CLEC3B is a novel causative gene for macular-retinal dystrophy.

Purpose: Macular degeneration is the leading cause of blindness worldwide. In this study, we aimed to define a new subtype of macular-retinal dystrophy and its genetic predisposition in 5 families.

Methods: Exome sequencing was performed to determine the putative disease-causing genes in patients with inherited macular disorders confirmed through comprehensive ophthalmic examinations.

Comparison of combination therapy of prednisolone and cyclosporine with corticosteroid pulse therapy in Vogt-Koyanagi-Harada disease.

Purpose: To compare the efficacy and safety of a combination therapy of prednisolone and cyclosporine and corticosteroid pulse therapy in Vogt-Koyanagi-Harada (VKH) disease.

Study Design: A prospective, multicenter, randomized, non-inferiority trial.

Methods: Patients of new-onset acute VKH disease at 11 centers in Japan between 2014 and 2018 were randomized to a combination (oral prednisolone 60 mg daily with gradual taper-off to 35 mg/day and cyclosporine 3 mg/kg/day) and corticosteroid (methylprednisolone 1000 mg for 3 days followed by oral prednisolone) groups, and were followed for 1 year.

Base Curve of Progressive Addition Lenses Influences Clear Vision and Stereopsis Area.

Purpose: Progressive addition lenses (PAL) are effective, particularly for middle-aged and elderly people who require reading spectacles. However, with PALs, peripheral vision may be distorted and blurred because of both the lateral bending of the surface and the effect of unequal bending of the light coming from an off-axis location in the tangential and sagittal directions, which may lead to a decrease in the quality of vision. Till date, no evaluation of PALs has been reported with regard to peripheral and binocular vision.

Correction to: Clinical and genetic characteristics of 14 patients from 13 Japanese families with -associated retinal disorder: report of eight novel variants.

[This corrects the article DOI: 10.1038/s41439-019-0065-7.].

Clinical and genetic characteristics of 14 patients from 13 Japanese families with -associated retinal disorder: report of eight novel variants.

Variants in the retinitis pigmentosa GTPase regulator (RPGR) gene are a major cause of X-linked inherited retinal disorder (IRD). We herein describe the clinical and genetic features of 14 patients from 13 Japanese families harboring variants in a nationwide cohort. Comprehensive ophthalmological examinations were performed to classify the patients into one of the phenotype subgroups: retinitis pigmentosa (RP) and cone rod dystrophy (CORD).

A Case Report of Intravitreal Bevacizumab for Iris Metastasis of Small Cell Lung Carcinoma with Neovascular Glaucoma.

A 79-year-old man who had been diagnosed with small cell lung carcinoma (SCLC) complained of right ocular pain and blurred vision. His right intraocular pressure (IOP) was 30 mm Hg, and anterior chamber cells and multiple grayish white iris masses associated with peripheral anterior synechia (PAS) and neovascularization of the right iris were observed. We presumed that the iris masses were iris metastasis of SCLC.

Vasodilatory effect of L-arginine on isolated rabbit and human posterior ciliary arteries in vitro and increased optic disc blood flow in vivo.

Purpose: This study aimed to clarify the vasodilatory effect of L-arginine on isolated rabbit and human posterior ciliary arteries (PCAs) and to investigate changes in optic disc blood flow after an infusion of L-arginine in vivo.

Methods: Vascular ring segments were mounted on a double myograph system. After obtaining maximal contraction following administration of high-K solution, L-arginine was administrated.

Evaluation of optic nerve head blood flow in normal rats and a rodent model of non-arteritic ischemic optic neuropathy using laser speckle flowgraphy.

Purpose: To evaluate optic nerve head (ONH) blood flow in normal rats and a rodent model of non-arteritic ischemic optic neuropathy (rNAION) in vivo using laser speckle flowgraphy (LSFG).

Methods: Rats were under general anesthesia; to induce NAION, Rose Bengal (RB) was injected into the tail vein. After the administration of RB, the left ONH was photoactivated using an argon green laser.

Bilateral Rhegmatogenous Retinal Detachment during External Beam Radiotherapy.

Herein, we report a case of nontraumatic bilateral rhegmatogenous retinal detachment (RRD) during external beam radiotherapy for nonocular tumor, presented as an observational case study in conjunction with a review of the relevant literature. A 65-year-old male was referred to our hospital due to bilateral RRD. He underwent a biopsy for a tumor of the left frontal lobe 4 months prior to presentation, and the tumor had been diagnosed as primary central nerve system B-cell type lymphoma.

Effects of L-arginine on anatomical and electrophysiological deterioration of the eye in a rodent model of nonarteritic ischemic optic neuropathy.

Purpose: The aims of this study were to clarify the effectiveness of L-arginine (1) for reducing the severity of anatomical changes in the eye and improving visual function in the acute stage of a rodent model of nonarteritic ischemic optic neuropathy (rNAION) and (2) in preventing those changes in anatomy and visual function.

Methods: For the first aim, L-arginine was intravenously injected into rats 3 h after rNAION induction; for the second aim, rNAION was induced after the oral administration of L-arginine for 7 days. The inner retinal thickness was determined over time by optical coherence tomography, and the amplitude of the scotopic threshold response (STR) and the number of surviving retinal ganglion cells (RGCs) were measured.

Laser speckle flowgraphy for differentiating between nonarteritic ischemic optic neuropathy and anterior optic neuritis.

Purpose: The aim of this study was to investigate the usefulness of laser speckle flowgraphy (LSFG) for the differentiation of acute nonarteritic ischemic optic neuropathy (NAION) from anterior optic neuritis (ON).

Methods: To investigate blood flow in the optic disc under normal conditions, NAION, and anterior ON, we compared the tissue blood flow of the right eye with that of the left eye in the control group, and that of the affected eye with that of the unaffected eye in the NAION and anterior ON groups.

Results: In the normal control group, the tissue blood flow did not significantly differ between the right and left eyes.

Effects of steroid administration and transcorneal electrical stimulation on the anatomic and electrophysiologic deterioration of nonarteritic ischemic optic neuropathy in a rodent model.

Purpose: To elucidate the effectiveness of steroid administration and transcorneal electrical stimulation (TES) on anatomic changes and visual function in a rodent model of nonarteritic ischemic optic neuropathy (rNAION).

Methods: Methylprednisolone (20 mg/kg) was injected through a central venous catheter twice a day for 3 days. TES was delivered with biphasic square pulses of 1 ms/phase, 100 μA of current, and 20 Hz of frequency for 60 min 3 h after induction on the 1st, 4th, 7th, 14th, and 28th days.

Evaluation of inner retinal thickness around the optic disc using optical coherence tomography of a rodent model of nonarteritic ischemic optic neuropathy.

Purpose: To clarify the usefulness of optical coherence tomography (OCT) for the objective and quantitative evaluation of retinal nerve fiber layer (RNFL) thickness around the optic disc in a rodent model of nonarteritic ischemic optic neuropathy (rNAION).

Methods: Inner retinal thickness was measured using OCT before and after rNAION induction. The thicknesses of the RNFL and the inner plexiform layer (IPL) were measured using a histologic preparation before and 56 days after induction.

Cytomegalovirus retinitis as an adverse immunological effect of pulses of vincristine and dexamethasone in maintenance therapy for childhood acute lymphoblastic leukemia.

We describe a 5-year-old female with acute lymphoblastic leukemia (ALL) who suffered from cytomegalovirus (CMV) retinitis during maintenance therapy consisting of 6-mercaptopurine (6-MP) and methotrexate (MTX) with pulses of vincristine (VCR) and dexamethasone (DEX). Administration of anticytomegaloviral drugs led to a complete regression of active retinitis. Her low CD4 positive T cells and serum immunoglobulin G (IgG) recovered when maintenance therapy was resumed without VCR and DEX.

Vasodilatory effects of antivascular endothelium growth factor (VEGF) antibody, corticosteroid, and nitric oxide on the posterior ciliary arteries.

Purpose: The purpose of this study was to determine whether an antivascular endothelium growth factor (VEGF) antibody, a corticosteroid, and sodium nitroprusside (SNP) [a nitric oxide (NO) donor] are possible treatment agents for nonarteritic ischemic optic neuropathy (NAION) by clarifying their effects on high-K (potassium) solution-induced contraction in isolated rabbit and human posterior ciliary arteries (PCA).

Methods: Vascular ring segments were cut from the distal section of the PCA and mounted in a double-myograph system. After obtaining the maximal contraction following the administration of high-K solution, bevacizumab as an anti-VEGF antibody, methylprednisolone as a corticosteroid, and SNP were administered separately.

Rodent model of nonarteritic ischemic optic neuropathy and its electrophysiological evaluation.

Purpose: Our aim was to establish a rodent model of nonarteritic ischemic optic neuropathy (rNAION).

Methods: To induce rNAION, after administration of Rose Bengal (RB) (2.5 mM), the small vessels of the left optic nerve were photoactivated using a 514-nm argon green laser with about 500-μm spot size for 12 s (RB-laser induction).

Application of Zen sitting principles to microscopic surgery seating.

This paper describes the application of an alternative seating concept for surgeons that reflects the research of Zen sitting postures, which require Zazen meditators to maintain fixed postures for long durations. The aim of this alternative approach is to provide sitters with a seat pan with sacral support(1) that provides a more even distribution of seat pressures, induces forward pelvic rotation and improves lumbar, buttock and thigh support. This approach was applied to the development of a chair for microscopic surgery.

Allelic copy number variation in FSCN2 detected using allele-specific genotyping and multiplex real-time PCRs.

Purpose: Allelic copy number variation (CNV) may alter the functional effects of a heterozygous mutation. The underlying mechanisms and their roles in hereditary diseases, however, are largely unknown. In the present study an FSCN2 mutation was examined that has been reported, not only in patients with retinitis pigmentosa (RP), but also in the normal population.

Macular hole formation in patients with retinitis pigmentosa and prognosis of pars plana vitrectomy.

Purpose: To investigate macular hole (MH) formation in patients with retinitis pigmentosa (RP) and to determine surgical prognosis for these patients.

Methods: Data for four RP patients with MHs were retrospectively reviewed. Comprehensive ocular examinations, including electroretinography, Goldmann perimetry, color funduscopy, B-scan ultrasonic tomography, and optical coherence tomography (OCT), were performed.

Somatic and gonadal mosaicism in X-linked retinitis pigmentosa.

The g.ORF15 + 652-653delAG mutation in the RPGR gene is the most frequent mutation in X-linked retinitis pigmentosa (XLRP). The objective of this study was to investigate the possibility of mosaicism in an XLRP family.

Identification of a novel RPGR exon ORF15 mutation in a family with X-linked retinitis pigmentosa.

Objective: To investigate the phenotypic and genotypic characteristics of a novel mutation associated with X-linked retinitis pigmentosa (XLRP).

Methods: Six individuals in a family with XLRP were recruited, and clinical examinations were performed. All of the members were genotyped with microsatellite markers at loci that were considered to be associated with XLRP.