Artificial intelligence-based model for predicting pulmonary arterial hypertension on chest x-ray images.

BACKGROUND: Pulmonary arterial hypertension is a serious medical condition. However, the condition is often misdiagnosed or a rather long delay occurs from symptom onset to diagnosis, associated with decreased 5-year survival. In this study, we developed and tested a deep-learning algorithm to detect pulmonary arterial hypertension using chest X-ray (CXR) images. METHODS: From the image archive of Chiba University Hospital, 259 CXR images from 145 patients with pulmonary arterial hypertension and 260 CXR images from 260 control patients were identified; of which 418 were used for training and 101 were used for testing. Using the testing dataset for each image, the algorithm outputted a numerical value from 0 to 1 (the probability of the pulmonary arterial hypertension score). The training process employed a binary cross-entropy loss function with stochastic gradient descent optimization (learning rate parameter, α = 0.01). In addition, using the same testing dataset, the algorithm's ability to identify pulmonary arterial hypertension was compared with that of experienced doctors. RESULTS: The area under the curve (AUC) of the receiver operating characteristic curve for the detection ability of the algorithm was 0.988. Using an AUC threshold of 0.69, the sensitivity and specificity of the algorithm were 0.933 and 0.982, respectively. The AUC of the algorithm's detection ability was superior to that of the doctors. CONCLUSION: The CXR image-derived deep-learning algorithm had superior pulmonary arterial hypertension detection capability compared with that of experienced doctors.

Treatment Effects of Switching to Faricimab in Eyes with Diabetic Macular Edema Refractory to Aflibercept.

Background and Objectives: Faricimab is a vascular endothelial growth factor A and angiopoietin-2 bispecific antibody. It is a novel therapeutic approach distinct from previous anti-vascular endothelial growth factor agents. This study aimed to evaluate the efficacy of switching from aflibercept to faricimab in the treatment of diabetic macular edema (DME) refractory to aflibercept, with a specific focus on the resolution of macular edema. Materials and Methods: The medical records of 29 eyes of 21 patients with DME that were refractory to intravitreal injections of aflibercept (IVAs) and who had completed the clinical follow-up of at least four intravitreal injections of faricimab (IVFs) were reviewed. The central retinal thickness (CRT), best-corrected visual acuity (BCVA), and the mean period (weeks) until the next injection were measured after the second-to-last IVA, first-to-last IVA, last IVA, and first to fourth IVFs following the transition to IVF. Results: The mean time from the first IVF to the assessment of effectiveness was significantly shorter than the time to the last IVA; however, no significant difference was found in the time from the second, third, and fourth IVFs to the assessment. The mean CRTs after the first and second IVFs were not significantly different from the CRT after the last IVA, but the mean CRT after the third and fourth IVFs was significantly thinner than that after the last IVA (p = 0.0025 and p = 0.0076, respectively). The mean BCVAs after the third and fourth IVFs significantly improved compared with that after the last IVA (p = 0.0050 and p = 0.0052, respectively). Conclusions: When switching the treatment to IVF for eyes with IVA-resistant DME, better treatment outcomes are achieved if IVF is performed three or more times.

A novel Menin-MLL1 inhibitor, DS-1594a, prevents the progression of acute leukemia with rearranged MLL1 or mutated NPM1.

BACKGROUND: Mixed lineage leukemia 1-rearranged (MLL1-r) acute leukemia patients respond poorly to currently available treatments and there is a need to develop more effective therapies directly disrupting the Menin‒MLL1 complex. Small-molecule-mediated inhibition of the protein‒protein interaction between Menin and MLL1 fusion proteins is a potential therapeutic strategy for patients with MLL1-r or mutated-nucleophosmin 1 (NPM1c) acute leukemia. In this study, we preclinically evaluated the new compound DS-1594a and its salts. METHODS: We evaluated the preclinical efficacy of DS-1594a as well as DS-1594a·HCl (the HCl salt of DS-1594a) and DS-1594a·succinate (the succinic acid salt of DS-1594a, DS-1594b) in vitro and in vivo using acute myeloid leukemia (AML)/acute lymphoblastic leukemia (ALL) models. RESULTS: Our results showed that MLL1-r or NPM1c human leukemic cell lines were selectively and highly sensitive to DS-1594a·HCl, with 50% growth inhibition values < 30 nM. Compared with cytrabine, the standard chemotherapy drug as AML therapy, both DS-1594a·HCl and DS-1594a·succinate mediated the eradication of potential leukemia-initiating cells by enhancing differentiation and reducing serial colony-forming potential in MLL1-r AML cells in vitro. The results were confirmed by flow cytometry, RNA sequencing, RT‒qPCR and chromatin immunoprecipitation sequencing analyses. DS-1594a·HCl and DS-1594a·succinate exhibited significant antitumor efficacy and survival benefit in MOLM-13 cell and patient-derived xenograft models of MLL1-r or NPM1c acute leukemia in vivo. CONCLUSION: We have generated a novel, potent, orally available small-molecule inhibitor of the Menin-MLL1 interaction, DS-1594a. Our results suggest that DS-1594a has medicinal properties distinct from those of cytarabine and that DS-1594a has the potential to be a new anticancer therapy and support oral dosing regimen for clinical studies (NCT04752163).

Long-term follow-up of retinal morphology and physiology after 2000 mg sildenafil overdose as a means of attempted suicide: a case report.

BACKGROUND: Few case reports have described sildenafil overdose, particularly ingestion of > 1000 mg, and overdose-induced changes in visual function remain unclear. We report retinal morphology, retinal sensitivity, and findings of electrophysiological evaluation over long-term follow-up in a case of sildenafil overdose (2000 mg). CASE PRESENTATION: Our patient developed visual abnormalities in the paracentral visual field accompanied by photophobia, decreased contrast sensitivity, and difficulty distinguishing colors in both eyes, 8 hours after the sildenafil overdose. These symptoms did not improve throughout the course, and although abnormalities of retinal morphology and sensitivity, as well as the electroretinogram findings showed slight improvement, the patient did not recover completely at 6-month follow-up. CONCLUSIONS: We observed that high-dose sildenafil ingestion leads to retinal toxicity; the ocular abnormalities may persist for at least 6 months. Optical coherence tomography, Humphrey perimetry, microperimetry, and multifocal electroretinography are useful to quantitatively monitor temporal changes.

Optical coherence tomography findings in three patients with Werner syndrome.

BACKGROUND: Werner syndrome is a rare, autosomal recessive disorder characterised by premature aging. It is a typical hereditary progeroid syndrome that can be difficult to diagnose owing to its rarity and the similarity of some of its symptoms, such as juvenile cataracts, to other common ophthalmologic conditions. Early onset of bilateral cataracts is currently used as the ophthalmological feature for Werner syndrome; however, ophthalmologists often find performing a detailed examination of the medical history and genetic testing for Werner syndrome at the time of an ophthalmologic consultation challenging. If a unique ocular finding was observed on ocular examinations in cases of juvenile bilateral cataracts, we could consider Werner syndrome as a differential diagnosis.  CASE PRESENTATION: We documented the cases of three patients with Werner syndrome in whom thinning of the retina in the retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) were observed using optical coherence tomography (OCT). Visual field tests revealed the loss of visual field mainly owing to glaucoma. The thinnig of the choroidal thickness (CT) in three patients was also observed using enhanced depth imaging (EDI)-OCT. CONCLUSIONS: Three patients have thinning of the RNFL, GCC, and choroidal thickness and the loss of visual field. These findings suggest the need for including Werner syndrome in the differential diagnosis when patients presenting with juvenile cataracts of unknown cause also show abnormal retinal and choroidal thinning in the OCT images.

Comparisons of surgical outcomes after epiretinal membrane peeling in the myopic eyes with long to that with normal axial length.

PURPOSE: To compare the outcomes of vitrectomy with removal of an idiopathic epiretinal membrane (ERM) in the myopic eyes with long axial length (AL) to that in the eyes with normal AL. METHODS: This was a retrospective, observational, case-control study. Fifty-six eyes of 56 patients with an idiopathic ERM were studied. Twenty-eight of these eyes had an axial length longer than 26.0 mm (Group A), and the other 28 eyes had axial lengths < 26.0 mm (Group B). The age and visual acuity of the two groups were not significantly different. All subjects were treated by vitrectomy and peeling of the ERM with a 25- or 27-gauge system. The postoperative best-corrected visual acuity (BCVA) and optical coherence tomographic findings were determined at 3 and 6 months postoperatively. RESULTS: The mean BCVA improved from 0.35 ± 0.25 to 0.15 ± 0.25 logMAR units in the eyes with the long AL and from 0.35 ± 0.25 to 0.10 ± 0.21 logMAR units in the eyes with normal AL at 6 months postoperatively (both P < 0.001). The postoperative BCVA was not significantly different between the two groups at 6 months (P = 0.221). The central macula was thinner in the eyes of Group A than Group B (P < 0.001). The restorations of the ellipsoid zone and the external limiting membrane were observed in both groups, but the interdigitation zone was less frequently restored in Group A (P < 0.001). CONCLUSIONS: Vitrectomy with the removal of the epiretinal membrane is effective in attaining a good BCVA even in the myopic eyes with long AL.

Refractive errors after sutureless intrascleral fixation of intraocular lens.

PURPOSE: To determine the difference between the attempted and the achieved postoperative refractive error after sutureless intrascleral fixation of the haptics of an implanted intraocular lens (IOL). PATIENTS AND METHODS: This was a retrospective study of the medical charts of patients who had undergone sutureless intrascleral fixation of an IOL. The IOLs were fixed by inserting the haptics into scleral tunnels with or without flanges. The differences between the attempted and achieved postoperative refractive error (spherical equivalent) were determined. The relationships between the surgical options and the differences of the refractive error and degree of astigmatism were also determined. RESULTS: Two hundred and twenty-three eyes were studied. There was a myopic shift of - 0.38 ± 1.13 diopters (D). The mean of the differences between the achieved postoperative and the attempted refractive error was 0.89 ± 0.79 D. The achieved refractive error was significantly correlated with the attempted refractive error (P < 0.001, R2 = 0.631). The surgical procedures selected were not significantly associated with the differences between the attempted and achieved refractive error. The size of sclerocorneal incision and presence of sutures were significantly associated with the final degree of astigmatism (P = 0.006 and 0.008, respectively). CONCLUSIONS: The postoperative refractive error was significantly correlated with the attempted refractive error after intrascleral fixation of an IOL. The wound construction was associated with the postoperative degree of astigmatism.

Review of clinical studies and recommendation for a therapeutic flow chart for diabetic macular edema.

Diabetic macular edema (DME), characterized by exudative fluid accumulation in the macula, is the most common form of sight-threatening retinopathy in patients with diabetes. The management of DME has changed considerably in recent years, especially following the development of intravitreal anti-vascular endothelial growth factor therapy which has emerged as a first-line therapy for center-involved DME. Laser treatment, intravitreal steroid therapy, and vitrectomy are also important treatment options for DME. We believe that it is important to choose the most appropriate treatment option for DME based on the clinical evidences, in addition to the careful consideration of individual patients' general or ocular condition, DME characteristics, patients' motivation, and compliance to the treatment in real-world clinical practice. In this review, we have summarized important clinical evidences for the main treatments for DME, presented an expert review for these evidences, and proposed a recommended therapeutic flow chart for DME. We hope that our review of the clinical evidences and the recommended therapeutic flow chart for DME will contribute to better treatment outcome for DME.

SIX MONTHS PRIMARY SUCCESS RATE FOR RETINAL DETACHMENT BETWEEN VITRECTOMY AND SCLERAL BUCKLING.

PURPOSE: To compare clinical outcomes between pars plana vitrectomy (PPV), scleral buckling (SB), and PPV+SB for rhegmatogenous retinal detachment in the Japan-RD Registry. METHODS: This is a nation-wide, multicenter, observational study based on the registry data between 2016 and 2017. The failure levels were defined as Level 1 (a failure of retinal detachment repair), Level 2 (remaining silicone oil), and Level 3 (multiple surgeries to achieve reattachment). We compared cases treated by SB or PPV in the subgroup of simple rhegmatogenous retinal detachment using multivariate Cox proportional hazard models. RESULTS: A total of 2,775 cases were included. Overall, 6 months any levels of failure in total, SB, PPV, and PPV+SB were 9.2% (n = 256), 6.9% (n = 48), 8.2% (n = 157), and 21.3% (n = 51), respectively. Poor visual acuity at baseline in SB and inferior rhegmatogenous retinal detachment and larger retinal tear in PPV were associated with a higher risk of failure. Pars plana vitrectomy was associated with a higher chance of achieving primary success in cases with simple RRD, especially for cases with superior RRD (adjusted hazard ratio 3.61, 95% confidence interval 2.22-5.94, P < 0.001). CONCLUSION: In this nationwide study, surgical anatomic outcomes were equally successful in either SB or PPV. There were different baseline characteristics associated with primary success between SB and PPV.

Ten-year epidemiological study of ocular and orbital tumors in Chiba University Hospital.

BACKGROUND: The purpose of this study is to determine the epidemiology of tumors of the ocular adnexa and orbit in Japan. METHODS: We conducted a retrospective study on the histopathological reports in the medical records of the Chiba University Hospital from April 2009 to March 2019. Three hundred and seventy two records were examined. In addition, we examined the annual changes in the major types of tumors including malignant lymphomas and IgG4-related diseases (IgG4-RDs). RESULTS: There were 270 conjunctival or eyelid tumors with 166 benign and 104 malignant. There were 102 orbital tumors with 55 benign, 47 malignant tumors, and 21 cases of IgG4-RDs. Ten cases of adenoma (2.7%), another benign tumor, was also diagnosed. The major malignant tumors were malignant lymphoma in 74 cases, sebaceous gland carcinoma (SGC) in 28 cases, basal cell carcinoma in 15 cases, and squamous cell carcinoma in 8 cases. The SGCs were the most common malignant eyelid tumor at 54%. Among the malignant lymphomas, extranodal marginal zone lymphomas of the mucosa-associated lymphoid tissue type, MALT lymphomas, was the most common at 51 cases and the second most common was the diffuse large B-cell lymphoma at 11 cases. The ratio of MALT lymphomas to that of all malignant lymphomas increased significantly with years. The serum IgG4 values were measured more often in the last 5 years (70%) than in the former 5 years (33%). CONCLUSIONS: We conclude that malignant lymphoma is a major malignant tumor in Japan and pathological biopsies should be done proactively to prevent missing IgG4-positive MALT lymphomas.

Comparison of Internal Limiting Membrane Peeling and Flap Removal to Flap Insertion on Visual Outcomes in Highly Myopic Eyes with Macular-Hole Retinal Detachment.

INTRODUCTION: To compare the morphological and visual outcomes after pars plana vitrectomy (PPV) with internal limiting membrane (ILM) peeling and flap removal to that with flap insertion in eyes with myopic macular-hole retinal detachment (MHRD). METHODS: Forty-six eyes of 45 patients with MHRD were studied. Eighteen eyes were treated by PPV with ILM peeling and flap removal and 28 eyes by flap insertion, respectively. The baseline characteristics and postoperative findings were compared between the 2 groups. RESULTS: The average age of the cases was 70.0 ± 8.5 years. The best-corrected visual acuity (BCVA) improved, from 1.51 ± 0.14 to 1.31 ± 0.20 logMAR units in the flap removal group, and from 1.20 ± 0.10 to 0.88 ± 0.10 logMAR units in the flap insertion group (p = 0.049). A significantly larger number of MHs were closed in the flap insertion group (96 vs. 50% in the removed group; p < 0.001). Restoration of the ellipsoid zone (EZ) and external limiting membrane (ELM) was also better in the flap insertion group than in the flap removal group (EZ 14 vs. 6%, p = 0.003; ELM 36 vs. 6%, p < 0.001). DISCUSSION: The technique of ILM peeling with flap insertion is more effective in closing the MH, and results in better BCVA and better restoration of the outer retinal microstructures than flap removal.

Improvements of Retinal Sensitivity after Intravitreal Injection of Aflibercept in Eyes with Neovascular Age-Related Macular Degeneration with or without Polypoidal Choroidal Vasculopathy.

PURPOSE: We aim to determine the effects of intravitreal aflibercept (IVA) on the mean sensitivity (MS) of the central retina, best-corrected visual acuity (BCVA), and central foveal thickness (CFT) in eyes with neovascular age-related macular degeneration (nAMD) with or without polypoidal choroidal vasculopathy (PCV). METHODS: This was a prospective, interventional study. All eyes were treatment-naive with nAMD with or without PCV. Each eye received 3 monthly IVA injections followed by an IVA injection every 2 months for 12 months. The primary outcome was the change in the MS within the central 2°. The secondary outcomes were the changes in BCVA, CFT, greatest linear dimension (GLD), and percentage of eyes with a dry macula. RESULTS: Thirty-seven eyes of 37 patients were studied. A significant improvement of the MS (dB) was observed +4.9 ± 4.6 dB (mean ± standard deviation) at 3 M (p < 0.001), +5.5 ± 4.9 dB at 6 (p < 0.001), and +7.0 ± 3.4 dB at 12 M (p < 0.001) compared to the baseline in all eyes. The MS of the eyes with non-PCV was not significantly different from that of eyes with PCV (p = 1.00, 1.00, 1.00, and 0.76 at baseline, 3, 6, and 12 M, respectively). The MS of 11 patients whose BCVA remained unchanged was significantly improved by +6.5 ± 2.8 dB at 3 M (p < 0.001), +6.1 ± 4.3 dB at 6 M (p < 0.001), and +6.4 ± 4.8 dB at 12 M (p = 0.003) compared to the baseline. The mean BCVA was significantly improved from the baseline to 3 M (p < 0.001), 6 M (p = 0.027), and 12 M (p = 0.003) in all eyes. The BCVA was improved or maintained in 97% of the patients at 12 M. The mean CFT and GLD were significantly reduced at 12 M (p < 0.001). Twenty-two eyes (71%) had a dry macula at 12 M. CONCLUSIONS: IVA administered by a fixed dosing regimen led to significant improvements of the central MS, BCVA, and macular morphology at 1 year in eyes with nAMD with or without PCV. These results were not significantly different between eyes with non-PCV and with PCV. The improvements of the MS of the retina of the central 2° in a subgroup whose BCVA remained unchanged through the 12-month experimental period was also significant. We conclude that the MS of the central 2° might be a better marker than the BCVA in determining the effectiveness of IVA treatments and might be helpful in determining early effects on the retina before BCVA changes can be detected.

Detecting Diabetic Retinal Neuropathy Using Fundus Perimetry.

Fundus perimetry is a new technique for evaluating the light sense in the retina in a point-to-point manner. Light sense is fundamentally different from visual acuity, which measures the threshold for discriminating and perceiving two points or lines, called the minimum cognoscible. The quality of measurement of retinal sensitivity has dramatically increased in the last decade, and the use of fundus perimetry is now gaining popularity. The latest model of fundus perimetry, MP-3, can be used for a wide range of measurements and has an advanced eye tracking system. High background illumination enables accurate measurement of mesopic retail sensitivity. Recent investigations have shown that neuronal damage precedes vascular abnormalities in diabetic retinopathy. The loss of retinal function has also been reported prior to morphological changes in the retina. In this review, the importance of measuring retinal sensitivity to evaluate visual function in the early stages of diabetic retinopathy was discussed. The usefulness of retinal sensitivity as an outcome measure in clinical trials for treatment modalities is also presented. The importance of fundus perimetry is promising and should be considered by both diabetes researchers and clinical ophthalmologists.

Surgical outcomes of vitrectomy for breakthrough vitreous hemorrhage in eyes with exudative age-related macular degeneration.

PURPOSE: To report the outcomes of pars plana vitrectomy in cases with breakthrough vitreous hemorrhage (VH) secondary to exudative age-related macular degeneration (AMD) and polypoidal choroidal vasculopathy. We also investigated the relationship between the preoperative b-mode ultrasonographic findings and the postoperative visual acuity to determine if the ultrasonographic findings can predict the surgical outcome after pars plana vitrectomy. METHODS: This was a retrospective, interventional, case series. Twenty eyes of 20 patients were studied. The associations between the pre- and intraoperative factors and the final best-corrected visual acuity (BCVA) were determined. Recombinant tissue-plasminogen activator (tPA) was used in cases with massive hemorrhagic retinal detachment. RESULTS: Ten eyes with polypoidal choroidal vasculopathy (PCV), two eyes with choroidal neovascularization (CNV), and eight eyes with an unknown type of AMD were studied. The mean BCVA was 0.73 ± 0.57 logarithm of the minimum angle of resolution (logMAR) units before developing the VH, 2.25 ± 0.45 logMAR units before the surgery, and 1.52 ± 0.87 logMAR units after the surgery. The BCVA improved significantly after the surgery (P = 0.004) but was significantly worse than that before developing the VH (P = 0.012). The cases of PCV had better final BCVA than cases of CNV (P = 0.043, Mann-Whitney test). The preoperative presence of a subretinal elevation at the macula detected by ultrasonography was significantly associated with a poorer final BCVA (P = 0.031). CONCLUSIONS: Vitrectomy significantly improved visual function in the eyes with VH associated with exudative AMD. The eyes with PCV and no macular subretinal elevation on ultrasonography had a better visual prognosis.

Preoperative factors to select vitrectomy or scleral buckling for retinal detachment in microincision vitrectomy era.

BACKGROUND: To determine the preoperative factors associated with selecting scleral buckling (SB) or pars plana vitrectomy (PPV) to treat rhegmatogenous retinal detachments (RRDs) in the era of microincision vitrectomy surgery. METHODS: A nationwide, multicenter, observational study in the Japan Retinal Detachment Registry was done. From February 2016 to March 2017, 3446 cases were registered, and 3219 among them were analyzed. The factors related to the patient or surgeon were selected; the surgeon's factor included years of experience, number of cases registered, and current activity. The odds ratio (OR) for factors associated with SB or PPV was estimated by a mixed logistic regression model. RESULTS: SB was selected in 24.2% (779/3219), PPV was in 71.5% (2238/3219), and PPV+SB was in 6.3% (202/3219). Multivariate analysis showed that age [< 70 years old, OR 4.27, 95% confidence interval (CI) 2.34-7.79, P < 0.001], visual acuity [1.0+ (OR 7.41, 95% CI 3.47-15.82, P < 0.001)], pseudo-phakia (OR 4.83, 95%CI 2.11-10.56, P < 0.001), retinal tear (OR 6.92, 95% CI 4.83-9.92, P < 0.001), breaks at/near the vitreous base (OR 8.07, 95% CI 3.50-18.62, P < 0.001), 90+° retinal tear (OR 108.24, 95% CI 7.44-1574.34, P < 0.001), and number of breaks 4+ (OR 3.15, 95% CI 1.95-5.09, P < 0.001), were significantly associated with selecting PPV over SB. The surgeons' activity defined as the number of cases experienced during the registry period was related to the selection of PPV (OR 3.83, 95% CI 2.11-6.93, P < 0.001). CONCLUSIONS: The choice of PPV for the RRD was associated not only with patients' preoperative factors but also the surgeon's activity. Active surgeons selected PPV more than SB.

REGIONAL DENSITIES OF RETINAL CAPILLARIES AND RETINAL SENSITIVITIES AFTER MACULAR HOLE SURGERY WITH INTERNAL LIMITING MEMBRANE PEELING.

PURPOSE: To measure the vascular density (VD) of the retinal capillary plexuses by optical coherence tomography angiography (OCTA) after surgery for an idiopathic macular hole. METHODS: Retrospective, observational case series.Sixteen eyes of 16 patients with an idiopathic macular hole underwent vitrectomy with internal limiting membrane peeling. The VDs of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) were determined by OCTA, and the retinal sensitivity (RS) and the inner retinal thickness (IRT) were measured before, and at 3, 6, and 12 months after the surgery. The VD, RS, and IRT were measured at the four parafoveal quadrants. RESULTS: The mean age was 68.9 years. The VDs of the SCP and DCP were significantly correlated with the RS and IRT at 12 months postoperatively (all P < 0.001 for both SCP and DCP). The VDs of the SCP and DCP were higher, the RS more sensitive, and the IRT thicker in the nasal than the temporal quadrant at 12 months (P < 0.001, <0.001, =0.009, <0.001, respectively). CONCLUSION: The significant correlation between the VDs of the SCP and DCP and the RS and IRT may be due to the nasal shift of the posterior retina.

Non-paraneoplastic autoimmune retinopathy that developed in fellow eye 10 years after onset in first eye: a case report.

BACKGROUND: Evidence-based criteria for the treatment of autoimmune retinopathy (AIR) have not been established. The pathology and clinical features of each antibody causing AIR, and its long-term course are still undetermined. We report our findings in a case of non-paraneoplastic AIR (npAIR) that developed in the fellow eye 10 years after the onset in the first eye. CASE PRESENTATION: Our patient had photophobia in both eyes and a rapidly progressing visual field defect in his right eye at the initial examination. He was diagnosed with non-paraneoplastic AIR based on the clinical findings and immunoblot analyses for anti-retinal antibodies, and he was treated with steroids. Ten years later, a visual field defect developed in the fellow eye, and a diagnosis of npAIR was made. Immunoblot analyses were positive for anti-α-enolase antibodies. He was treated with steroids, immunosuppressants, and plasma exchange. However, the response to the treatment was poor and both eyes eventually became blind. CONCLUSIONS: As best we know, this is the first case report of npAIR that developed in the fellow eye over 10 years after the development in the first eye. Long-term follow-up and a search for tumor lesions are necessary in cases of npAIR. Further understanding of the long-term course of AIR can contribute to an understanding of the pathology and treatment of npAIR.

Comparison of the Efficacy of Sub-Tenon versus Intravitreal Triamcinolone Acetonide Injection during Cataract Surgery for Diabetic Macular Edema.

PURPOSE: We compared the efficacy of sub-Tenon triamcinolone acetonide (STTA) to intravitreal triamcinolone aceto-nide (IVTA) injections during cataract surgery (CS) for patients with diabetic macular edema (DME). METHODS: The medical records of 33 eyes (26 patients) with DME which had undergone CS with STTA were compared to those of 34 eyes (27 patients) with DME which had undergone CS with IVTA. Central foveal thickness and best-corrected visual acuity (BCVA) were measured at the baseline and 1, 3, and 6 months after the surgery. RESULTS: The BCVAs after STTA and IVTA were significantly improved at 3 and 6 months. Thirteen eyes in the IVTA group and 21 eyes in the STTA group required other therapies (p < 0.05). One case developed intraocular pressure elevation after IVTA and underwent selective la ser trabeculoplasty. CONCLUSIONS: Ophthalmologists should consider the merits and demerits of IVTA and STTA for DME treatment after CS.

Case of convulsive seizure developing during electroretinographic recordings: a case report.

BACKGROUND: To present our findings in a case of convulsive seizures and loss of consciousness that developed during recording electroretinograms (ERG). CASE PRESENTATION: A 34-year-old man had reduced vision in his left eye for about 15 years, and night blindness for about two years. His visual acuity was 20/15 in the right eye and 20/50 in the left eye. The fundus was normal but the sensitivity in the macular region of the left eye was decreased. Optical coherence tomography (OCT) showed partial loss of the interdigitation zone. Upon completion of the flicker ERG recording, a paralysis developed in both upper limbs, then convulsions of the lower limbs followed by a loss of consciousness. The convulsions disappeared after an intravenous injection of diazepam. After that incident, he reported that he had had previous conscious-loss seizures. CONCLUSIONS: Photosensitive epileptic seizures can occur with the light stimuli used for conventional ERG recordings. We recommended that clinicians request information on any prior seizure episodes of the patients and their family members before ERG recordings.

The Case of IgG4-related Ophthalmic Disease Accompanied by Compressive Optic Neuropathy.

A 76-year-old woman was diagnosed with thyroid-associated orbitopathy (TAO) associated with hypothyroidism at another clinic. Magnetic resonance imaging showed enlargements of the extraocular muscles. Because her best-corrected visual acuity (BCVA) was 0.01OD, she was referred to the Chiba University Hospital. She had an elevation of serum IgG4. She was diagnosed with possible IgG4-related ophthalmic disease (IgG4-ROD) accompanied by optic nerve compression with the mass. She underwent methylprednisolone pulse therapy, and, two months later, her BCVA and visual field defect were greatly improved. Our findings indicate that ophthalmologists should be aware that IgG4-ROD can be masked by lesions associated with TAO.