Clinical features and possible pathogenesis of multiple evanescent white dot syndrome with different retinal diseases and events: a narrative review.

Multiple evanescent white dot syndrome (MEWDS) is a rare fundus disease, characterized by acute vision loss and visual field defects. Many previous studies have explained the possible pathogenesis and clinical features of primary MEWDS. However, as the number of reported cases increases, secondary MEWDS occurs in other related retinal diseases and injuries, exhibiting some special characteristics. The associated retinal diseases include multifocal choroiditis/punctate inner choroidopathy (MFC/PIC), acute zonal occult outer retinopathy, best vitelliform macular dystrophy, pseudoxanthoma elasticum, and ocular toxoplasmosis. The related retinal injury is laser photocoagulation, surgery, and trauma. Although primary MEWDS often have a self-limiting course, secondary MEWDS may require treatment in some cases, according to the severity of concomitant diseases and complications. Notably, MEWDS secondary to MFC/PIC that is prone to forming choroidal neovascularization and focal choroidal excavation, needs positive treatment with corticosteroids. The possible underlying pathogenesis of secondary MEWDS is the exposure of choroidal antigen after the disruption of Bruch's membrane. The MEWDS-related features in secondary MEWDS are still evanescent under most circumstances. Its prognosis and treatment depend on the severity of complications. Current studies propose that the etiology is associated with immune factors, including viral infection, inflammation in choroid and Bruch's membrane, and antigen exposure caused by retinal and/or choroidal insults. More pathogenic studies should be conducted in the future. Accurate diagnosis for secondary MEWDS could benefit patients in aspects of management and prognosis.

Vitreoretinal Lymphoma with Intraretinal Infiltration, Simulating Retinal Necrosis.

PURPOSE: To describe the clinical features and outcomes of vitreoretinal lymphoma (VRL) with intraretinal infiltration, a pseudonecrotic variant. DESIGN: Retrospective, comparative analysis. SUBJECTS: Patients with biopsy-proven VRL at a single center from August 2016 to April 2022. METHODS: A retrospective record review was conducted for clinical, imaging, and laboratory data. MAIN OUTCOME MEASURES: Clinical features, visual, and survival outcomes. RESULTS: We included 67 eyes of 40 patients with biopsy-proven VRL. Pseudonecrotic retinal lesions (PRLs) were found in 24 (35.8%) eyes of 19 patients; these eyes were classified as a pseudonecrotic variant, whereas the remaining 43 (64.2%) eyes were classified as nonnecrotic. Comparison (pseudonecrotic vs. nonnecrotic) revealed that eyes with PRLs at presentation had a worse median best-corrected visual acuity (BCVA; 2.4 vs. 0.5 logarithm of the minimum angle of resolution [logMAR], P < 0.0001) and severe ocular manifestations (P < 0.0001), including optic disc swelling (79.2% vs. 0%), retinal vasculitis (93.8% vs. 4.7%), retinal hemorrhage (83.3% vs. 0%), and retinal detachment (RD) (79.2% vs. 0%). Follow-up data were available for 20 eyes (17 patients) in the pseudonecrotic group and 43 eyes (21 patients) in the nonnecrotic group. An equally worse median BCVA was noted in pseudonecrotic eyes at 6 months after treatment and the final follow-up as compared with nonnecrotic eyes (2.4 vs. 0.3 logMAR, P < 0.0001). The median follow-up period did not differ significantly (16.6 vs. 18.4 months, P = 0.47). Initial BCVA (ß = 0.300, P = 0.003), presence of anterior chamber cell (ß = 0.472, P = 0.013), and RD (ß = 1.137, P < 0.0001) were significantly associated with poor visual outcomes in multivariate linear regression analysis (adjusted R2 = 0.693). There were no significant differences in survival outcomes. CONCLUSION: Vitreoretinal lymphoma can present as pseudonecrotic retinopathy, with more advanced clinical presentations and worse final visual outcomes. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Automated Classification of Inherited Retinal Diseases in Optical Coherence Tomography Images Using Few-shot Learning.

Objective: To develop a few-shot learning (FSL) approach for classifying optical coherence tomography (OCT) images in patients with inherited retinal disorders (IRDs). Methods: In this study, an FSL model based on a student-teacher learning framework was designed to classify images. 2,317 images from 189 participants were included. Of these, 1,126 images revealed IRDs, 533 were normal samples, and 658 were control samples. Results: The FSL model achieved a total accuracy of 0.974-0.983, total sensitivity of 0.934-0.957, total specificity of 0.984-0.990, and total F1 score of 0.935-0.957, which were superior to the total accuracy of the baseline model of 0.943-0.954, total sensitivity of 0.866-0.886, total specificity of 0.962-0.971, and total F1 score of 0.859-0.885. The performance of most subclassifications also exhibited advantages. Moreover, the FSL model had a higher area under curves (AUC) of the receiver operating characteristic (ROC) curves in most subclassifications. Conclusion: This study demonstrates the effective use of the FSL model for the classification of OCT images from patients with IRDs, normal, and control participants with a smaller volume of data. The general principle and similar network architectures can also be applied to other retinal diseases with a low prevalence.

Comparison of Clinical and Immunological Features of Para- and Non-Paraneoplastic Autoimmune Retinopathy in Chinese - A Series of 48 Cases.

PURPOSE: To characterize and compare clinical and immunological features of para(p)-autoimmune retinopathy (AIR) and non-para(np)-AIR and to assess the clinical significance of the presence of serum anti-retinal antibodies (ARAs). METHODS: We retrospectively reviewed 48 Chinese patients with p-AIR or np-AIR who took comprehensive ophthalmic examinations and lab tests of the presence of serum ARAs. RESULTS: p-AIR patients differed from np-AIR patients in terms of disease progression, ocular inflammation, findings of OCT, FFA, and presence of ARAs. No significant difference was found in the band number of serum ARAs between AIR patients and healthy controls. The prevalence of antibodies to recoverin and ɑ-enolase in the sera of p-AIR was significantly higher than that of the healthy individuals. CONCLUSION: While having many similar clinical signs, patients with p-AIR or np-AIR nevertheless displayed unique characteristics. Detection of ARAs subtypes, rather than their quantity, may be helpful in evaluating the conditions in the verified instances.

Management of autoimmune retinopathy treated with intravitreal dexamethasone implant.

PURPOSE: The purpose of this study is to evaluate clinical outcomes of autoimmune retinopathy (AIR) in the patients treated with intravitreal dexamethasone implant (IDI). METHOD: Twenty-one eyes of 11 AIR patients treated with at least 1 injection of IDI were retrospectively reviewed. Clinical outcomes before and after treatment, including best corrected visual acuity (BCVA), optic coherence tomography (OCT), fundus autofluorescence (FAF), full-field electroretinography (ff-ERG), and visual field (VF) at last visit within 6 and/or 12 months, were recorded. RESULTS: Among all the patients, 3 had cancer-associated retinopathy (CAR) and 8 had non-paraneoplastic-AIR (npAIR) with mean followed up of 8.52 ± 3.03 months (range 4-12 months). All patients achieved improved or stable BCVA within 6 and/or 12 months after the treatment. Cystoid macular edema (CME) in 2 eyes and significant retinal inflammation in 4 eyes were markedly resolved after single injection. Central retinal thickness (CFT) in all eyes without CME, ellipsoid zone (EZ) on OCT in 71.4% of eyes, ERG response in 55% of eyes, and VF in 50% of eyes were stable or improved within 6 months after treatment. At last visit within 12 months, both BCVA and CFT remained stable in the eyes treated with either single or repeated IDI; however, progression of EZ loss and damage of ERG response occurred in some patients with single IDI. CONCLUSION: Clinical outcomes, including BCVA and parameters of OCT, ERG, and VF, were stable or improved after IDI in a majority of AIR patients. Local treatment of AIR with IDI was a good option to initiate the management or an alternative for the patients' refractory to the systemic therapy but with limited side effect.

Re-vitrectomy for recurrent retinal detachment in post-vitrectomy eyes of rhegmatogenous retinal detachment.

BACKGROUND: Recurrent retinal detachment (Re-RD) usually affects the prognosis of surgery for rhegmatogenous retinal detachment (RRD). Previous clinical studies of Re-RD were not specific. This study aimed to analyze the clinical characteristics of Re-RD in post-vitrectomy eyes with RRD and surgical outcomes after revitrectomy without combining it with retinectomy or scleral buckling. METHODS: This is a retrospective case series analyzed the ocular characteristics of 20 recurrent and contralateral eyes, evaluated the significance of the associations between variables before reoperation and the final best-corrected visual acuity (BCVA), and calculated the outcome of revitrectomy. RESULTS: Patients with phakic eyes, those undergoing only one surgery, and those with more than one break had better final BCVA. The final BCVA was negatively correlated with the axial length and positively correlated with the preoperative BCVA. Among the 12 eyes with no break detected before surgery, 11 (92%) were found to have a small crevice-like break beside the pigment scar of a large number of original laser spots. The single-operation complete retinal reattachment rate was 75%, the complete retinal reattachment rate was 80%, and the final incomplete retinal reattachment rate was 90%. The BCVA improved from 1.2 ± 0.6LogMAR (0.06 ± 0.25) before surgery to 0.8 ± 0.7LogMAR (0.15 ± 0.2) at the last follow-up. The BCVA of 16 patients with complete retinal reattachment improved from 1.0 ± 0.5LogMAR (0.1 ± 0.3) to 0.6 ± 0.4LogMAR (0.25 ± 0.4). In the contralateral eyes, 15% already had vision-damaging disease, and the incidence of eyesight-threating lesions was 5.9% during follow-up. CONCLUSIONS: Revitrectomy without retinectomy or scleral buckling can effectively treat Re-RD in post-vitrectomy eyes. In Re-RD patients with no definite retinal break detected preoperatively, the retinal hole usually shows small crevice-like changes alongside a large number of original laser pigment scars.

Optic disc coloboma associated with macular retinoschisis: A case report.

BACKGROUND: To report an unusual case of bilateral optic disc coloboma associated with macular retinoschisis in the left eye. CASE SUMMARY: A 37-year-old woman presented with complaints of blurred and distorted vision in her left eye for more than 1 year. Fundus examination demonstrated choroidal atrophy around the optic disc in both eyes, with a cup-to-disc ratio of 0.9. Serous retinal detachment in the macular area of the left eye. Left eye macular blood flow imaging optical coherence tomography (Angio-OCT) showed macular retinal serous cleavage. En-face OCT showed that the canal gully-like structure formed by the defect of the optic disc nerve fiber layer between the optic disc and macula, serous detachment area was connected with the enlarged optic disc coloboma through the canal gully-like structure, and the fluid leaked from the enlarged and thinned optic disc coloboma into the retinal layer of the macular area. Patients with optic disc abnormalities and macular degeneration must be monitored appropriately. During the follow-up period, the use of optic disc stereography and 3D-OCT, en-face, and Angio-OCT imaging can clarify the correlation between macular retinoschisis and optic disc coloboma. CONCLUSION: Macular retinoschisis may be owing to the combined force of disc edge loss, enlarged optic disc coloboma, the canal gully-like structure formed by the defect of the nerve fiber layer around the optic disc, and the traction of the posterior vitreous cortex.

Effects of antiseizure medications on alternative psychosis and strategies for their application.

Forced normalization (FN) is a unique phenomenon that is often seen in the treatment of epilepsy. FN is characterized by abnormal mental behavior and disordered emotions in epilepsy patients despite a significantly improved electroencephalogram and successful seizure control; the occurrence of FN seriously affects patients' quality of life. The causes of FN include antiseizure medications (ASMs), epilepsy surgery and vagus nerve stimulation, with ASMs being the most common cause. However, with the timely reduction or discontinuation of ASMs and the use of antipsychotic drugs, the overall prognosis is good. Here, we perform an extensive review of the literature pertaining to FN, including its epidemiology, possible mechanisms, clinical features, treatment and prognosis.

Effects of intensive insulin therapy on the retinal microvasculature in patients with type 2 diabetes mellitus: a prospective observational study.

BACKGROUND: We examined the retinal microvascular changes and associated factors in type 2 diabetes mellitus (T2DM) before and after intensive insulin therapy. METHODS: This prospective observational study recruited patients with T2DM and divided them into intensive insulin therapy and oral hypoglycemic agent groups. All patients enrolled in this study had diabetes without retinopathy or non-proliferative diabetic retinopathy. Optical coherence tomography angiography (OCTA) was used in all patients before treatment and at 1, 3, and 6 months after treatment. Vessel density (VD) and thickness changes in the macular and optic disc areas were assessed. RESULTS: The study included 36 eyes in the intensive insulin therapy group and 36 in the oral hypoglycemic agent group. One month after treatment, VD in the deep capillary plexus (DCP) and peripapillary capillary VD (ppVD) were significantly decreased by intensification (P = 0.009, 0.000). At three months after treatment, decreases in VD induced by intensification were found in the superficial capillary plexus (SCP), DCP, foveal density in a 300-µm-wide region around the foveal avascular area (FD-300), and ppVD (P = 0.032, 0.000, 0.039, 0.000). Six months after treatment, decreases in VD by intensification were observed in the DCP and ppVD groups (P = 0.000, 0.000). Vessel density showed no significant change in the oral hypoglycemic agent group after treatment. The amount of DCP-VD reduction was correlated with macular thickening (r = 0.348, P = 0.038; r = 0.693, P = 0.000 and r = 0.417, P = 0.011, respectively) after intensive insulin therapy. CONCLUSIONS: Insulin-intensive treatment caused a transient reduction in vessel density in the macular and optic disc areas. DCP-VD and ppVD were more susceptible at an earlier stage. Retinal microvasculature monitoring using OCTA is vital for patients with type 2 diabetes receiving intensive insulin therapy.

Analysis of Radial Peripapillary Capillary Density in Patients with Bietti Crystalline Dystrophy by Optical Coherence Tomography Angiography.

OBJECTIVE: We wanted to investigate the radial peripapillary capillary (RPC) network in patients with Bietti crystalline dystrophy (BCD). METHODS: We compared RPC densities in the disk and different peripapillary regions, obtained using optical coherence tomography angiography in 22 patients with BCD (37 eyes) and 22 healthy subjects (37 eyes). The BCD group was then divided into Stage 2 and Stage 3 subgroups based on Yuzawa staging, comparing the RPC densities of the two. RESULTS: The disk area RPC density was 38.8% ± 6.3% in the BCD group and 49.2% ± 6.1% in the control group ( P < 0.001), and peripapillary region RPC density was significantly lower in the BCD group than in the control group (49.1% ± 4.7% and 54.1% ± 3.0%, respectively, P < 0.001). There were no significant RPC density differences between the tempo quadrant and inside disk of Stages 2 and 3 subgroups; the other areas showed a significantly lower RPC density in Stage 3 than in Stage 2 BCD. CONCLUSION: The BCD group RPC density was significantly lower than the control group. The reduction of RPC density in the tempo quadrant occurred mainly in the Stage 1 BCD. In contrast, the reduction of RPC density in superior, inferior, and nasal quadrants occurred mainly in Stage 2.

Peripapillary hyper-reflective ovoid mass-like structure and dome-shaped maculopathy: A case report.

RATIONALE: Peripapillary hyperreflective ovoid mass-like structures (PHOMS) and dome-shaped macula (DSM) are 2 optical coherence tomography findings reported in 2018 and 2008, respectively. To date, there have been no ophthalmic case reports of concomitant PHOMS and DSM. PATIENT CONCERNS: A 19-year-old woman presented to our clinic with complaints of decreased vision in both eyes. DIAGNOSIS: The patient was diagnosed with PHOMS and a dome-shaped macula complicated by subretinal fluid in both eyes. INTERVENTIONS: A micropulse laser under the guidance of Indocyanine green angiography was applied to the hyperfluorescent areas and drugs to improve retinal microcirculation. OUTCOMES: No response to any intervention over the 41 months of follow-up, her visual acuity remained the same, and the subretinal fluid often recurred. LESSONS: PHOMS and DSM are associated with myopia; myopia may be a mediator between PHOMS and DSM. Dome-like structural changes may occur in different parts of the retina (optic disc and macula), caused by asymmetric myopic posterior scleral growth.

Macular ganglion cell complex injury in different stages of anterior ischemic optic neuropathy.

BACKGROUND: Anterior ischemic optic neuropathy (AION) is a group of ophthalmic diseases in which the optic nerve is injured causing blindness. However, the pathogenesis, clinical manifestations, and clinical treatments of AION are yet elusive. Only a few related experimental or clinical reports are available on the disease. In this study, spectral domain optical coherence tomography (SD-OCT) was used to examine the morphology of thickness swelling and atrophic changes of macular ganglion cell complex (mGCC) in the different stages of AION that were then compared with the visual fields. Thus, the clinical value of mGCC examination was alleged to be similar to that of the visual field. AIM: To explore the mGCC injury at different stages in AION and the clinical significance. METHODS: Cases with AION were analyzed in a retrospective study. SD-OCT was used to analyze the correlation between mGCC and peripapillary retinal nerve fiber layer thicknesses at different stages of AION and the changes in the corresponding stages of visual fields. RESULTS: A total of 21 cases (28 eyes) presented AION. The onset time of AION was defined as early stage (within 3 wk of onset), middle stage (from 3 wk to 2 mo), and late stage (disease span > 2 mo). In the early stage, the mGCC thickness of SD-OCT was within the normal high limit, and the perioptic nerve fibers thickness was more than the normal. The changes in the visual field in early stage were not consistent with the swelling changes in mGCC and peri-disc nerve fibers. In addition, atrophy and thinning appeared in mGCC, and the perioptic nerve fibers were swollen. However, the thickness was lower in the middle period than that in the early stage. The change in visual field was consistent with that of mGCC in this period. In the late stage, mGCC shrank and thinned, and the thickness of the nerve fibers around the optic disc in the corresponding region shrank and thinned. CONCLUSION: The changes in mGCC thickness in patients with AION showed early, middle, and late stages of development by SD-OCT. Although the early stage visual field changes of AION were not consistent with the swelling changes of mGCC, the horizontal delimitation or annular atrophy of mGCC was consistent with that in the middle and late stage of the disease. The atrophy of peripheral nerve fibers was later than that of the mGCC atrophy.

Outcome of scleral buckling with or without gas tamponade for recurrent retinal detachment in post-vitrectomy eyes.

BACKGROUND: Treatment of recurrent retinal detachment (re-RD) following vitrectomy (post-gas/air tamponade and post-silicone oil removal) is challenging. Previously reported treatment is commonly revision pars plana vitrectomy (PPV) combined with tamponade, which is invasive and a burden both economically and emotionally when compared with scleral buckling (SB). The purpose of this study is to report anatomical and functional outcomes of SB with or without gas tamponade in eyes with recurrent retinal detachment (re-RD) that previously underwent PPV at least once. METHODS: We retrospectively reviewed the medical records of 14 patients (14 eyes) who underwent PPV at least once and were treated with SB after re-RD. Preoperative characteristics, intraoperative complications, and postoperative data were assessed. The final anatomical and functional outcomes were analyzed. RESULTS: The original PPV was performed for primary rhegmatogenous retinal detachment in 11 eyes, macular hole retinal detachment in 2 eyes, and myopic foveoschisis in 1 eye. Previously, 3 eyes underwent one PPV with gas tamponade, and the remaining 11 (79%) eyes underwent 2-5 operations. Seven eyes underwent the procedure with gas injection. At the last follow-up, 13 eyes achieved total retinal attachment and 1 eye had re-RD. The postoperative intraocular pressure was within the normal range, except in 1 eye (6 mmHg). The finest postoperative best-corrected visual acuity (BCVA) was 20/25. There was a significant improvement in BCVA from 20/160 ± 20/63 at baseline to 20/80 ± 20/50 at the last visit in the 13 successfully treated eyes (P = 0.025). CONCLUSIONS: SB can be effective for re-RD after PPV in specific cases.

Bilateral Necrotizing Retinitis following Encephalitis Caused by the Pseudorabies Virus Confirmed by Next-Generation Sequencing.

Purpose: The objective of this study was to report a case of bilateral necrotizing retinitis following viral encephalitis caused by the pseudorabies virus.Case report: A 49-year-old male had decreased bilateral visual acuity after the recovery of consciousness for one month. He had been in an unconsciousness status due to encephalitis for two months before the ocular symptoms developed. He was a pig slaughterer. Ocular ultrasound showed bilateral vitreous haze and retinal detachment. A vitrectomy and silicone oil tamponade were performed on the left eye. During surgery, massive periphery retinal necrosis appearing as a tattered fish net, and multiple retinal holes were observed. The pseudorabies virus was detected by next-generation sequencing in the vitreous specimen.Conclusion: The pseudorabies virus may cause bilateral necrotizing retinitis following viral encephalitis among those with close contact to pigs. Intraocular fluid provides a greater selection of samples and a longer time window for pathogenic detection.

Fumonisin B1 Affects the Biophysical Properties, Migration and Cytoskeletal Structure of Human Umbilical Vein Endothelial Cells.

Fumonisin B1 (FB1) is an important mycotoxin in nature and is a serious threat to human and animal health, but its specific target and molecular mechanism of the toxicity and potential carcinogenicity remain unclear. In this study, we first detected the effects of FB1 on the cell viability, biophysical properties, migration ability, and reactive oxygen species (ROS) of human umbilical vein endothelial cells (HUVECs). Subsequently, changes in the cytoskeletal structure and its binding proteins were analyzed by immunofluorescence and real-time PCR, respectively. The results showed that FB1 could inhibit the viability of HUVECs in a dose-dependent manner. After treatment of HUVECs with FB1, the hypotonic resistance, cell surface charges, cell membrane fluidity, and migration ability were weakened, whereas the ROS levels were significantly increased. Moreover, the cytoskeletal structure of the HUVECs was significantly changed, and the mRNA expression of some important actin-binding proteins was altered. Therefore, this study revealed that FB1 can affect the migration and cytoskeletal structure of HUVECs, which provides a new perspective for further understanding the molecular mechanisms of FB1 toxicity.

Macular Perfusion Changes and Ganglion Cell Complex Loss in Patients with Silicone Oil-related Visual Loss.

OBJECTIVE: The aim of this study was to investigate macular perfusion changes and ganglion cell complex (GCC) loss in patients with unexplained visual loss following vitrectomy and silicone oil (SO) tamponade, and to evaluate the correlation between retinal blood flow and GCC loss using optical coherence tomography angiography (OCTA) and optical coherence tomography (OCT). METHODS: This retrospective study included seven eyes (seven patients) with unexpected visual loss after vitrectomy and SO tamponade. OCTA was used to evaluate the alterations in retinal vessel density (VD) in the superficial capillary plexus (SCP), deep capillary plexus (DCP), and radial peripapillary capillary plexus (RPCP). OCT was used to measure the thickness of GCC and retinal nerve fiber layer (RNFL). Medical records of patients were reviewed. RESULTS: Quantitative analysis of OCTA images revealed a significant reduction in SCP VD in the affected eyes compared with the controls (all sections P < 0.05). No difference was found in GCC thickness, but FLV (focal loss volume) and GLV (global loss volume) were significantly higher in the affected eyes (both P < 0.001). SCP VD was inversely correlated with FLV and GLV. CONCLUSIONS: Silicone oil-related severe visual loss was associated with superficial retinal microvasculature damage and ganglion cell apoptosis.

Genetic factors for idiopathic choroidal neovascularization.

Objective: The aim of this study was to investigate genetic factors associated with idiopathic choroidal neovascularization (ICNV). Methods: We conducted a case-control study including 69 cases with ICNV and 114 controls who underwent cataract surgery. Single nucleotide polymorphisms (SNPs) from genes reported to be related to AMD, CNV and uveitis were selected for this study. Results: In an univariate analysis, the rs669676 SNP located in the COL8A1 gene was associated with the proportion of people who has idiopathic CNV ( X2 = 9.3453, corrected p-value = 0.1). For the rs669676 SNP, minor allele homozygotes, in the dominant model of genotype analysis (GG versus AA-GA), it showed significant differences in the ICNV group vs controls (p = .01, OR = 1.219 (95%CI: 1.04-1.429)). Conclusions: The rs669676 SNP located in the COL8A1 gene may contribute to a genetic susceptibility for ICNV.

Detection of serum anti-retinal antibodies in the Chinese patients with presumed autoimmune retinopathy.

PURPOSE: To explore the presence of serum anti-retinal antibodies (ARAs) in the Chinese patients with presumed autoimmune retinopathy (AIR). METHODS: Twenty-three Chinese patients with presumed AIR, disease controls including 40 RP patients, 22 bilateral uveitis patients, 18 acute zonal outer occult retinopathy (AZOOR) patients, and 30 healthy donors were included. Serum samples of all the subjects were obtained and analyzed for the presence of four ARAs including recoverin, α-enolase, carbonic anhydraseII (CAII), and collapsin response-mediated protein (CRMP)-5 by Western bolt assay. RESULTS: ARAs were present in the serum of either presumed AIR patients, disease control, or healthy donors. One or more ARAs were present in the 78.2% of presumed AIR while they were indicated in the 35.0% of RP patients (p < 0.01) and 33.3% of healthy donors (p < 0.01). The prevalence of ARAs in the bilateral uveitis and AZOOR was 63.3% and 100% respectively. Positive rate of α-enolase antibody present in the presumed AIR, disease control, and healthy donors was 73.9%, 47.5%, and 33.3% respectively. Positive rate of CAII antibody present above groups was 52.1%, 50%, and 33.3% respectively. Recoverin antibody seemed to be specifically present in the serum of patients with cancer-associated retinopathy. CONCLUSION: Presence of serum ARAs including recoverin, α-enolase, CAII, or CRMP-5 in the Chinese patients with presumed AIR occurred significantly more often than RP patients and healthy donors. Seropositivity of ARAs had diagnostic value for the presumed AIR but mere presence was not sufficient for the diagnosis due to identification of them in the healthy controls and other retinal diseases.

Quantitative evaluation of primary retinitis pigmentosa patients using colour Doppler flow imaging and optical coherence tomography angiography.

PURPOSE: To find the potential relation between changes in retinal large vessels and terminal vessels using colour Doppler flow imaging (CDFI) and optical coherence tomography angiography (OCTA) and to compare the respective advantages of CDFI and OCTA in evaluating vascular changes in retinitis pigmentosa (RP) patients. METHODS: A prospective series of case study was conducted to enrol RP patients and age-matched controls, who were, respectively, imaged by CDFI and OCTA. Repeatability and reproducibility of both CDFI and OCTA were performed among healthy volunteers. The central retinal artery (CRA) was detected by CDFI analysis to provide parameters of peak systolic velocity (PSV), end-diastolic velocity (EDV) and time-averaged maximum velocity (TAMV). Retinal parameters were evaluated from OCTA images, including vascular area density (VAD) of the superficial vascular layer, the fovea avascular zone (FAZ) area and retinal thickness. RP patients were separated into a high-vision group and a low-vision group, according to median vision (0.3, LogMAR 0.5). Multiple comparisons were used to analyse the data between groups. A correlation analysis was used to determine the correlation between CDFI and OCTA parameters. RESULTS: Twenty RP patients (40 eyes) and thirteen normal volunteers (26 eyes) were enrolled in this study. Repeatability and reproducibility of the measurements by CDFI had higher CVs, from 4.5% to 15.4%, than those measurements by OCTA (<5%). All the CDFI and OCTA parameters examined had significant reductions in RP patients compared to those in the controls (p < 0.01). Compared to the high-vision group, the low-vision group exhibited a statistically significant decrease in vascular parameters of the FAZ area, fovea VAD and parafovea nasal side VAD (p < 0.05); as well as in the parameters of the fovea thickness, and the parafovea nasal, superior and inferior side thickness (p < 0.05). From the correlation analysis, a significant association was found between the vision and CDFI parameters (PSV and time-averaged maximum velocity (TAMX), p < 0.05), and the vision and OCTA parameters (FAZ area, fovea and nasal side VAD, retinal thickness in all sides, p < 0.05). PSV and TAMX of the CRA were closely related to the OCTA superficial VAD in all sides, whereas the CDFI parameters showed poor correlation with retinal thickness. CONCLUSIONS: Colour Doppler flow imaging (CDFI) and OCTA parameters revealed a significant reduction in RP patients when compared to the controls. OCTA can detect vision-related microvascular and thickness changes around the macula between high- and low-vision groups, which happen earlier than the changes in large vessels. In addition to good repeatability and reproducibility, OCTA may have significant utility in the diagnosis and monitoring of disease progression in RP patients.