channels and mediated by the transmitters glutamate and GABA. Moreover, the gap junctions coupled networks promoted the lateral-spread of the e-evoked activity in the outer (~60 μm) and inner (~120 μm) retina. The activated GCs were identified as subtypes of the ON, OFF and ON-OFF classes. In conclusion, d-Phrs are the ideal interface partners for implants to elicit enhanced visual responses at higher temporal and spatial resolution. Furthermore, the retina’s intact circuity at the onset of complete blindness makes it a tempting target when considering the implantation of implants into young patients to provide a seamless transition from blinding to chip-aided vision.
关键词： blind retina,MEA recording,subretinal implant,gap junctions,glutamate,rd1 mouse,calcium imaging,ganglion cells,GABA,electrical stimulation,Retinitis Pigmentosa,degenerated photoreceptors
更新于2025-09-19 17:15:36
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Retinotopic Responses in the Visual Cortex Elicited by Epiretinal Electrical Stimulation in Normal and Retinal Degenerate Rats
摘要： Electronic retinal prostheses restore vision in people with outer retinal degeneration by electrically stimulating the inner retina. We characterized visual cortex electrophysiologic response elicited by electrical stimulation of retina in normally sighted and retinal degenerate rats. rats were used to map cortical responses elicited by epiretinal electrical stimulation in four quadrants of the retina. Six normal and six rd rats were used to compare the dendritic spine density of neurons in the visual cortex. Results: The rd rats required higher stimulus amplitudes to elicit responses in the visual cortex. The cortical electrically evoked responses (EERs) for both healthy and rd rats show a dose-response characteristic with respect to the stimulus amplitude. The EER maps in healthy rats show retinotopic organization. For rd rats, cortical retinotopy is not well preserved. The neurons in the visual cortex of rd rats show a 10% higher dendritic spine density than in the healthy rats. Conclusions: Cortical activity maps, produced when epiretinal stimulation is applied to quadrants of the retina, exhibit retinotopy in normal but not rd rats. This is likely due to a combination of degeneration of the retina and increased stimulus thresholds in rd, which broadens the activated area of the retina. Translational Relevance: Loss of retinotopy is evident in rd rats. If a similar loss of retinotopy is present in humans, retinal prostheses design must include flexibility to account for patient specific variability.
关键词： cortical electrophysiology,retinitis pigmentosa,retinotopy,electrical stimulation,rat
更新于2025-09-19 17:15:36
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A review on the current status of retinal prostheses (bionic eye); Pregled informacija o trenutnom stanju retinalnih proteza (bioni?ko oko);
摘要： Argus? II, the first retinal prosthesis is approved for the treatment of patients with retinal pigmentosis. 2011 has received the CE (European Committee) designation and 2013 FDA (Food and Drug Administration) Approval. Retinal prosthesis provides a stable, safe and long-lasting retinal stimulation. The improvement of vision with retinal prosthesis depends on the improvement of the visual resolution, development of the correct neural form for imaging and improvement of the biological compatibility of the device to ensure durability. Once the theme of folklore and science fiction, the idea of returning sight to blind people today is approaching reality.
关键词： retinitis pigmentosa,visual prosthesis,retinal prosthesis
更新于2025-09-16 10:30:52