A slow-release dexamethasone implant for cancer-associated retinopathy / Implante de liberacao lenta de dexametasona para retinopatia associada ao cancer

ABSTRACT This case report aims to show the anatomical and functional results of a patient diagnosed as having cancer-associated retinopathy treated with a controlled-release dexamethasone implant (Ozurdex). Anatomical outcomes were assessed using spectral domain optical coherence tomography; and functional outcomes, by measuring visual acuity, microperimetry, and mutifocal electroretinography. The follow-up period was 1 year.

RESUMO Este relato de caso tem como objetivo mostrar os resultados anatômicos e funcionais de um paciente com diagnóstico de retinopatia associada ao câncer tratado com implante de liberação controlada de dexametasona (Ozurdex®). Os resultados anatômicos foram avaliados por SD-OCT e os resultados funcionais por medida de acuidade visual, microperimetria e eletrorretinograma multifocal. O período de acompanhamento foi de um ano.

Electrophysiological detection of visible wavelengths of artificial lights inducing take-off in adults of Rhodnius prolixus (Hemiptera: Triatominae)

ABSTRACT Rhodnius prolixus is the most important vector of Trypanosoma cruzi in the northern part of South America. The compound eyes in adults of R. prolixus are involved in the nocturnal flight dispersion from sylvatic environments into human dwellings. During this behavior, the artificial lights play an important role in attracting R. prolixus; however, it is still not clear whether the compound eyes of this species use different visible wavelengths as a cue during active dispersion. We applied electrophysiological (electroretinography or ERG) and behavioral (take-off) experiments in a controlled laboratory setting to determine the spectral sensitivity of the compound eyes and the attraction of R. prolixus adults to discrete visible wavelengths. For the ERG experiments, flashes of 300 ms at wavelengths ranging between 350 and 700 nm at a constant intensity of 3.4 µW/cm2 were tested after adaptation to darkness and to blue and yellow lights. For the behavioral experiments, the adults were exposed to nine visible wavelengths at three different intensities, and their direction of take-off in an experimental arena was established with circular statistics. The ERG results showed peaks of spectral sensitivity at 470-490 nm and 520-550 nm in adults, while behavioral experiments showed attractions to blue, green and red lights, depending on the intensity of the light stimuli. The electrophysiological and behavioral results confirm that R. prolixus adults can detect certain wavelengths in the visible spectrum of light and be attracted to them during take-off.

Research status and prospect on the cerebral vision in myopic patients / 中华实验眼科杂志

The visual signal generated by the eye receiving external light stimulation reaches the visual center through the visual pathway and is processed and integrated by the visual center to form a subjective sensation called cerebral vision.Cerebral vision reflects the function of the whole visual nervous system from the retina to the visual cortex.Abnormal cerebral vision has been found in myopes using electrophysiology, magnetic resonance imaging (MRI) and functional near-infrared spectroscopy (fNIRS). The abnormal electrophysiological results are mainly found in patients with high myopia and pathological myopia.MRI shows abnormal changes in the structure and function of several vision-related brain regions in patients with high myopia.Recently, by applying near-infrared brain function imaging technology combined with self-developed equipment, our team conducted a series of studies on the brain function of myopic patients and found that adult patients with moderate myopia and patients with pathological myopia have the abnormal cerebral vision.The mechanism of neural regulation of eye accommodation in myopic children needs further investigation.In this article, we reviewed the current status and outlook of myopic brain vision research in terms of myopia classification, myopic brain vision research methods, and myopic visual electrophysiology, MRI, and fNIRS studies.

Rescue of photoreceptor with human mesenchyme stem cell and human mesenchyme stem cell expressing erythropoietin in total degeneration of retina animal model

Purpose: This study aimed to investigate the efficacy of human?derived umbilical cord mesenchymal stem cells (HDUMSC) and human?derived umbilical cord mesenchymal stem cells expressing erythropoietin (HDUMSC?EPO) to rescue total degenerated retina in a rat model. Methods: The study included four treatment groups, namely negative control using normal saline (HBSS) injection, positive control using sodium iodide 60 mg/kg (SI), SI treated with HDUMSC, and SI treated with HDUMSC?EPO given via subretinal and intravenous routes, to test the efficacy of retinal regeneration following SI?induced retinal degeneration. Retinal function in both phases was tested via electroretinography (ERG) and histological staining examining the outer nuclear layer (ONL). Results: There was a statistically significant result (P < 0.05) in the SI treated with HDUMSC?EPO only when comparing day 11 (mean = 23.6 ?v), day 18 (mean = 25.2 ?v), day 26 (mean = 26.3 ?v), and day 32 (mean = 28.2 ?v) to the b?wave ERG on day 4 rescue injection day (mean = 12.5 ?v). The SI treated with HDUMSC?EPO showed significant improvement in b?wave ERG readings in the Sprague–Dawley (SD) rat but did not restore baseline readings prior to degeneration (day 0). Both treated groups’ ONL thicknesses did not show significant changes compared to the negative control group (HBSS) following rescue therapy. Conclusion: Total retinal degeneration following intravenous SI injection was observed at 60 mg/kg. SI treated with HDUMSC and HDUMSC?EPO showed no regenerative potential compared to baseline in SI?induced total retina degeneration on ERG or histology, whereas SI treated with HDUMSC?EPO group showed a substantial increase in b?wave ERG amplitude over time

Preliminary study of rabbits as an animal model of mammalian eye transplantation and literature review / Estudo preliminar de coelhos como modelo animal para transplante de olho em mamíferos e revisão da literatura

ABSTRACT Purpose: To describe an innovative animal model of eye transplantation used in rabbits. Methods: six Dutch-belted male rabbits were submitted to lateral orbitotomy in the right eye, wide retrobulbar anatomy exposure, dissection of the structures, identification and distal section of the optic nerve followed by anastomosis either by vicryl (group 1) or fibrin glue (group 2). Electroretinography recording was performed before the section of the optic nerve and every 30 seconds after, to monitor the function of retina. Left eye was used as control group. Results: After optic nerve resection and anastomosis, stable ERG amplitude of the right eye was lost after 302 seconds in group 1 and after 296 seconds on group 2. Left eye kept longer stable ERG amplitude curves. Conclusions: The animal model of whole eye transplantation was effective in describing a novel technique to be used in rabbits, with success of the anatomic procedure. Further studies will clarify the best anastomosis methods and maintenance of function of the receptor organ. Translational relevance: this animal model of whole eye transplantation provides a novel perspective for blind patients and the research models, since we describe a novel mammal animal model. This model can be used as basis of a human model of whole eye transplantation in future studies.

RESUMO Objetivo: Descrever uma técnica cirúrgica inovadora para transplante de olho em um modelo animal em coelhos. Métodos: Seis coelhos machos com Dutch Belted foram submetidos à orbitotomia lateral do olho direito, com ampla exposição da anatomia retrobulbar, dissecção do cone muscular, exposição e secção distal do nervo óptico seguida de anastomose por vicryl (Grupo 1) ou cola de fibrina (Grupo 2). O registro da eletrorretinografia foi realizado antes da secção do nervo óptico e a cada 30 segundos após, para monitorar a função da retina. O olho esquerdo foi usado como grupo controle. Resultados: Após a ressecção do nervo óptico, a estabilidade da amplitude da eletrorretinografia foi perdida no olho direito após 302 segundos no Grupo 1 e após 296 segundos no Grupo 2. O olho esquerdo manteve eletrorretinografia estável por períodos mais longos. Conclusão: O modelo animal de transplante total de olho foi eficaz em descrever uma nova técnica cirúrgica para ser utilizada em laboratório com coelhos, com sucesso do procedimento anatômico. Novos estudos esclarecerão os melhores métodos de anastomose e manutenção da função do órgão receptor.

Unusual clinical phenotype of Stargardt disease / Fenótipo clínico incomum da doença de Stargardt

ABSTRACT Mutations in the ABCA4 gene are a common cause of Stargardt disease; however, other retinal phenotypes have also been associated with mutations in this gene. We describe an observational case report of an unusual clinical phenotype of Stargardt disease. The ophthalmological examination included best corrected visual acuity, color and autofluorescence photography, fluorescein angiography, optical coherence tomography, and electrophysiology tests. Targeted next-generation sequencing of 99 genes associated with inherited retinal dystrophies was performed in the index patient. A 48-year-old woman presented with a best corrected visual acuity of 20/25 and 20/20. Fundoscopy revealed perifoveal yellow flecked-like lesions. Fluorescein angiography and fundus autofluorescence findings were consistent with pattern dystrophy. Pattern electroretinogram demonstrated bilateral decrease of p50 values. Genetic testing identified two heterozygous missense mutations, c.428C>T, p.(Pro143Leu) and c.3113C>T, p.(Ala.1038Val), in the ABCA4 gene. Based on our results, we believe that these particular mutations in the ABCA4 gene could be associated with a specific disease phenotype characterized by funduscopic appearance similar to pattern dystrophy. A detailed characterization of the retinal phenotype in patients carrying specific mutations in ABCA4 is crucial to understand disease expression and ensure optimal clinical care for patients with inherited retinal dystrophies.

RESUMO Mutações no gene ABCA4 são causa comum da doença de Stargardt, mas outros fenótipos da retina também foram associados a mutações nesse gene. Apresentamos um relato de caso observacional de um fenótipo clínico incomum da doença de Stargardt. O exame oftalmológico incluiu a acuidade visual com melhor correção, fotografia em cores e com autofluorescência, angiofluoresceinografia, tomografia de coerência óptica e testes de eletrofisiologia. Na paciente em questão, realizou-se o sequenciamento de próxima geração de 99 genes associados a distrofias retinais hereditárias. Tratava-se de uma mulher de 48 anos com melhor acuidade visual corrigida de 20/25 e 20/20. A fundoscopia revelou lesões puntiformes amarelas perifoveais. Os resultados da angiofluoresceinografia e da autofluorescência do fundo de olho foram consistentes com distrofia em padrão. A eletrorretinografia por padrões mostrou diminuição bilateral dos valores de p50. Os testes genéticos revelaram duas mutações missense heterozigóticas, c.428C>T, p. (Pro143Leu) e c.3113C>T, p. (Ala.1038Val), no gene ABCA4. Nossos resultados nos fazem pensar que essas mutações específicas em ABCA4 talvez possam estar associadas a um fenótipo específico da doença, caracterizado por uma aparência fundoscópica semelhante à da distrofia em padrão. Uma caracterização detalhada do fenótipo da retina em pacientes portadores de mutações específicas em ABCA4 é crucial para compreender a expressão da doença e para garantir o tratamento clínico ideal para pacientes com distrofias retinais hereditárias.

Clinical and electroretinographic profile of 27 patients with Stargardt disease treated at a hospital in Brazil / Perfil clínico e eletrorretinográfico de 27 pacientes com doença de Stargardt atendidos em um hospital do Brasil

ABSTRACT Purpose: Stargardt disease is the most common type of juvenile-onset macular dystrophy. It is bilateral and symmetrical in appearance, affects the macula, and its main characteristic is the loss of central vision that starts in the first or second decade of life. The purpose of this study was to describe the profile of the patients evaluated at the Complexo Hospital de Clínicas da Universidade Federal do Paraná, as well as describe the electroretinographic findings with the full-field electroretinogram in these patients. Methods: An observational, retrospective study was performed by analysis of records and electroretinographic examinations of 27 patients with Stargardt disease and fundus flavimaculatus who were treated at the Complexo Hospital de Clínicas da Universidade Federal do Paraná's Department of Ocular Electrophysiology and Neuro-Ophthalmology between 1997 and 2014. The patients included in this study presented clinical features, fundus examination and/or electroretinographic findings compatible with Stargardt disease. Results: The visual acuity in the best eye varied from 0 to 1.6 logMAR (20/20 to 20/800) with an average of 0.89 ± 0.42 logMAR. The age at onset of symptoms varied from since birth to 36 years old (average 19.2 ± 9.2) with the majority of patients having symptom onset in the first or second decade of life. The mean time from the disease's first symptoms until the diagnosis was 7.3 years. In the fundus examination, every patient presented some kind of abnormality. In the electroretinogram analysis, the majority of patients had results that differed from those of sample controls, i.e., reduced amplitude and increased implicit time in the photopic and scotopic phases. Conclusions: The visual acuity and the age at symptoms onset in this study were compatible with the natural history of this dystrophy. The typical fundus appearance of Stargardt disease and altered electroretinogram were more frequent because of the delay until diagnosis. New prospective studies are necessary to evaluate these patients based on emergent technologies.

RESUMO Objetivo: A doença de Stargardt é a forma mais comum de distrofia macular de início juvenil. É bilateral e simétrica em aparência, afeta a mácula e sua característica principal é a diminuição da visão central que geralmente inicia-se na primeira ou segunda década de vida. O objetivo do estudo é descrever o perfil clínico dos pacientes avaliados no Complexo Hospital de Clínicas da Universidade Federal do Paraná, bem como descrever os achados eletrorretinográficos destes pacientes com o eletrorretinograma de campo total. Métodos: Foi realizado um estudo observacional retrospectivo, baseado na análise de prontuários e eletrorretinograma de 27 pacientes com Doença de Stargardt e Fundus Flavimaculatus, atendidos em consulta oftalmológica no ambulatório de Eletrofisiologia Ocular e Neuro-Oftalmologia do Complexo Hospital de Clínicas da Universidade Federal do Paraná, entre 1997 e 2014. Os pacientes incluídos no estudo apresentavam quadro clínico, fundoscopia e/ou achados eletrorretinográficos compatíveis com a doença. Resultados: A acuidade visual no melhor olho variou de 0 a 1,6 logMAR (20/20 a 20/800), com média de 0,89 ± 0,42 logMAR. A idade de aparecimento dos sintomas variou desde o nascimento a 36 anos (19,2 ± 9,2), sendo a maioria nas 1ª e 2ª década de vida. Em relação ao tempo entre o início dos sintomas e o diagnóstico, a média foi de 7,3 anos. Na fundoscopia, todos os pacientes apresentaram alguma alteração. Na análise do eletrorretinograma, a maioria dos pacientes demonstrou resultados que diferem da amostra de pacientes controles, ou seja, amplitudes reduzidas e tempos de culminação aumentados nas fases fotópicas e escotópicas. Conclusões: A acuidade visual e idade de início de aparecimento dos sintomas encontrados neste estudo são compatíveis com a evolução desta distrofia. Achados fundoscópicos típicos da doença de Stargardt e eletrorretinograma alterados foram mais frequentes em decorrência do atraso no diagnóstico. Novos estudos prospectivos são necessários para avaliar estes pacientes, fundamentando-se em novas tecnologias.

Damage of blue-light exposure to retinal morphology and function in mice / 中华实验眼科杂志

Objective To study the damage effects of blue-light exposure on retinal morphology and function in mouse.Methods Twenty 8-week-old clean C57BL/6J mice were randomly divided into blue-light exposure group and normal control group by coin tossing method.The mice in the blue-light exposure group was exposed to 10 000 lx blue light for 5 days after dark adaptation for 24 hours,and the mice in the normal control group was kept under the normal light intensity for 5 days at 12-hour light/12-hour darkness cycles.The retinal thickness was detected by optical coherence tomography (OCT),and retinal function was evaluated by electroretinogram (ERG).The mice was sacrificed and the frozen section and flat mount of eyeball wall was created at 24 hours after irradiation.The expressions of rhodopsin (Rho),zonula occludens-1 (ZO-1) and β-catenin in the retinas were detected by immunofluorescent staining.The use and care of the experimental animals adhered to ARVO Statement by American Society of Visual and Ophthalmological Sciences (No.IACUC-1803029).Results The thickness of the retinal outer nuclear layer at 200,400,600,800 and 1 000 μm from the superior and inferior to optic nerves were thinned in the mice of the blue-light exposure group compared with those of the normal control group,showing significant differences between the two groups (all at P＜0.05).The b-wave amplitude of the scotopic and photopic ERG was (305.50±41.52) μV and (119.50±6.67)μV in the blue-light exposure group,respectively,which was significantly reduced in comparison with (415.50±28.77) μV and (139.75±8.26) μV of the normal control group (both at P＜ 0.05).Immunofluorescent staining showed that the retinal pigment epithelial (RPE) cells of the mice exhibited hexagonal shape with regular arrangement,retinal morphology was regular,and the expressions of Rho,ZO-1 and β-catenin proteins showed stronger fluorescence in the retinas of normal control group.However,structural disorder,diminishing fluorescence intensity of Rho,ZO-1 and β-catenin were found in the blue-light exposure group.The morphology of the retina was disordered while the cell structure was destroyed.Conclusions Blue-light irradiation results in morphological and functional damages of retina.

Damage of blue-light exposure to retinal morphology and function in mice / 中华实验眼科杂志

Objective@#To study the damage effects of blue-light exposure on retinal morphology and function in mouse.@*Methods@#Twenty 8-week-old clean C57BL/6J mice were randomly divided into blue-light exposure group and normal control group by coin tossing method.The mice in the blue-light exposure group was exposed to 10 000 lx blue light for 5 days after dark adaptation for 24 hours, and the mice in the normal control group was kept under the normal light intensity for 5 days at 12-hour light/12-hour darkness cycles.The retinal thickness was detected by optical coherence tomography (OCT), and retinal function was evaluated by electroretinogram (ERG). The mice was sacrificed and the frozen section and flat mount of eyeball wall was created at 24 hours after irradiation.The expressions of rhodopsin (Rho), zonula occludens-1 (ZO-1) and β-catenin in the retinas were detected by immunofluorescent staining.The use and care of the experimental animals adhered to ARVO Statement by American Society of Visual and Ophthalmological Sciences (No.IACUC-1803029).@*Results@#The thickness of the retinal outer nuclear layer at 200, 400, 600, 800 and 1 000 μm from the superior and inferior to optic nerves were thinned in the mice of the blue-light exposure group compared with those of the normal control group, showing significant differences between the two groups (all at P<0.05). The b-wave amplitude of the scotopic and photopic ERG was (305.50±41.52)μV and (119.50±6.67)μV in the blue-light exposure group, respectively, which was significantly reduced in comparison with (415.50±28.77)μV and (139.75±8.26)μV of the normal control group (both at P<0.05). Immunofluorescent staining showed that the retinal pigment epithelial (RPE) cells of the mice exhibited hexagonal shape with regular arrangement, retinal morphology was regular, and the expressions of Rho, ZO-1 and β-catenin proteins showed stronger fluorescence in the retinas of normal control group.However, structural disorder, diminishing fluorescence intensity of Rho, ZO-1 and β-catenin were found in the blue-light exposure group.The morphology of the retina was disordered while the cell structure was destroyed.@*Conclusions@#Blue-light irradiation results in morphological and functional damages of retina.

Enhanced S-cone syndrome: Clinical spectrum in Indian population

Purpose: Enhanced S-cone syndrome (ESCS), a rare disorder, is often misdiagnosed as other forms of retinal degenerations, which have a poorer prognosis than ESCS. The aim of this study is to report the varied clinical features of ESCS and distinguish it from other similar disorders. Methods: We retrospectively scrutinized the records of patients with confirmed diagnosis of ESCS and analyzed the findings. Results: We included 14 patients (age range 4–39 years) who were confirmed to have ESCS according to pathognomonic electroretinography (ERG) showing reduced photopic, combined responses, and 30 Hz flicker with reduced L, M cone responses and supernormal S cone responses. The disease presented in the 1st decade with night blindness and was almost stationary or minimally progressive. Mid-peripheral fundus changes in form of nummular pigmentary alterations, yellow punctate lesions, and macular schisis were noted. The vision ranged from 6/6 to 6/36 with follow-up ranging from 1month to 22 years. Conclusion: ESCS shows varied clinical features ranging from unremarkable fundus to pigment clumping and atrophic lesions. It has good prognosis with patients mostly maintaining their vision. ERG is diagnostic. More awareness and knowledge about this entity can help to differentiate it from other forms of night blindness.

Fundus Albipunctatus Diagnosed in a 9-year-old Female

PURPOSE: We report a case of fundus albipunctatus discovered in a young patient. CASE SUMMARY: A 7.6-year-old female showed numerous small whitish-yellow flecks in the perimacular area and retinal periphery. Dark adapted 0.01 electroretinography (ERG) and dark adapted 3.0 ERG were profoundly reduced. At 26 months after the first visit, the best-corrected visual acuities were 1.0 right eye and 0.9 left eye. There were no pigmented lesions, atrophic lesions, or vascular abnormalities in the retina. Humphrey and Goldmann visual field tests were performed, but neither of the tests revealed any scotomas or other visual field defect. The number and size of characteristic numerous small whitish-yellow retinal flecks seemed almost unchanged. In spectral domain-optical coherence tomography (SD-OCT), the subretinal hyper-reflective lesions spanned the retinal pigment epithelium and the external limiting membrane. ERG showed improved dark adapted responses (dark adapted 0.01 ERG and dark adapted 3.0 ERG) after prolonged dark adaptation (2.5 hours). No family member showed any abnormal findings. CONCLUSIONS: Fundus albipunctatus is a rare disease in Koreans. We report a case diagnosed using fundus photography, SD-OCT, visual field tests, and ERG after prolonged dark adaptation (2.5 hours).

Acute zonal occult outer retinopathy / 中华眼底病杂志

Acute zonal occult outer retinopathy (AZOOR) is an acquired retinal diseases.The majority of patients who develop AZOOR are women characterized by an acute onset of visual blurred and scotoma with photopsias.The fundus examination is often normal or appeared mild abnormal.The RPE atrophy of fundus is similar with white syndrome.Although FFA and ICGA features are either unremarkable or unrelated to AZOOR,there are still important in differential diagnosis.The characteristic abnormalities appearance of FAF (complicated and varied),OCT (regional anomaly of ellipsoid zone),visual field (visual field defect) and ERG (decreased amplitude and prolonged latency of rod reaction,maximum reaction,cone reaction and scintillation reaction) are considered critical examinations to the diagnosis of AZOOR.Although there is no effective therapy for AZOOR,it has some self-limitation.

Effect of simulated microgravity on the photopic negative response of flash electroretinogram in adult mice / 中华眼底病杂志

Objective To observe the effect of simulated microgravity on the photopic negative response (PhNR) of full-field flash ERG in adult mice.Methods In an experimental study,forty-eight adult male C57BL/6J mice (48 eyes) were randomly divided into model and control groups.Model mice were further divided into three subgroups of 8 each:tail-suspended for 15 days (subgroup A),tail-suspended for 30 days (subgroup B),and tail-suspended for 30 days followed by returning to normal position for 30 days (subgroup C).The three control subgroups were similarly fixed with a harness but kept in the normal position for corresponding periods of 15,30,and 60 days.The mice were immediately examined using ERG-PhNR,flash VEP,OCT and visually-guided behavior in vivo,and subsequently sacrificed to analyze the retinal histology in vitro.PhNR amplitude was measured from baseline to PhNR trough.N1 peak-time and N1-P1 amplitude of VEP was analyzed.The escape duration was used to quantitatively evaluate the visual function of mice.In addition,inner retinal thickness was analyzed by OCT imaging.Data were compared by the independent sample t-test.Results PhNR amplitude in the model subgroup A was obviously lower than the corresponding control subgroup,the difference was statistically significant (t=-3.196,P＜0.01).There was no significant difference in PhNR amplitude between the model subgroup B or C and the corresponding control subgroup (t=-1.976,0.285;P＞0.05).There was no significant difference in FVEP N1 peak-time or N1-P1 amplitude between any of the three model subgroups and the corresponding control subgroup (P＞0.05).There was no significant difference in OCT-measured inner retinal thickness between any of the three model subgroups and the corresponding control subgroup (t=-0.461,2.073,-0.402;P＞0.05).The three model subgroups showed almost normal retinal structure,including the retinal ganglion cell,inner pexiform layer,inner nuclear layer,outer plexiform layer,outer nuclear layer,ellipsoid zone and RPE.There was no significant difference in visually-guided escape time between any of the three model subgroups and the corresponding control subgroup (t=-0.637,-0.955,1.297;P＞0.05).Conclusion Via tail-suspension,short-term simulated microgravity can affect the PhNR of flash ERG;however,the change is reversible and does not affect visual function of mice.

A Case of Circadian Rhythm Sleep-Wake Disorder in Partial Blindness

Although it is well known that sleep disturbances can be developed in complete blindness, normally entrained circadian rhythm was observed in the majority of patients with partial blindness. Here, we describe a case with circadian rhythm sleep-wake disorder in partial loss of light perception. A 58-year-old man presented with difficulty in sleep initiation and excessive daytime sleepiness after retinal surgery. The electroretinography revealed partial impairment of light perception in the right side and preserved light perception in the left side. He was diagnosed as circadian rhythm sleep-wake disorder due to impaired light perception. While taking 2 mg of melatonin regularly at 9 every night, his sleep cycle and difficulty in sleep initiation were gradually improved and became fully normalized after 2 weeks. Circadian rhythm sleep-wake disorder could be developed even in partial blindness. Melatonin supplements could effectively improve the circadian rhythm sleep-wake disorder in partial blindness, like as in complete blindness.

Blue-on-Green Flash Induces Maximal Photopic Negative Response and Oscillatory Potential and Serves as a Diagnostic Marker for Glaucoma in Rat Retina

The purpose of this study was to investigate the application of various electroretinography (ERG) to the diagnosis of inner retinal dysfunction induced by mild intraocular pressure (IOP) elevation in a rat glaucoma model. For inner retinal function measurements, available photopic ERG protocols were applied under various light conditions including monochromatic combinations, which complement conventional scotopic ERG. Three episcleral veins in the right eyes of Sprague-Dawley rats were cauterized to induce an experimental model of glaucoma, leading to mild IOP elevation. ERG responses were measured before surgery and at 1, 2, 4, and 8 weeks after cauterization. We first confirmed that the amplitude reduction in the standard photopic b-wave was almost comparable to the amplitudes of scotopic a- and b-waves in glaucomatous eyes over time. We have implemented additional photopic ERG protocols under different stimulus conditions, which consisted of a longer duration and different monochromatic combinations. Such a change in the stimulations resulted in more pronounced differences in response between the two groups. Especially in normal animals, blue stimulation on a green background produced the largest b-wave and photopic negative response (PhNR) amplitudes and caused more pronounced oscillatory potential (OP) wavelets (individual components). In glaucomatous eyes, blue stimulation on a green background significantly reduced PhNR amplitudes and abolished the robust OP components. These results, by providing the usefulness of blue on green combination, suggest the applicable photopic ERG protocol that complements the conventional ERG methods of accessing the progression of glaucomatous damage in the rat retina.

Early multifocal electroretinography findings after intravitreal Conbercept in the treatment of wet age-related macular degeneration / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To evaluate the early changes of retinal function in patients with wet age-related macular degeneration(ARMD)after intravitreal Conbercept treatment by multifocal electroretinography(mfERG). <p>METHODS: Wet ARMD patients diagnosed by fundus fluorescein angiography and received intravitreal injection of Conbercept(0.05mL/0.5mg)were included. Best corrected visual acuity(BCVA), incubation of N1, P1 and amplitude of P1 in mfERG test were recorded before and 1mo after treatment. <p>RESULTS: Twenty patients(20 eyes)were enrolled in total. The mean BCVA was 0.80±0.48 LogMAR at baseline and improved to 0.65±0.50 LogMAR after treatment(<i>P</i><0.001). Mean P1 amplitude density of the mfERG Ring 1 increased from 39.59±16.60nV/deg² to 53.81±20.41nV/deg²(<i>P</i>=0.006). The change in visual acuity was positively correlated with the change in P1 amplitude density of Ring 1(<i>r</i>=-0.776, <i>P</i><0.001). <p>CONCLUSION: Intravitreal conbercept injection can improve the central retinal function of wet ARMD patients in the short term.

Functional Analysis and Immunochemical Analyses of Ca²⁺ Homeostasis-Related Proteins Expression of Glaucoma-Induced Retinal Degeneration in Rats

The retinal degeneration resulting from elevated intraocular pressure was evaluated through functional and morphological analyses, for better understanding of the pathophysiology of glaucoma. Ocular hypertension was induced via unilateral episcleral venous cauterization in rats. Experimental time was set at 1 and 3 days, and 1, 2, 4, and 8 weeks post-operation. Retinal function was analyzed using electroretinography. For morphological analysis, retinal tissues were processed for immunochemistry by using antibodies against the calcium-sensing receptor and calcium-binding proteins. Apoptosis was analyzed using the TUNEL method and electron microscopy. Amplitudes of a- and b-wave in scotopic and photopic responses were found to be reduced in all glaucomatous retinas. Photopic negative response for ganglion cell function significantly reduced from 1-day and more significantly reduced in 2-week glaucoma. Calcium-sensing receptor immunoreactivity in ganglion cells remarkably reduced at 8 weeks; conversely, protein amounts increased significantly. Calcium-binding proteins immunoreactivity in amacrine cells clearly reduced at 8 weeks, despite of uneven changes in protein amounts. Apoptosis appeared in both photoreceptors and ganglion cells in 8-week glaucomatous retina. Apoptotic feature of photoreceptors was typical, whereas that of ganglion cells was necrotic in nature. These findings suggest that elevated intraocular pressure affects the sensitivity of photoreceptors and retinal ganglion cells, and leads to apoptotic death. The calcium-sensing receptor may be a useful detector for alteration of extracellular calcium levels surrounding the ganglion cells.

Study of natural history of Chinese patients with choroideremia / 中华实验眼科杂志

Objective To provide detailed clinical and molecular genetic findings and describe the characteristics of natural history in Chinese choroideremia (CHM) patients.Methods The patients with CHM who met the inclusion criteria of at least two visits over a minimum period of 5 years were recruited on a voluntary basis at the Ophthalmic Genetics Clinic in Peking Union Medical College Hospital from April 2009 to August 2017.Molecular genetic analysis results,best-corrected visual acuity (BCVA),color fundus photograph,optical coherence tomography (OCT),visual field (VF),full-field electroretinography (fERG) were obtained.This study protocol was approved by the Institutional Review Board of Peking Union Medical College Hospital (S-K125).Written informed consent was obtained from each participant.Results Ten Chinese Han patients from seven CHM families were included.The mutations were confirmed by molecular genetic analysis,and two novel mutations were found.The median age of 10 patients at first visit was 44 years (range 8-52 years).The mean first-last visit period was 6.08 years (range 5.03-7.24 years).The mean BCVA at first visit in logMAR equivalents was 0.56 (range 0.0-2.0) or approximately 0.28 decimal acuity.The correlation between BCVA at first visit and age showed that relative good vision remained until 35 years old and BCVA subsequently reduced rapidly.OCT showed a thickening of the central retinal thickness at early stage,followed by a thinning over decades.Outer retinal tabulation (ORT) was shown in some patients.There was a strong negative correlation (r=-0.861,P＜0.001) between residual VF and age.Five patients did not need to record fERG because of serious fundus lesions.Two patients exhibited decreased amplitudes for both rod and cone-driven responses,and three patients exhibited no fERG amplitudes.Conclusions The progression of CHM may be severer and faster in Chinese patients than that in Western patients.ORT is an important manifestation of OCT in CHM patients.VF and fERG are applicable to evaluate the condition of very-early phase of CHM.

Intravitreal injection of polysorbate 80: a functional and morphological study / Injeção intravítrea de polissorbato 80: estudo funcional e morfológico

ABSTRACT Objective : to determine the functional and morphological effects at rabbits retina of PS80 concentration used in the preparation of intravitreal drugs. Methods: eleven New Zealand rabbits received a intravitreal injection of 0.1ml of PS80. As control, the contralateral eye of each rabbit received the same volume of saline. Electroretinography was performed according to a modified protocol, as well as biomicroscopy and retina mapping before injection and seven and ten days after. Animals were euthanized in the 30th day and the retinas were analyzed by light microscopy. Results: eyes injected with PS80 did not present clinical signs of intraocular inflammation. Electroretinography did not show any alteration of extent and implicit time of a and b waves at scotopic and photopic conditions. There were no morphological alterations of retinas at light microscopy. Conclusion: intravitreal injection of PS80 in the used concentration for intravitreal drug preparations do not cause any functional or morphological alterations of rabbit retinas. These results suggest that PS80 is not toxic to rabbit retinas and may be safely used in the preparation of new lipophilic drugs for intravitreal injection.

RESUMO Objetivo: determinar os efeitos funcionais e morfológicos na retina de coelhos da concentração de PS80 utilizada na preparação de drogas intravítreas. Métodos: onze coelhos New Zealand receberam injeção intravítrea de 0,1ml de PS80. Como controle, o olho contralateral de cada coelho recebeu o mesmo volume de soro fisiológico. Foram realizados eletrorretinogramas de acordo com o protocolo modificado, biomicroscopia e mapeamento de retina antes da injeção, sete e dez dias depois. Os animais foram sacrificados no 30o dia e as retinas analisadas por microscopia de luz. Resultados: os olhos injetados com PS80 não apresentaram sinais clínicos de inflamação intraocular. O eletrorretinograma não apresentou alteração de amplitude e tempo implícito das ondas a e b nas condições escotópica e fotópica. Não houve alteração morfológica da retina na microscopia de luz. Conclusão: a injeção intravítrea de PS80 na concentração utilizada na preparação de drogas intravítreas não causa alterações funcionais e morfológicas na retina de coelhos. Esses resultados sugerem que o PS80 não é tóxico para a retina de coelhos e pode ser usado com segurança na preparação de novas drogas lipofílicas para injeção intravítrea.

Retinal function in patients with the neuronal ceroid lipofuscinosis phenotype / Estudo da função retiniana em pacientes com o fenótipo da lipofuscinose ceróide neuronal

ABSTRACT Purpose: To analyze the clinical features, visual acuity, and full-field electroretinogram (ERG) findings of 15 patients with the neuronal ceroid lipofuscinosis (NCL) phenotype and to establish the role of ERG testing in NCL diagnosis. Methods: The medical records of five patients with infantile NCL, five with Jansky-Bielschowsky disease, and five with juvenile NCL who underwent full-field ERG testing were retrospectively analyzed. Results: Progressive vision loss was the initial symptom in 66.7% of patients and was isolated or associated with ataxia, epilepsy, and neurodevelopmental involution. Epilepsy was present in 93.3% of patients, of whom 86.6% presented with neurodevelopmental involution. Fundus findings ranged from normal to pigmentary/atrophic abnormalities. Cone-rod, rod-cone, and both types of dysfunction were observed in six, one, and eight patients, respectively. Conclusion: In our study, all patients with the NCL phenotype had abnormal ERG findings, and the majority exhibited both cone-rod and rod-cone dysfunction. We conclude that ERG is a valuable tool for the characterization of visual dysfunction in patients with the NCL phenotype and is useful for diagnosis.

RESUMO Objetivo: Analisar o quadro clínico, a acuidade visual e o eletrorretinograma de campo total (ERG) de 15 pacientes com o fenótipo da lipofuscinose ceróide neuronal (LCN), estabelecendo o papel do eletrorretinograma no seu diagnóstico. Métodos: Eletrorretinograma foi realizado em 5 pacientes com lipofuscinose ceróide neuronal infantil, 5 com doença de Jansky-Bielschowsky e 5 com lipofuscinose ceróide neuronal juvenil sendo feita uma análise retrospectiva dos registros médicos. Resultados: A perda progressiva da acuidade visual foi o sintoma inicial em 66,7%; isolada ou associada à ataxia, epilepsia e involução do desenvolvimento neuropsico motor. Epilepsia foi o sintoma inicial em 93,3% e 86,6% apresentaram involução do desenvolvimento neuropsicomotor. Achados fundoscópicos variaram de normal a alterações pigmentares/atróficas. Disfunção de cone-bastonete foi constatada em 6 pacientes, bastonete-cone em 1 e em 8 pacientes observou-se disfunção proporcional de ambos os sistemas. Conclusão: O eletrorretinograma foi alterado em todos os pacientes, e o achado mais frequente foi o comprometimento de cones e bastonetes. O eletrorretinograma constitui, portanto, uma ferramenta valiosa para caracterizar a disfunção visual em pacientes com o fenótipo da lipofuscinose ceróide neuronal, contribuindo para seu diagnóstico.