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Objective: To compare four screening strategies for diabetic macular edema (DME).Research Design and Methods: Patients attending diabetic retinopathy screening were recruited and received macular optical coherence tomography (OCT), in addition to visual acuity (VA) and fundus photography (FP) assessments, as part of the standard protocol. Two retina specialists provided the reference grading by independently assessing each subject's screened data for DME. The current standard protocol (strategy A) was compared for sensitivity, specificity, quality-adjusted life-year (QALY) gained, and incremental cost-effectiveness ratio (ICER) with three alternative candidate protocols using a simulation model with the same subjects. In strategy B, macular hemorrhage or microaneurysm on FP were removed as surrogate markers for possible DME. Strategy C used best-corrected instead of habitual/pinhole VA and added central subfield thickness (CST) >290 μm on OCT in suspected cases as a confirmation marker for possible DME. Strategy D used CST >290 μm OCT in all subjects as a surrogate marker for suspected DME.Results: We recruited 2,277 subjects (mean age 62.80 ± 11.75 years, 43.7% male). The sensitivities and specificities were 40.95% and 86.60%, 22.86% and 95.63%, 32.38% and 100%, and 74.47% and 98.34% for strategies A, B, C, and D, respectively. The costs (in U.S. dollars) of each QALY gained for strategies A, B, C, and D were $7,447.50, $8,428.70, $5,992.30, and $4,113.50, respectively.Conclusions: The high false-positive rate of the current protocol generates unnecessary referrals, which are inconvenient for patients and costly for society. Incorporating universal OCT for screening DME can reduce false-positive results by eightfold, while improving sensitivity and long-term cost-effectiveness. [ABSTRACT FROM AUTHOR]

Although there have been many population-based studies of age-related macular degeneration (AMD), only limited information is available in Asia on the epidemiology of geographic atrophy (GA). We aimed to determine the prevalence and patterns of GA through an analysis of multiple studies conducted within the Asian Eye Epidemiology Consortium (AEEC). Cross-sectional meta-analyses. A total of 97 213 individuals aged 40 years and older. Data from 22 population-based studies from countries belonging to the AEEC were included. In all studies, AMD was defined on the basis of standardized grading systems. Geographic atrophy was defined as an area of pallor in the fundus with visibility of the underlying choroidal blood vessels and sharply defined borders. Random-effects meta-analysis was performed to estimate overall and age-, gender-, and region-specific pooled prevalence of GA. Prevalence of GA per 1000 persons. The mean age was 60.8 ± 10.0 years, and 42 673 (43.9%) were male. Overall, a total of 223 individuals (0.2%) had GA. The pooled overall prevalence of GA was 1.57 per 1000 persons (95% confidence interval [CI], 1.04–2.10), which was 3 times less than that of neovascular AMD of 5.20 per 1000 persons (95% CI, 3.97–6.43). Compared with those aged 50 to 59 years, the prevalence of GA increased from 0.34 per 1000 persons (95% CI, 0.07–0.62) to 2.90 per 1000 persons (95% CI, 1.55–4.25) in those aged ≥70 years. The GA prevalence per 1000 persons was similar between urban (2.22; 95% CI, 1.22–3.23) and rural residents (1.33; 95% CI, 0.70–1.96). Geographic atrophy was more prevalent in South Asia (based on studies from India and Nepal, 3.82 per 1000 persons; 95% CI, 1.72–5.93) compared with East Asia (based on studies from China, Korea, Hong Kong, Taiwan, and Japan, and the Singapore Chinese Eye Study, 0.76 per 1000 persons; 95% CI, 0.31–1.22, P = 0.005). Geographic atrophy is uncommon in Asian populations compared with those of European ancestry. Even within Asia, geographic differences in GA prevalence were seen. The findings of this meta-analysis suggest that better dissection of risk factors in the Asian population for GA may provide insights into the biological pathways that drive these late-stage manifestations, thus suggesting better targets for prevention. [ABSTRACT FROM AUTHOR]

Purpose: At present, the standard treatment of neovascular age-related macular degeneration (AMD) is the repeated administration of antivascular endothelial growth factor (VEGF) agents. However, we often encounter patients who develop tachyphylaxis for anti-VEGF agents. In this study, we investigated the characteristics of patients who developed tachyphylaxis on repeated intravitreal aflibercept (IVA) injections for neovascular AMD and the frequency of tachyphylaxis. Methods: Three hundred thirteen eyes (313 patients) with treatment-naïve AMD who achieved resolution soon after starting IVA and were followed up for ≥ 12 months were enrolled in this retrospective, interventional, consecutive case series. The eyes were investigated for tachyphylaxis to aflibercept. Tachyphylaxis was defined as absence of any improvement (more than 100 μm) in or worsening of CRT within 1 month after more than two repeated monthly IVA injections when the exudative change remained. Results: Twenty-eight (8.9%) of the 313 eyes developed tachyphylaxis (occult with no classic, n = 14; polypoidal choroidal vasculopathy, n = 14) at an annual rate of about 3%. The mean number of IVA injections was 10.5 ± 7.8, and the mean interval until tachyphylaxis was 20.9 ± 14.0 months. There was a significant difference in the AMD subtypes between the group with tachyphylaxis and the group without it (p = 0.0029). Occult with no classic type and polypoidal choroidal vasculopathy were the only AMD subtypes in the eyes with tachyphylaxis. In the analysis of the eyes that had occult with no classic or polypoidal choroidal vasculopathy, only intraretinal edema was significantly less common (p = 0.042). A combination of photodynamic therapy and aflibercept was effective in 13 (87%) of 15 eyes with tachyphylaxis, and switching to intravitreal ranibizumab was effective in 5 (56%) of 9 eyes. Conclusions: Tachyphylaxis occurs after repeated IVA injections in a minority of patients with AMD for a long term and is more likely to occur in eyes with lesions beneath the retinal pigment epithelium and no intraretinal edema. Treatment of AMD should be performed keeping this fact in mind, while considering the consecutive treatment. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the visual impairment certification status in Japan.Study design: Observational cross-sectional study.Methods: We asked all welfare offices throughout Japan to submit data concerning age, sex, causative diseases, and visual impairment grades for newly certified visually impaired individuals aged ≥ 18 years in the fiscal year of 2015. The certification was based on criteria of the Act on Welfare of Physically Disabled Persons.Results: In total, data were collected for 12,505 newly certified visually impaired individuals. The most common age group for these individuals was 80-89 years (29.6%), followed by 70-79 (26.3%) and 60-69 (17.3%) years. The most common causative disease was glaucoma (28.6%), followed by retinitis pigmentosa (14.0%), diabetic retinopathy (12.8%), and macular degeneration (8.0%). Glaucoma was the most common causative disease in both sexes (30.2% in men and 27.0% in women). The most common impairment grade was grade 2 (31.8%), followed by grades 5 (24.3%) and grade 1 (16.1%). The number of visually impaired individuals with underlying glaucoma had increased in comparison with the number in the most recent surveys (from fiscal years 2007 to 2009), whereas the number of individuals with underlying diabetic retinopathy and macular degeneration had decreased.Conclusion: To our knowledge, this is the first nation-wide complete enumeration survey of newly certified visually impaired individuals in Japan. These findings may contribute to administrative activities concerning medical welfare as well as educational activities for preventing visual impairment. [ABSTRACT FROM AUTHOR]

Age-dependent formation of macular drusen caused by the focal accumulation of extracellular deposits beneath the retinal pigment epithelium precede the development of age-related macular degeneration (AMD), one of the leading causes of blindness worldwide. It is established that inflammation contributes to the pathogenesis of drusen and AMD. However, development of a preemptive therapeutic strategy targeting macular drusen and AMD has been impeded by the lack of relevant animal models because most laboratory animals lack macula, an anatomic feature present only in humans and a subset of monkeys. Reportedly, macular drusen and macular degeneration develop in monkeys in an age-dependent manner. In this study, we analyzed blood test results from 945 Macaca fascicularis, 317 with and 628 without drusen. First, a trend test for drusen frequency (the Cochran–Armitage test) was applied to the quartile data for each parameter. We selected variables with an increasing or decreasing trend with higher quartiles at P < 0.05, to which multivariate logistic regression analysis was applied. This revealed a positive association of age (odds ratio [OR]: 1.10 per year, 95% confidence interval [CI]: 1.07–1.12) and white blood cell count (OR: 1.01 per 1 × 103/μl, 95% CI: 1.00–1.01) with drusen. When the monkeys were divided by age, the association between drusen and white blood cell count was only evident in younger monkeys (OR: 1.01 per 1 × 103/μl, 95% CI: 1.00–1.02). In conclusion, age and white blood cell count may be associated with drusen development in M. fascicularis. Systemic inflammation may contribute to drusen formation in monkeys. [ABSTRACT FROM AUTHOR]

Objective: Diabetic retinopathy (DR) is linked to cardiovascular risk in diabetic patients. This study examined whether mild-stage DR is associated with risk of coronary heart disease (CHD) and stroke in type 2 diabetic patients of the Japan Diabetes Complications Study (JDCS). Design: Prospective cohort study. Participants: In the JDCS, there were 2033 Japanese persons with type 2 diabetes free of cardiovascular diseases at baseline. Methods: Diabetic retinopathy was ascertained from clinical and photographic grading (70%) following the international clinical diabetic retinopathy and diabetic macular edema disease severity scales. Incident CHD and stroke were followed up prospectively annually up to 8 years. Main Outcome Measures: Eight-year incidence of CHD and stroke compared between persons with or without DR. Results: After adjusting for traditional cardiovascular risk factors, persons with mild to moderate nonproliferative DR had a higher risk of CHD (hazard ratio [HR], 1.69; 95% confidence interval [CI], 1.17–2.97) and stroke (HR, 2.69; 95% CI, 1.03–4.86). Presence of retinal hemorrhages or microaneurysms was associated with risk of CHD (HR, 1.63; 95% CI, 1.04–2.56) but was not associated with stroke (P = 0.06). Presence of cotton-wool spots was associated with risk of incident stroke (HR, 2.39; 95% CI, 1.35–4.24) but was not associated with CHD (P = 0.66). When information about DR was added in the prediction models for CHD and stroke based on traditional cardiovascular risk factors, the area under the receiver operating curve improved from 0.682 to 0.692 and 0.640 to 0.677, and 9% and 13% of persons were reclassified correctly for CHD and stroke, respectively. Conclusions: Type 2 diabetic patients with even a mild stage of DR, such as dot hemorrhages, are already at higher risk of CHD and stroke independent of traditional risk factors. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

A bstract Background: To describe the changes in retinal vascular calibre in response to intravitreal ranibizumab injections in patients with neovascular age-related macular degeneration. Design: Prospective interventional case series. Participants: Treatment naïve patients with neovascular age-related macular degeneration were recruited over a 1-year period. Methods: Each patient received three monthly intravitreal injections according to a 'loading dose'. Retinal arteriolar and venular calibre was measured from digital fundus photographs and summarized as central retinal artery equivalent and central retinal vein equivalent at baseline and 3 months. Main Outcome Measure: Central retinal artery equivalent and central retinal vein equivalent changes from baseline to 3 months. Results: Seventy-four eyes of 71 patients had good quality images for grading vessel calibre at baseline and at 3 months in treated (study) eyes and 51 eyes of 51 patients had good quality images in fellow (control) eyes. Over 3 months, in study eyes treated with ranibizumab, there was a significant increase in central retinal vein equivalent over baseline (+6.20 µm, P = 0.005), but no significant change in central retinal artery equivalent (+0.86 µm, P = 0.55). In control eyes, there was no change in central retinal vein equivalent (−0.82 µm, P = 0.70) or central retinal artery equivalent (0.34 µm, P = 0.75). Conclusion: Intravitreal ranibizumab has a significant vasodilational effect on retinal venular calibre in eyes treated for neovascular age-related macular degeneration. The reason for this change is unclear, but may relate to changes in blood flow or inflammatory changes within the retina. [ABSTRACT FROM AUTHOR]

Purpose: To compare the frequency and pattern of bilateral involvement of early and late age-related macular degeneration (AMD) between Asian Malays and Caucasians. Methods: We used cross-sectional data from the baseline examination for subjects aged 50-79 years in the Singapore Malay Eye Study (SiMES) ( N == 2,453) and the Blue Mountains Eye Study (BMES) ( N == 3,265). We assessed AMD signs using a common protocol modified from the Wisconsin Age-related Maculopathy Grading System at the University of Sydney. We compared frequencies or proportions of AMD cases with bilateral involvement between the two populations. Results: There were 173 cases and 169 cases with any AMD (either early or late AMD in at least one eye), and 78 cases (45.1%) and 52 cases (30.8%) with bilateral AMD in the BMES and the SiMES, respectively. Age-standardized frequency of bilateral involvement was comparable between the BMES (29.5%, 95% confidence interval(CI) 18.5-40.5%) and the SiMES (25.6%, 95%CI 17.0-34.0%). Older age was associated with higher risk of bilateral AMD (gender-adjusted odds ratio per 1 year for the BMES and the SiMES: 1.08 [95% CI 1.05-1.11] and 1.06 [95% CI 1.02-1.10], respectively). Conclusions: The frequency of bilateral AMD was comparable between Asian Malays in the SiMES and the Caucasian population of the BMES. Other than older age, we did not find any characteristics associated with the bilateral involvement of AMD. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the subfoveal choroidal thickness in eyes with typical age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy (PCV), using enhanced depth imaging optical coherence tomography. Methods: Retrospective observational case series of 44 eyes of 44 patients (12 females and 32 males) with typical AMD or PCV located in the subfoveal region. Cross-sectional images of the choroid of each of the involved eyes were obtained by a spectral-domain OCT. The choroidal thickness under the fovea was retrospectively studied. Results: Of the 44 eyes involved in this study, 21 eyes were diagnosed as typical AMD and the other 23 eyes were diagnosed as PCV. The difference in subfoveal choroidal thickness between the eyes with typical AMD (245 μm) and those with PCV (293 μm) was statistically significant, even after adjusting for age, spherical equivalent, and gender distribution ( P = 0.045). When compared to eyes with subfoveal choroidal thickness less than 300 μm, those with subfoveal choroidal thickness of 300 μm or more were 5.6 times more likely to have PCV (adjusted odds ratio 5.60, 95% confidence interval 1.30-24.0, P = 0.021). Conclusions: The choroid under the fovea was thicker in eyes with PCV than those with typical AMD. This result suggests that the choroidal vascular lesion seen in PCV may not be just the choroidal neovascularization accompanied by saccular capillary dilations at the border, but may have a significant structural difference in the choroid compared to typical AMD. [ABSTRACT FROM AUTHOR]

Objective: To determine the prevalence of age-related macular degeneration (AMD) in Asian populations and to compare this with prevalence in white populations. Design: A clear understanding of AMD prevalence in Asians is essential to meet future demands for eye health care. Methods: We searched published literature reporting AMD prevalence in Asian populations. We limited studies examined to those using standardized grading systems (either the Wisconsin Age-Related Maculopathy Grading System or the international classification proposed by the International ARM Epidemiological Study Group). We used metaanalytical methods to calculate age-specific pooled prevalence of AMD using inverse-variance weighting in a random effect model. We also calculated pooled estimates of age-standardized prevalence. A metaregression model was used to examine gender differences and differences between Asian and white populations. Results: We identified 9 studies reporting AMD prevalence from 4 Asian populations. Pooled prevalence estimates of early and late AMD in Asian populations aged 40 to 79 years were 6.8% (95% confidence interval [CI], 4.6%–8.9%) and 0.56% (95% CI, 0.30%–0.81%), respectively; corresponding prevalence estimates in white populations were 8.8% (95% CI, 3.8%–13.8%) and 0.59% (95% CI, 0.35%–0.84%), respectively. Reliable prevalence estimates of AMD in Asian persons aged ≥80 years were not available owing to small subject numbers in this age category. Conclusions: Among persons aged 40 to 79 years, the age-specific prevalence of late AMD in Asians was comparable with that reported from white populations, but early AMD signs were less common among Asians. Further studies in Asian populations are warranted to investigate whether certain specific AMD phenotypes or subtypes, such as polypoidal choroidal vasculopathy, are more common. Financial Disclosure(s): Proprietary or commercial disclosure may be found after the references. [Copyright &y& Elsevier]

Purpose: Dilation of retinal vessels in response to diffuse luminance flicker may reflect endothelial function. Although this has previously been shown to be reproducible in whites, there have been no similar data in Asians. We assess the reproducibility of repeated measurements of this response in Asians. Material and Methods: Healthy Asians ( n = 33) with normal vision and no history of glaucoma, age-related macular degeneration, cataract, or retinal arterial/venous occlusion participated in this study. Repeated measures from the same subjects were taken 30–60 min apart using the Dynamic Vessel Analyser (DVA, IMEDOS, Jena, Germany). Modification was made to the shape of the light source for Asian participants. Correlations of the first and second measures were assessed using Pearson correlation ( R2), and agreement between the two measures was shown using Bland–Altman plots. Results: After modification to the shape of the light source, almost perfect correlation was found between the 1st and 2nd measurements of baseline arteriolar ( R2 = 0.95) and venular diameters ( R2 = 0.98) of arteriolar maximum dilation ( R2 = 0.85). Substantially high correlation between the 1st and 2nd measurements of venular maximum dilation was found ( R2 = 0.80). Conclusions: Measurements of the dilation response of retinal vessels to diffuse luminance flicker an Asian sample using the DVA show high reproducibility for repeated measures over a short period of time. Such measurements may allow non-invasive quantification of endothelial function to study its association with systemic and ocular diseases. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the relationship between retinal vascular caliber and age-related macular degeneration (AMD). Design: Population-based cross-sectional study. Methods: Three thousand two hundred and eighty (78.7% response rate) Malay Singaporeans aged 40 to 80 years residing in 15 districts of Singapore underwent retinal photography. Retinal vessel caliber was measured from retinal photographs using a validated computer-based technique. AMD was assessed following a modified Wisconsin Age-Related Maculopathy Grading System. Results: Retinal data were available from 3,265 subjects (99.5% of 3,280) for this study. Early and late AMD prevalence were 4.9% (n = 160) and 0.7% (n = 23) of the population, respectively, or in 205 (3.1%) and 30 (0.5%) eyes examined, respectively. After controlling for age and arteriolar caliber, wider venular caliber was associated with higher prevalence of early AMD (odds ratio [OR] per one standard deviation [SD] increment in venular caliber, 1.53; 95% confidence interval [CI], 1.13 to 2.09). This association remained significant after further adjustment for gender, smoking, hypertension, diabetes, and body mass index (OR per one SD, 1.52; 95% CI, 1.11 to 2.09). There was no significant association between retinal arteriolar caliber and early AMD, or between arteriolar or venular caliber and late AMD. Conclusions: Wider venular caliber was associated independently with early AMD. This finding may suggest that pathogenic processes linking to wider venular caliber be shared by early AMD and common cardiovascular risk factors such as inflammation, dyslipidemia, and endothelial dysfunction. [Copyright &y& Elsevier]

Objective: To describe the prevalence of age-related macular degeneration (AMD) in an Asian Malay population. Design: Population-based cross-sectional study. Participants: An age-stratified random sample of Malay persons aged 40 to 80 years living in Singapore. Methods: Participants were invited to a central clinic for a comprehensive examination. Main Outcome Measures: Early and late AMD signs were graded from retinal photographs following the Wisconsin grading system. Results: Of 3280 participants who participated (78.7% response rate), 3265 had photographs of sufficient quality for grading of AMD signs. Early and late AMD were present in 160 (4.9%) and 23 (0.70%) participants, respectively. After age standardization, the prevalence of early AMD in Malay persons aged 40 to 80 years was estimated to be 3.5% (95% confidence interval [CI], 2.9%–4.1%) and that of late AMD was 0.34% (95% CI, 0.20%–0.49%). Early AMD was more prevalent in men than in women (6.1% vs. 3.8%); this was significant despite adjusting for age and smoking (odds ratio [OR], 1.56; 95% CI, 1.11–2.20). Late AMD also was more prevalent in men than in women (1.0% vs. 0.4%), although this was not statistically significant after adjusting for age and smoking (OR, 1.39; 95% CI, 0.52–3.68). The prevalence of early and late AMD was similar to that reported in the Blue Mountains Eye Study among white persons. Conclusions: The prevalence of AMD in Asian Malay people is similar to that in white persons from the Blue Mountains Eye Study. Early AMD signs were more frequent in men compared with women, an association that was not fully explained by the higher smoking rates in men. Financial Disclosure(s): The authors have no proprietary or commercial interest in any materials discussed in this article. [Copyright &y& Elsevier]

Objective: To describe the prevalence and risk factors for age-related macular degeneration (AMD) in a Japanese population and to compare these with data from a white population. Design: Population-based cross-sectional epidemiologic study. Participants: A population-based sample of Japanese persons 35 years or older from Funagata, Japan. Methods: The Funagata study is a population-based study of 1758 (43% of eligible) Japanese persons 35 years or older from Funagata, Japan. In 2000 to 2002, 1625 (92.4%) participants had a nonmydriatic fundus photograph of one eye with sufficient quality for grading of AMD lesions, using the Wisconsin protocol. Age-standardized prevalence rates compared with the Blue Mountains Eye Study (BMES) population, odds ratios (ORs), and 95% confidence intervals (CIs) were calculated. Risk factors were assessed by logistic regression. Main Outcome Measures: Early and late AMD. Results: Of 1625 participants, early AMD and late AMD were present in 3.5% and 0.5%, respectively. Age-standardized early AMD prevalence in right eyes was 4.1%, similar to the corresponding prevalence of 4.4% in the BMES. For men, age-standardized prevalences of late AMD in right eyes were 1.1% and 1.2% in the BMES; for women, the corresponding prevalences were 0.3% and 2.1%, respectively. Increasing age (per 10 years; gender-adjusted OR, 2.27; 95% CI, 1.10–4.67) and current cigarette smoking (age- and gender-adjusted OR, 5.03; 95% CI, 1.00–25.47) were associated with late AMD. Conclusions: In this Japanese population, prevalence of early AMD was similar to that for whites in the BMES. Although the late AMD prevalence was lower in Japanese women, in Japanese men it was similar to that in whites. This could have resulted from the substantially high proportion of Japanese men who are smokers. Cigarette smoking and increasing age were the 2 principal factors found associated with late AMD. [Copyright &y& Elsevier]

This article presents a sub-analysis of an age-related maculopathy statin study (ARMSS) data to examine the impact of statins on retinal vascular caliber changes compared to placebo treatment. The ARMSS examined the effect of simvastatin intake versus placebo in preventing the age-related macular degeneration progression. It concludes that statins may contribute to the prevention or management of retinal vascular diseases related to cardiovascular risks and inflammation.

To present phenotypic features of 22 patients with S-antigen (SAG) mutations. Retrospective cohort study. Twenty-one Japanese patients from 16 families with a homozygous c.924delA mutation and 1 patient with a homozygous c.636delT mutation in the SAG gene. Clinical records on symptoms; best-corrected visual acuity; and Goldmann perimetry, fundus photography, fundus autofluorescence (FAF), OCT, and electroretinography results were reviewed. Best-corrected visual acuity, Goldmann perimetry results, imaging findings, and electroretinography results. Ten patients had Oguchi disease and 12 had retinitis pigmentosa (RP) with mean follow-up periods of 13.8 and 10.2 years, respectively. Retinitis pigmentosa patients were older (mean age, 56.0 years) than those with Oguchi disease (mean age, 22.1 years; P < 0.001) at the initial visit. Night blindness noted in childhood was the most common initial symptom for both Oguchi disease (80.0%) and RP (91.7%) patients. Best-corrected visual acuity in the logarithm of the minimum angle of resolution (logMAR) was well preserved in Oguchi disease patients (mean, 0.02 logMAR in both eyes) but reduced in most RP patients (mean, 1.32 logMAR [right eye] and 1.35 logMAR [left eye]). Similarly, the visual field in the retinal area was preserved in Oguchi disease patients (mean, 677 mm2 right eye and 667 mm2 left eye) and reduced in RP patients (mean, 369 mm2 right eye and 294 mm2 left eye). Fundus images revealed a characteristic golden sheen with no retinal degeneration in Oguchi disease patients, excluding 2 with macular degeneration detected by FAF, OCT, or both and 1 with mild retinal degeneration confirmed by OCT and fluorescein angiography. Pigmentary retinal degeneration most evident posteriorly was observed in RP patients, accompanied by a characteristic golden sheen in 12 of 14 patients undergoing ultra-widefield fundus imaging. OCT showed disrupted macular structure, and FAF revealed variable hypofluorescence. Electroretinography identified absent rod responses in both diseases, along with relative preservation of cone responses in Oguchi disease patients. Three patients showed progressive loss of the golden sheen based on fundus images, including 1 who demonstrated RP 26 years after the initial diagnosis of Oguchi disease. Retinitis pigmentosa with SAG mutations often shows a characteristic golden sheen surrounding posterior pigmentary retinal degeneration. Oguchi disease can show progressive degeneration in adulthood, rarely resulting in RP. [ABSTRACT FROM AUTHOR]