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We then promptly reduced the dose of PSL to below a physiological dosage (5 mg/day) over 3 weeks without relapse after episodes of SIPS and quickly resolved psychiatric symptoms. CsA in combination with PSL can reduce the initial dosage of PSL, shorten the time to remission, and easily maintain clinical remission. This protocol appears clinically useful and potentially applicable as a future treatment strategy for FRNS troubled by SIPS. Copyright © 2020 by S. Karger AG, Basel.Purpose To present the natural course and describe the characteristic findings of a case of unilateral retinal pigment epithelium dysgenesis (URPED) and highlight the optical coherence tomography angiography characteristics of this rare fundus pattern. Methods Case report. Results A 52-year-old male was referred 8 years ago to our clinic due to a distinctive unilateral lesion in his left fundus, of which he was aware from early adulthood. Clinical evaluation revealed an irregularly shaped patch of retinal pigment epithelium atrophy surrounding his left optic nerve, with a characteristic scalloped border and with severe distortion of the overlying retinal and vascular tissue. Retinal pigment epithelial hyperplasia was noted in the margin as well as in distinct lacunae clustered around the periphery of the lesion. Right fundus examination was normal. The patient was diagnosed with URPED and was followed annually with multimodal imaging ever since. No choroidal neovascularization or other complication was noted. However, the lesion appeared to slowly but steadily grow to eventually affect the foveal region causing severe visual loss. Best corrected visual acuity was 20/40 at baseline and 20/200 at last follow-up 8 years after the original diagnosis. Conclusion URPED is a rare retinal disease with unique clinical characteristics that can progress relentlessly even in the absence of secondary complications. Copyright © 2019 by S. Karger AG, Basel.Background There are limited reports of intra-arterial chemotherapy (IAC) for retinoblastoma (RB) from developing world. Objectives In this study, we report our 4-year experience of IAC for RB from India. Methods Retrospective, interventional case series. Primary outcome measures included tumor regression, vitreous seeds and subretinal seeds control, and globe salvage. Secondary outcome measures were best-corrected visual acuity and treatment complications. Results Fifteen eyes underwent 53 IAC procedures over mean 28.6 ± 13.8 months (range 10-51 months). IAC was employed as primary (n = 6) or secondary (n = 9) chemotherapy. Following IAC, complete regression of main tumor was seen in 7 eyes (47%) and partial regression in 3 (20%) eyes. Enucleation was done in 5(33%) eyes. Globe salvage rates were achieved in 1 eye (100%) in group B, 2 eyes (67%) in group C (n = 3), 6 eyes (67%) in group D (n = 9), and 1 eye (50%) in group E (n = 2). Following IAC, Kaplan-Meier survival curve showed 93% globe survival rate at 1 year, 76% at 2 years, and 66% at 3, and 4 years. Conclusion IAC has enhanced globe salvage rates in eyes with RB. Multicenter studies with longer follow-up are necessary to better understand outcomes in the long term. Copyright © 2019 by S. Karger AG, Basel.Objectives The aim of this study was to train and validate deep learning algorithms to quantitate relative amyloid deposition (RAD; mean amyloid deposited area per stromal area) in corneal sections from patients with familial amyloidosis, Finnish (FAF), and assess its relationship with visual acuity. Methods Corneal specimens were obtained from 42 patients undergoing penetrating keratoplasty, stained with Congo red, and digitally scanned. Areas of amyloid deposits and areas of stromal tissue were labeled on a pixel level for training and validation. The algorithms were used to quantify RAD in each cornea, and the association of RAD with visual acuity was assessed. Results In the validation of the amyloid area classification, sensitivity was 86%, specificity 92%, and F-score 81. For corneal stromal area classification, sensitivity was 74%, specificity 82%, and F-score 73. There was insufficient evidence to demonstrate correlation (Spearman's rank correlation, -0.264, p = 0.091) between RAD and visual acuity (logMAR). Conclusions Deep learning algorithms can achieve a high sensitivity and specificity in pixel-level classification of amyloid and corneal stromal area. Further modeling and development of algorithms to assess earlier stages of deposition from clinical images is necessary to better assess the correlation between amyloid deposition and visual acuity. The method might be applied to corneal dystrophies as well. Copyright © 2019 by S. Karger AG, Basel.Purpose Histologic correlation of clinical patterns of recurrent choroidal melanoma following I-125 plaque brachytherapy was performed to identify pathologic mechanisms of recurrence. Methods We reviewed 7 cases of recurrent choroidal melanoma following I-125 plaque brachytherapy managed with enucleation. Clinical characteristics included tumor dimensions, radiation dose, time to local recurrence, and clinical pattern of recurrence. Histopathology (hematoxylin and eosin and periodic acid - Schiff) and immunohistochemistry (Ki-67, CD-163, HMB45, and SOX10) were performed. Dapansutrile NLRP3 inhibitor Results Mean follow-up time and time to local recurrence were 42 and 21 months after brachytherapy, respectively. Tumor recurrences were described clinically as marginal in 43%, diffuse in 29%, and extraocular extension (EOE) in 29%. Eighty-six percent were classified as mixed cell type and 14% were epithelioid type. Tumor zonation (histologic demarcation between zones of recurrent and nonrecurrent tumor cells by immunohistochemistry) was present in marginal and EOE cases (n = 6) and absent in the diffuse cases (n = 2). Ki-67 proliferative index was higher in marginal and EOE recurrences, while diffuse cases showed uniform -Ki-67 staining. CD-163 staining was found to be greater in nonrecurrent tumor. HMB45 correlated with SOX10 with a greater staining in recurrent tumor. Conclusion Our observations provide a correlation between histopathologic and clinical patterns of local recurrence of choroidal melanoma after brachytherapy. Copyright © 2019 by S. Karger AG, Basel.