DISCREPANCY BETWEEN FUNDUS AUTOFLUORESCENCE ABNORMALITY AND VISUAL FIELD LOSS IN BIETTI CRYSTALLINE DYSTROPHY.

PURPOSE: The aim of this study was to explore the potential benefits of retinal pigment epithelium replacement therapy in patients with Bietti crystalline dystrophy (BCD) by assessing the disease pathology with the distinctive relationship between fundus autofluorescence (FAF) abnormality and visual field defect. METHODS: Sixteen eyes from 16 patients with BCD and 16 eyes from 16 patients with RHO-associated retinitis pigmentosa were included. Fundus autofluorescence, optical coherence tomography, and Goldmann perimetry results were retrospectively reviewed and assessed using image analyses. RESULTS: In patients with BCD, the FAF abnormality area was not correlated with the overall visual field defect area and median overall visual field defect area (57.5%) was smaller than FAF abnormality area (98.5%). By contrast, the ellipsoid zone width was significantly correlated with the central visual field area (r = 0.806, P < 0.001). In patients with RHO-associated retinitis pigmentosa, the FAF abnormality area and ellipsoid zone width were significantly correlated with the overall visual field defect area (r = 0.833, P < 0.001) and central visual field area (r = 0.887, P < 0.001), respectively. CONCLUSION: The FAF abnormality shown in patients with BCD involves retinal pigment epithelium degeneration without complete loss of photoreceptors or visual function. These results suggest that patients with BCD are good candidates for retinal pigment epithelium replacement therapy for preservation of residual visual function.

Ocular biometry with swept-source optical coherence tomography-based optical biometer in Japanese patients with EYS-related retinitis pigmentosa: a retrospective study.

BACKGROUND: This study aimed to identify the features of ocular biometry in patients with EYS-related retinitis pigmentosa using IOLMaster 700. METHODS: We retrospectively reviewed the medical records of patients with retinitis pigmentosa. Patients with records of the following were included: (1) ocular biometry measurements using the IOLMaster 700 and (2) genetic diagnostic tests. Axial length, keratometry, anterior chamber depth, aqueous depth, lens thickness, central corneal thickness (CCT), and corneal diameter (white to white) measurements were extracted. Based on keratometry measurements, (1) standard keratometric astigmatism, (2) posterior corneal astigmatism, and (3) total corneal astigmatism were obtained. Demographics and biometric parameters were compared between patients with EYS-related retinitis pigmentosa and other patients with retinitis pigmentosa. RESULTS: A total of 86 eyes of 44 patients (23 females and 21 males; mean age: 47.7 years) with retinitis pigmentosa were included. Of these, 18 were identified as having EYS variants. CCT was significantly thinner (P < 0.001) and the posterior corneal curvature at the steepest meridian was significantly smaller (P = 0.024) in patients with EYS-related retinitis pigmentosa than in other patients with retinitis pigmentosa. The magnitudes of all corneal astigmatism measurements was higher in patients with EYS-related RP, although these differences were not statistically significant. CONCLUSION: Patients with EYS-related retinitis pigmentosa had unique features in ocular biometry, such as thinner central corneal thickness and smaller posterior corneal curvature radius at the steepest meridian compared with other patients with retinitis pigmentosa. The findings suggest that patients with retinitis pigmentosa have different ocular dimension features among the different causative genes.

Optogenetic Therapy for Visual Restoration.

Optogenetics is a recent breakthrough in neuroscience, and one of the most promising applications is the treatment of retinal degenerative diseases. Multiple clinical trials are currently ongoing, less than a decade after the first attempt at visual restoration using optogenetics. Optogenetic therapy has great value in providing hope for visual restoration in late-stage retinal degeneration, regardless of the genotype. This alternative gene therapy consists of multiple elements including the choice of target retinal cells, optogenetic tools, and gene delivery systems. Currently, there are various options for each element, all of which have been developed as a product of technological success. In particular, the performance of optogenetic tools in terms of light and wavelength sensitivity have been improved by engineering microbial opsins and applying human opsins. To provide better post-treatment vision, the optimal choice of optogenetic tools and effective gene delivery to retinal cells is necessary. In this review, we provide an overview of the advancements in optogenetic therapy for visual restoration, focusing on available options for optogenetic tools and gene delivery methods.

Correlation between tangential distortion of the outer retinal layer and metamorphopsia in patients with epiretinal membrane.

PURPOSE: To evaluate tangential morphological changes in the outer retina and assess their correlation with the degree of metamorphopsia in patients with idiopathic epiretinal membrane (ERM). METHODS: This retrospective study included patients with idiopathic ERM who underwent vitrectomy between January 2018 and December 2019. We evaluated the preoperative examination results. Using cross-sectional spectral-domain optical coherence tomography (OCT) images along the horizontal/vertical meridian through the fovea, we defined a new parameter, tangential displacement (TD), as the tangential component of the position vector of the distorted outer nuclear layer caused by ERM. Visual function measurements included M-CHARTS results (vertical/horizontal metamorphopsia score [MV/MH]) and best-corrected visual acuity (BCVA). The correlations among the OCT parameters including TD and central foveal thickness (CFT) with visual function measurements were determined. RESULTS: Overall, 78 eyes of 76 patients (49 females; mean age, 67.9 [± standard deviation, 7.5 years]) were included. The mean horizontal TD was 24.0 ± 73.9 µm, which was significantly different from 0 (p = 0.005). The mean vertical TD was 6.0 ± 76.2 µm, which was not significantly different from 0. The absolute value of horizontal TD was significantly correlated with MV (r = 0.513, p < 0.01) and MH (r = 0.423, p < 0.01). The absolute value of vertical TD was also significantly correlated with MV (r = 0.274, p = 0.02) and MH (r = 0.413, p < 0.01). However, neither value was significantly correlated with BCVA. Multiple regression analysis showed that the horizontal absolute TD was an independent factor associated with both MV (ß = 0.635, p < 0.001) and MH (ß = 0.259, p = 0.048). CONCLUSION: We found that ERM tended to distort the outer retinal layer toward the temporal side of the fovea. The tangential distortion of this layer was associated with the degree of metamorphopsia, suggesting that misalignment of parafoveal photoreceptors causes metamorphopsia in patients with ERM.

Discovery of novel 2-(4-aryl-2-methylpiperazin-1-yl)-pyrimidin-4-ones as glycogen synthase kinase-3ß inhibitors.

We herein describe the results of further evolution of glycogen synthase kinase (GSK)-3ß inhibitors from our promising compounds containing a 3-methylmorpholine moiety. Transformation of the morpholine moiety into a piperazine moiety resulted in potent GSK-3ß inhibitors. SAR studies focused on the nitrogen atom of the piperazine moiety revealed that a phenyl group afforded potent inhibitory activity toward GSK-3ß. Docking studies indicated that the phenyl group on the piperazine nitrogen atom and the methyl group on the piperazine make cation-π and CH-π interactions with GSK-3ß respectively. 4-Methoxyphenyl analogue 29 showed most potent inhibitory activity toward GSK-3ß with good in vitro and in vivo pharmacokinetic profiles, and 29 demonstrated a significant decrease in tau phosphorylation after oral administration in mice.

Discovery of novel 2-(alkylmorpholin-4-yl)-6-(3-fluoropyridin-4-yl)-pyrimidin-4(3H)-ones as orally-active GSK-3ß inhibitors for Alzheimer's disease.

We herein describe the results of further evolution of GSK-3ß inhibitors for Alzheimer's disease from our promising compounds with in vivo tau phosphorylation inhibitory activity by oral administration. Introduction of a low alkyl group instead of the phenyl group at the 3-position of the morpholine moiety aiming to improve pharmacokinetic profiles resulted in potent low molecular weight GSK-3ß inhibitors with good in vitro pharmacokinetic profiles, which also showed in vivo tau phosphorylation inhibitory activity by oral administration. Effect of the stereochemistry of the alkyl moiety is also discussed using docking models.

Relationship between residual visual field and full-field stimulus testing in patients with late-stage retinal degenerative diseases.

This study aimed to investigate how the extent and central/peripheral location of the residual visual field (VF) in patients with late-stage inherited retinal diseases (IRDs) are related to retinal sensitivity detected using full-field stimulus testing (FST). We reviewed the results of Goldmann perimetry and FST from the medical records of patients with IRDs whose VF represents central (within 10°) and/or peripheral islands, or undetectable. In total, 19 patients (19 eyes) were analyzed in this study. The median value of residual VF area was 1.38%. The median values of rod and cone sensitivities were - 14.9 dB and 7.4 dB, respectively. Patients with only the peripheral island (- 33.9 dB) had better median rod sensitivity than other groups (only central, - 18.9 dB; both, - 3.6 dB). VF area significantly correlated with rod sensitivity (r = - 0.943, p = 0.005) in patients with only peripheral island, but not with cone sensitivity. Peripheral VF islands were significant contributors to FST results, especially rod sensitivity. With reduced or loss of central vision, the extent of residual peripheral VF significantly affected rod sensitivity, suggesting that FST can be useful in quantitatively estimating the overall remaining vision in patients with late-stage IRD.

Genetic and Clinical Features of ABCA4-Associated Retinopathy in a Japanese Nationwide Cohort.

PURPOSE: To clarify the genetic and clinical features of Japanese patients with ABCA4-associated retinopathy. DESIGN: Retrospective, multicenter cohort study. METHODS: Patients with retinal degeneration and biallelic ABCA4 variants were recruited from 13 different hospitals. Whole exome sequencing analysis was used for genetic testing. Comprehensive ophthalmic examinations were performed on matched patients. The primary outcome measure was identifying multimodal retinal imaging findings associated with disease progression. RESULTS: This study included 63 patients: 19 with missense/missense, 23 with missense/truncation, and 21 with truncation/truncation genotypes. In total, 62 variants were identified, including 29 novel variants. Six patients had a mild phenotype characterized by foveal-sparing or preserved foveal structure, including 4 with missense/missense and 2 with missense/truncation genotypes. The p.Arg212His variant was the most frequent in patients with mild phenotypes (4/12 alleles). Clinical findings showed a disease duration-dependent worsening of the phenotypic stage. Patients with the truncation/truncation genotype exhibited rapid retinal degeneration within a few years and definite fundus autofluorescence imaging patterns, including hyper autofluorescence at the macula and few or no flecks. CONCLUSIONS: Our results indicate that missense/missense or missense/truncation genotypes, including the p.Arg212His variant, are associated with a relatively mild phenotype. In contrast, the truncation/truncation genotype causes rapid and severe retinal degeneration in Japanese patients with ABCA4-associated retinopathy. These data are vital in predicting patient prognosis, guiding genetic counseling, and stratifying patients for future clinical trials.

Ultraflexible Wireless Imager Integrated with Organic Circuits for Broadband Infrared Thermal Analysis.

Flexible imagers are currently under intensive development as versatile optical sensor arrays, designed to capture images of surfaces and internals, irrespective of their shape. A significant challenge in developing flexible imagers is extending their detection capabilities to encompass a broad spectrum of infrared light, particularly terahertz (THz) light at room temperature. This advancement is crucial for thermal and biochemical applications. In this study, a flexible infrared imager is designed using uncooled carbon nanotube (CNT) sensors and organic circuits. The CNT sensors, fabricated on ultrathin 2.4 µm substrates, demonstrate enhanced sensitivity across a wide infrared range, spanning from near-infrared to THz wavelengths. Moreover, they retain their characteristics under bending and crumpling. The design incorporates light-shielded organic transistors and circuits, functioning reliably under light irradiation, and amplifies THz detection signals by a factor of 10. The integration of both CNT sensors and shielded organic transistors into an 8 × 8 active-sensor matrix within the imager enables sequential infrared imaging and nondestructive assessment for heat sources and in-liquid chemicals through wireless communication systems. The proposed imager, offering unique functionality, shows promise for applications in biochemical analysis and soft robotics.

Physical findings of crowned dens syndrome.

If meningitis is suspected, head computed tomography is performed before cerebrospinal fluid collection. Crown dens syndrome can be diagnosed using simultaneous CT scans of the head and neck. Thus, unnecessary CSF tap test can be avoided.

Use of ellipsoid zone width for predicting visual prognosis after cataract surgery in patients with retinitis pigmentosa.

OBJECTIVE: To predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa by measuring ellipsoid zone (EZ) width using spectral-domain optical coherence tomography. METHODS: This retrospective study included patients with retinitis pigmentosa who underwent uncomplicated cataract surgery between December 2017 and June 2020. Preoperative best-corrected visual acuity (BCVA) and the best postoperative BCVA during follow-up were collected. EZ width was measured on preoperative cross-sectional optical coherence tomography images along the horizontal/vertical meridian through the fovea. RESULTS: Thirty-eight eyes of 38 patients (22 female; mean [±standard deviation] age, 62.1 ± 11.8 years) were included. The median preoperative logarithm of the minimum angle of resolution BCVA of 0.52 (range, 0.00-3.00) significantly improved to 0.07 (range, -0.18-3.00) after surgery (P < 0.001). On preoperative spectral-domain optical coherence tomography images, the median horizontal, vertical, and average EZ widths were 783 (range, 0-9837), 761 (range, 0-10 250), and 769 (range, 0-10 043) µm, respectively. Postoperative BCVA significantly correlated with the horizontal (r = -0.784, P < 0.001), vertical (r = -0.777, P < 0.001), and average EZ widths (r = -0.777, P < 0.001). The area under the receiver operating characteristic curve for the ability of the horizontal, vertical, and average EZ widths to discriminate eyes with and without postoperative BCVA ≤ 0.3 was 0.971, 0.960, and 0.963, respectively, with best cut-off values of 513, 608, and 515 µm, respectively. CONCLUSIONS: EZ width measurement can help predict the visual prognosis of cataract surgery in patients with retinitis pigmentosa. A preferable visual acuity prognosis can be expected in patients with an EZ width of approximately 600 µm.

Changes in Arterial Stiffness Monitored Using the Cardio-Ankle Vascular Index in Patients with Rheumatic Disease Receiving Initial Glucocorticoid Therapy: A Clinical Pilot Study.

Systemic inflammatory rheumatic diseases predispose to premature birth, accelerated atherosclerosis, and increased cardiovascular disease (CVD). While glucocorticoids (GCs) are used in various rheumatic diseases, and the associations between GC excess and increased prevalence of CVD complications are well established, the mechanisms underlying GCs' role in atheroma development are unclear. We conducted an observational study to address GC therapy's effect on arterial stiffness using the cardio-ankle vascular index (CAVI) in patients with rheumatic diseases. Twenty-eight patients with rheumatic disease received initial GC therapy with prednisolone at doses ranging from 20 to 60 mg/d. CAVI was examined at baseline and 3 and 6 months after GC therapy. Changes in CAVI and inflammatory parameters were evaluated. GC therapy increased the mean CAVI after 3 months but decreased it to pretreatment levels after 6 months. The mean CAVI substantially decreased with GC treatment in patients <65 years but increased in patients ≥65 years. Alterations in CAVI during the 6-month GC treatment negatively correlated with the lymphocyte-to-monocyte ratio (LMR) at baseline. Conversely, no correlation was observed between alterations in CAVI values and conventional inflammatory markers (C-reactive protein and erythrocyte sedimentation rate). Multivariate analysis of factors related to changes in CAVI highlighted young age, high prednisolone dosage, and LMR at baseline. GC temporarily exacerbates but eventually improves arterial stiffness in rheumatic diseases. Particularly in young patients, GC may improve arterial stiffness by reducing inflammation. Therefore, the LMR before GC therapy in rheumatic diseases may be a potential predictor of arterial stiffness.

Ten-year outcomes of cataract surgery for glaucoma management in patients with primary angle-closure disease.

PURPOSE: To investigate the long-term outcomes of cataract surgery for glaucoma management in patients with primary angle-closure disease (PACD). STUDY DESIGN: Retrospective case series. METHODS: We reviewed the medical records of 87 eyes of 87 patients with PACD who underwent uncomplicated cataract surgery alone at the Kobe City Medical Center General Hospital. Only patients with a minimum follow-up of 10 years were included. The patients were divided into PACD spectrum categories: primary angle-closure glaucoma (PACG), primary-angle closure (PAC), and primary angle-closure suspect (PACS). The treatment outcomes were compared among the 3 groups. Intraocular pressure (IOP), number of glaucoma eye drops, requirement of additional glaucoma treatment, visual field progression, and progression to glaucoma during the follow-up period were evaluated. RESULTS: Among the 87 patients, 39 had PACG; 26, PAC; and 22, PACS. Ten years after surgery, the IOP had significantly decreased from baseline in all 3 groups. The rate of requirement of additional glaucoma treatment during the follow-up period was significantly higher in the PACG group than in the other groups. Almost half of the patients with PACG required additional glaucoma treatment; of those patients, six (15.4%) underwent glaucoma surgery. Three patients (11.5%) with PAC required additional glaucoma medication. Visual field progression was observed in 28.1% of the patients with PACG. In 1 patient with PAC, the condition progressed to PACG, but there was no such progression in any of the patients with PACS. CONCLUSIONS: We confirmed that cataract surgery had a long-term (> 10 years) effect on IOP reduction in eyes with PACD. Early intervention with cataract surgery may be preferable for glaucoma management in patients with PACD.

A CASE OF RAPIDLY PROGRESSIVE RETINOPATHY ASSOCIATED WITH PEMBROLIZUMAB IMMUNOTHERAPY FOR METASTATIC UROTHELIAL CARCINOMA.

PURPOSE: To describe a novel case of bilateral rapidly progressive retinopathy after immunotherapy with pembrolizumab for metastatic urothelial carcinoma. METHODS: Case report. RESULTS: A 64-year-old man undergoing pembrolizumab immunotherapy was referred to our hospital because of bilateral acute vision loss. His best-corrected visual acuity was 20/30 in the right eye and 20/320 in the left eye, and a visual field test revealed central and paracentral scotomas in the right eye and central scotoma in the left eye. We suspected immune-related retinopathy based on the progressive photoreceptor damage with abnormal electroretinogram findings, absence of overt intraocular inflammation, and presence of malignancy. Cessation of pembrolizumab and steroid pulse therapy followed by decreasing oral prednisolone improved visual function and photoreceptor damage, although there was recurrence after pembrolizumab was restarted. CONCLUSION: We reported a case of rapidly progressive retinopathy that may have been triggered by pembrolizumab immunotherapy for metastatic urothelial carcinoma. High-dose corticosteroid pulse therapy was effective in reversing photoreceptor damage.

Serum IgG4-negative IgG4-related disease with a cardiac mass: A case report.

RATIONALE: Although IgG4-related disease (IgG4-RD) can affect various organs, its association with a cardiac mass is exceptionally rare. Here, we report a case of a woman with IgG4-RD and a cardiac mass and discuss 10 similar cases reported previously. PATIENT CONCERNS: A 65-year-old woman was referred to our hospital for chest discomfort and back pain. DIAGNOSES: In accordance with the 2019 ACR/EULAR diagnostic criteria for IgG4-RD, she was diagnosed with IgG4-RD based on dense lymphocytic infiltration on histopathology, IgG/IgG4-positive cell ratio <40%, >10/hpf IgG4-positive cells on immunostaining, and paraspinal zone soft tissue lesions in the chest. INTERVENTIONS: An external pacemaker was implanted for the complete atrioventricular block on the electrocardiogram. After the diagnosis of IgG4-RD, she was treated with glucocorticoids and rituximab. OUTCOMES: She remains under observation without disease recurrence. LESSONS: IgG4-RD are usually treated with glucocorticoids; however, in cases of a cardiac mass, life-threatening complications may occur and surgery is often needed. Combination therapy with glucocorticoids and rituximab may be effective even in patients with IgG4-RD and cardiac mass, which may avoid the need of invasive treatments, such as surgery.

Development of giant cell arteritis after vaccination against SARS-CoV2: A case report and literature review.

RATIONALE: Giant cell arteritis (GCA) is an autoimmune vasculitis that affects large and medium-sized blood vessels. The mRNA vaccine against severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) has been associated with the development of immune-mediated diseases. In this article, we present a case of GCA that developed after vaccination against SARS-CoV2. PATIENT CONCERNS: A 77-year-old man developed fever, general fatigue, and headache 1 day after the third dose of vaccination against SARS-CoV2. Nodular swelling and tenderness of the bilateral temporal arteries were observed. DIAGNOSES: Although right temporal artery biopsies were negative, the patient was diagnosed with GCA based on criteria established by the American College of Rheumatology for the classification of GCA. INTERVENTIONS: The patient received methylprednisolone 1000 mg for 3 days. This was followed by prednisolone 1 mg/kg/d, which was decreased by 10 mg every week to 30 mg. From day 16 of hospitalization, the patient received tocilizumab 162 mg/wk every other week. OUTCOMES: There was no occurrence of acute side effects. After 38 days of treatment, the condition improved and the patient was discharged from the hospital; as stated above, the dose of prednisolone was tapered to 30 mg/d. LESSONS: We experienced a case of GCA that occurred immediately after vaccination against SARS-CoV2 with an mRNA vaccine. Early signs of GCA include fever, fatigue, and headache, and often resemble those noted after vaccination against SARS-CoV2. The potential presence of GCA should be determined in individuals with persistent fever and headache after vaccination against SARS-CoV2.

Performance of ChatGPT in Board Examinations for Specialists in the Japanese Ophthalmology Society.

We investigated the potential of ChatGPT in the ophthalmological field in the Japanese language using board examinations for specialists in the Japanese Ophthalmology Society. We tested GPT-3.5 and GPT-4-based ChatGPT on five sets of past board examination problems in July 2023. Japanese text was used as the prompt adopting two strategies: zero- and few-shot prompting. We compared the correct answer rate of ChatGPT with that of actual examinees, and the performance characteristics in 10 subspecialties were assessed. ChatGPT-3.5 and ChatGPT-4 correctly answered 112 (22.4%) and 229 (45.8%) out of 500 questions with simple zero-shot prompting, respectively, and ChatGPT-4 correctly answered 231 (46.2%) questions with few-shot prompting. The correct answer rates of ChatGPT-3.5 were approximately two to three times lower than those of the actual examinees for each examination set (p = 0.001). However, the correct answer rates for ChatGPT-4 were close to approximately 70% of those of the examinees. ChatGPT-4 had the highest correct answer rate (71.4% with zero-shot prompting and 61.9% with few-shot prompting) in "blepharoplasty, orbit, and ocular oncology," and the lowest answer rate (30.0% with zero-shot prompting and 23.3% with few-shot prompting) in "pediatric ophthalmology." We concluded that ChatGPT could be one of the advanced technologies for practical tools in Japanese ophthalmology.

Genotype and phenotype characteristics of RHO-associated retinitis pigmentosa in the Japanese population.

PURPOSE: To identify the genotypic and phenotypic characteristics of rhodopsin (RHO)-associated retinitis pigmentosa (RP) in the Japanese population. STUDY DESIGN: Cross-sectional, single-center study METHODS: The medical records of 1336 patients with RP who underwent genetic testing at our clinic between November 2008 and September 2021 were reviewed, and patients with RHO variants were included. The patients were divided into class A and class B to assess genotype-phenotype correlations based on previous reports. The clinical findings, including best-corrected visual acuity (BCVA), OCT parameters (ellipsoid zone [EZ] width and central retinal thickness [CRT]), and presence of macular degeneration, of the 2 groups were compared. RESULTS: The study included 28 patients diagnosed with RHO-associated RP (class A, 19; class B, 9). The BCVA was significantly worse in class A patients than in class B patients (P = 0.045). Superior EZ width was significantly shorter in class A than in class B patients (P = 0.016). Class A patients tended to have thinner CRT and shorter inferior EZ width than those of class B patients, although this difference was not significant. Macular degeneration was observed in 61.5% of class A and 12.5% of class B patients, demonstrating that macular degeneration can be a common complication in class A variants. CONCLUSION: Patients with class A variants presented with a severer form of RP than that of patients with class B variants in the Japanese population. These results suggest that the phenotype of RHO-associated RP is linked to the location of the variants and that such a genotype-phenotype correlation is less affected by ethnicities with different genetic backgrounds.

Detailed Evaluation of Chromatic Pupillometry and Full-Field Stimulus Testing to Assess Ultralow Vision in Retinitis Pigmentosa.

Purpose: Novel therapeutic options, such as regenerative medicine and gene therapy, are now emerging as viable treatment options for patients with severe visual impairments, such as retinitis pigmentosa (RP). Gradable assessment of patients' visual function is essential to consider treatment options and to evaluate treatment outcomes; however, evaluation of visual function in patients with advanced low vision is often challenging because of patients' poor and sometimes unpredictable responses. In this study, we attempted to accurately assess visual capabilities and disease stage in patients with RP with a visual acuity (VA) of ≤ 0.01. Design: Retrospective analysis of visual function indicators, including VA, retinal thickness, full-field stimulus testing (FST), and chromatic pupillometry. Subjects: Overall, 43 patients (84 eyes) with advanced RP with a VA of ≤ 0.01 visited Kobe City Eye Hospital from 2019 to 2021. Methods: Hierarchical (multilevel) Bayesian modeling was used to estimate individual eye's pupil response and FST threshold, taking into account the ambiguity and randomness often observed in patients with ultralow vision. Using the estimated ability obtained from each test, the correlation between each test and retinal thickness was further analyzed to make a comprehensive assessment of the data. Main Outcome Measures: Visual acuity, retinal thickness, FST threshold, and pupil diameter change to different light stimuli. Results: Full-field stimulus testing and pupillometry measurements were moderately correlated with VA but exhibited a wide range of values within the same VA groups. Full-field stimulus testing was not correlated with central retinal thickness at counting fingers/hand motion VA range and seemed to reflect overall remaining photoreceptor function, including peripheral retina. Pupillometry may be able to distinguish between different levels of inner retinal function. Conclusions: The combination of pupillometry and FST allowed for graded evaluation of visual function within patients grouped in the same VA groups in patients with advanced RP with ultralow vision. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

Recent Progress in Development of Carbon-Nanotube-Based Photo-Thermoelectric Sensors and Their Applications in Ubiquitous Non-Destructive Inspections.

The photo-thermoelectric (PTE) effect in electronic materials effectively combines photo-absorption-induced local heating and associated thermoelectric conversion for uncooled and broadband photo-detection. In particular, this work comprehensively summarizes the operating mechanism of carbon nanotube (CNT)-film-based PTE sensors and ubiquitous non-destructive inspections realized by exploiting the material properties of CNT films. Formation of heterogeneous material junctions across the CNT-film-based PTE sensors, namely photo-detection interfaces, triggers the Seebeck effect with photo-absorption-induced local heating. Typical photo-detection interfaces include a channel-electrode boundary and a junction between P-type CNTs and N-type CNTs (PN junctions). While the original CNT film channel exhibits positive Seebeck coefficient values, the material selections of the counterpart freely govern the intensity and polarity of the PTE response signals. Based on these operating mechanisms, CNT film PTE sensors demonstrate a variety of physical and chemical non-destructive inspections. The device aggregates broad multi-spectral optical information regarding the targets and reconstructs their inner composite or layered structures. Arbitrary deformations of the device are attributed to the macroscopic flexibility of the CNT films to further monitor targets from omni-directional viewing angles without blind spots. Detection of blackbody radiation from targets using the device also visualizes their behaviors and associated changes.