Simultaneous Unilateral Abducens Nerve Palsy and Contralateral Anterior Ischaemic Optic Neuropathy as the Presenting Signs of Giant Cell Arteritis.

Ischaemic optic neuropathy is the most common, feared, and recognised ocular manifestation of giant cell arteritis (GCA), while extraocular muscle palsy rarely occurs in the disease. Overlooking the diagnosis of GCA in aged patients with acquired diplopia and strabismus is not only sight- but also life-threatening. Here, we present, for the first time, a case of unilateral abducens nerve palsy and contralateral anterior ischaemic optic neuropathy as the presenting signs of GCA in a 98-year-old woman.

Retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome.

Purpose: To describe a case of retinitis pigmentosa and nanophthalmos in a patient with attenuated Hunter's syndrome.

Methods: Fundus photography, total field electroretinogram, ultrasound, computerized visual field examination, biochemical examination and genetic testing were obtained.

Results: The fundus exam showed diffuse arteriolar attenuation, optic disc with regular contours, and pigment agglomerates like "bone spicules" in the middle periphery.

Quality of Life (QoL) in Non-Acute Vogt-Koyanagi-Harada Disease (VKHD) at Two Time Points 24 Months Apart.

Aims: To investigate the changes in quality-of-life (QoL) metrics at a 24-month interval in non-acute VKHD patients and their association with inflammation, treatment, and visual function.

Methods: SF-36 and VFQ-25 questionnaires were administered at two 24-month-apart moments to 22 non-acute VKHD patients followed for ≥12 months since acute disease onset. "Improvement," "unchanged," or "worsening" in questionnaires scores (difference >5-point) between M1 and M2 and their associations were sought.

Electroretinography reveals retinal dysfunction in Parkinson's disease despite normal high-resolution optical coherence tomography findings.

Introduction: Parkinson's disease (PD)-associated inner retinal abnormalities, particularly the retinal ganglion cells (RGC) layer, on optical coherence tomography (OCT) have recently gained importance as a biomarker of non-motor involvement of the disease but functional RGC evaluation using photopic negative response (PhNR) has not yet been determined. This study aims to compare structural and functional findings of the retina and optic nerve in PD with healthy controls (CT) including PhNR and OCT.

Methods: Forty-one eyes of 21 PD patients and 38 eyes of 19 CT underwent ophthalmic examination including visual contrast sensitivity test (CS), OCT, light-adapted full-field electroretinography (ffERG), and PhNR.

Fingerprint sign in Vogt-Koyanagi-Harada disease: a case series.

Background: The tomographic finding, which has been called the "fingerprint sign" in en face reconstructions, seems to be the result of a variety of processes that cause distension of the outer plexiform layer (OPL) and the Henle fiber layer (HFL). The aim of this paper is to describe the appearance of concentric rings at the OPL/HFL interface visualized using en face reconstructions of cross-sectional optical coherence tomography images of patients with Vogt-Koyanagi-Harada disease.

Methods: Retrospective analysis of images of six eyes of three patients obtained by cross-sectional OCT imaging and en face reconstruction at the level of the OPL/HFL interface.

Comparison of Visual Evoked Potentials in Patients Affected by Optic Neuritis From Multiple Sclerosis or Neuromyelitis Optica Spectrum Disorder.

Purpose: To compare the visual evoked potentials (VEPs) of optic neuritis (ON) patients with multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and controls. To evaluate correlations between VEP and optical coherence tomography (OCT), contrast sensitivity (CS), and automated perimetry.

Methods: Fifty-five eyes with ON from 29 patients (MS = 14 and NMOSD = 15) and 57 eyes from 29 controls were evaluated using VEP, automated perimetry, CS, and optical coherence tomography.

Associations between functional and structural measurements in non-acute Vogt-Koyanagi-Harada disease.

Purpose: To evaluate associations between functional and structural measurements in patients with non-acute VKHD.

Methods: In this cross-sectional study, 16 non-acute VKHD patients (32 eyes; 14 female) were evaluated with multifocal electroretinogram (mfERG), standardized automated perimetry (SAP) and optical coherence tomography (OCT)examinations. All included patients had a minimum 12 months of follow-up from acute onset and were participants of an ongoing prospective study since acute phase with systematic clinical imaging evaluations and electroretinogram examinations within a predefined treatment.

Impact of Inflammation and Treatment on Self-reported Quality of Life in Patients with Non-acute Vogt-Koyanagi-Harada Disease (VKHD).

: To evaluate associations between vision-related (VR-) and health-related (HR-) QoL metrics and inflammation and treatment in non-acute VKHD patients.: Cross-sectional study in a tertiary center in Sao Paulo, Brazil with 22 patients with non-acute VKHD followed prospectively for ≥12 months since acute disease onset, with systematic evaluation and predefined treatment protocols. VR- and HR-QoL aspects were assessed by VFQ-25 and SF-36 questionnaires, respectively.

Outer Retinal Dysfunction on Multifocal Electroretinography May Help Differentiating Multiple Sclerosis From Neuromyelitis Optica Spectrum Disorder.

To evaluate the intermediate and outer retina of patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) using OCT and multifocal electroretinography (mf-ERG). Patients with MS ( = 30), NMOSD ( = 30), and healthy controls ( = 29) underwent visual field (VF), OCT, and mf-ERG testing. The eyes were distributed into 5 groups: MS with or without history of ON (MS+ON, MS-ON), NMOSD with or without ON (NMOSD+ON, NMOSD-ON), and controls.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts.

A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts.

Full-field electroretinogram behavior in Vogt-Koyanagi-Harada disease: a 24-month longitudinal study in patients from acute onset evaluated with multimodal analysis.

Purpose: To prospectively evaluate the dynamic changes of the full-field electroretinogram (ff-ERG) and its association with inflammatory signs in patients with Vogt-Koyanagi-Harada disease (VKHD) followed up after acute onset.

Methods: Twelve acute VKHD patients, who were followed up for at least 24 months, were enrolled at a tertiary center from June 2011 to January 2017. Treatment consisted of intravenous methylprednisolone followed by 1 mg/kg/day of oral prednisone with a slow tapering associated with late non-steroidal immunosuppressive therapy in previously defined cases.

Prospective evaluation of intravitreal bevacizumab for ischemic central retinal vein occlusion.

Background: Although previous studies have evaluated the effect of anti-VEGF therapies for central retinal vein occlusion (CRVO) patients, the majority of previous studies have excluded or included a very small number of patients with ischemic CRVO (iCRVO). The aim of our study is to examine the effects of bevacizumab on macular edema secondary to ischemic central retinal vein occlusion, as well as the effects on central choroidal thickness and best-corrected visual acuity.

Methods: In this prospective, interventional case series, iCRVO was defined by the presence of ≥ 10 or more disc diameter areas of retinal nonperfusion by fluorescein angiography (FA) and by the presence of a b/a ratio less than 1.

Self-Reported Quality of Life in Patients with Long-Standing Vogt-Koyanagi-Harada Disease.

: To assess health-related (HR-) and vision-related (VR-) quality of life (QoL) in patients with long-standing Vogt-Koyanagi-Harada disease (VKHD).: Cross-sectional study of 49 patients with disease duration ≥12 months followed at Uveitis Service, Universidade de São Paulo, BR, for at least 12 months. HR- and VR-QoL were evaluated using SF-36 and NEI VFQ-25 questionnaires, respectively.

Macular and Multifocal PERG and FD-OCT in Preperimetric and Hemifield Loss Glaucoma.

Purpose: To evaluate the ability of macular and multifocal (mf) pattern electroretinogram (PERG) to differentiate preperimetric glaucoma (PG) and glaucoma with hemifield loss (GHL) from controls, to compare the discrimination ability of PERG and fourier-domain optical coherence tomography (FD-OCT), and to assess the relationship between measurements.

Patients And Methods: Standard automated perimetry, steady-state and transient PERG and mfPERG measurements were obtained from PG (n=14, 24 eyes), GHL (n=5, 7 eyes), and controls (n=19, 22 eyes). Circumpapillary retinal nerve fiber layer (cpRNFL), full-thickness macula, and segmented macular layer thicknesses on FD-OCT were investigated.

Morphological and Functional Inner and Outer Retinal Layer Abnormalities in Eyes with Permanent Temporal Hemianopia from Chiasmal Compression.

Purpose: The aims of this study are to compare optical coherence tomography (OCT)-measured macular retinal layers in eyes with permanent temporal hemianopia from chiasmal compression and control eyes; to compare regular and slow-flash multifocal electroretinography (mfERG) in patients and controls; and to assess the correlation between OCT, mfERG, and central visual field (SAP) data.

Methods: Forty-three eyes of 30 patients with permanent temporal hemianopia due to pituitary tumors who were previously submitted to chiasm decompression and 37 healthy eyes of 19 controls were submitted to macular spectral domain OCT, mfERG, and 10-2 SAP testing. After segmentation, the thickness of the macular retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), inner nuclear layer (INL), outer plexiform layer (OPL), outer nuclear layer, and photoreceptor layer (PRL) was measured.

Multifocal Visual Evoked Potential in Eyes With Temporal Hemianopia From Chiasmal Compression: Correlation With Standard Automated Perimetry and OCT Findings.

Purpose: To verify whether multifocal visual evoked potential (mfVEP) can differentiate eyes with temporal hemianopia due to chiasmal compression from healthy controls. To assess the relationship between mfVEP, standard automated perimetry (SAP), and Fourier domain-optical coherence tomography (FD-OCT) macular and peripapillary retinal nerve fiber layer (RNFL) thickness measurements.

Methods: Twenty-seven eyes with permanent temporal visual field (VF) defects from chiasmal compression on SAP and 43 eyes of healthy controls were submitted to mfVEP and FD-OCT scanning.

Multimodal image analysis of the retina in Hunter syndrome (mucopolysaccharidosis type II): Case report.

Introduction: We report a case of retinal and posterior ocular findings in a 33-year-old man diagnosed with Hunter syndrome (Mucopolysaccharidosis type II) in a multimodal imaging way.

Case Presentation: Our patient was complaining of blurred night vision for the past 3 years. He had not received any systemic treatment for Hunter syndrome.

Ophthalmologic manifestations of celiac disease.

Celiac disease is an autoimmune disorder that affects the small intestine of genetically predisposed individuals. Ophthalmic manifestations are within the extra-intestinal manifestations, and can be divided into those of autoimmune disorders or those due to absorptive disabilities. This article reviewed the ophthalmologic manifestation of celiac disease.

Relationship Between Pattern Electroretinogram, Frequency-Domain OCT, and Automated Perimetry in Chronic Papilledema From Pseudotumor Cerebri Syndrome.

Purpose: We evaluated the ability of transient pattern electroretinogram (PERG) parameters to differentiate between eyes with visual field (VF) loss and resolved papilledema from pseudotumor cerebri syndrome (PTC) and controls, to compare PERG and optical coherence tomography (OCT) with regard to discrimination ability, and to assess the correlation between PERG, frequency domain OCT (FD-OCT), and VF measurements.

Methods: The VFs and full-field stimulation PERGs based on 48 and 14-min checks were obtained from patients with PTC (n = 24, 38 eyes) and controls (n = 26, 34 eyes). In addition, FD-OCT peripapillary retinal nerve fiber layer (RNFL) and segmented macular layer measurements were obtained and correlation coefficients were determined.

The role of pattern-reversal electroretinography in the diagnosis of glaucoma.

Pattern electroretinography is used to assess the function of the inner retinal layers, particularly the retinal ganglion cell layer, using a reversing checkerboard or grating pattern that maintains a constant overall mean luminance over time. A normal transient response comprises a positive component of the wave (P50) followed by a longer negative component of the wave (N95). Glaucomatous optic neuropathy causes progressive loss of retinal ganglion cells, potentially detectable as abnormalities on examination, particularly in the N95 component.

Mutations in PCYT1A cause spondylometaphyseal dysplasia with cone-rod dystrophy.

Spondylometaphyseal dysplasia with cone-rod dystrophy is a rare autosomal-recessive disorder characterized by severe short stature, progressive lower-limb bowing, flattened vertebral bodies, metaphyseal involvement, and visual impairment caused by cone-rod dystrophy. Whole-exome sequencing of four individuals affected by this disorder from two Brazilian families identified two previously unreported homozygous mutations in PCYT1A. This gene encodes the alpha isoform of the phosphate cytidylyltransferase 1 choline enzyme, which is responsible for converting phosphocholine into cytidine diphosphate-choline, a key intermediate step in the phosphatidylcholine biosynthesis pathway.

Pattern electroretinogram in neuromyelitis optica and multiple sclerosis with or without optic neuritis and its correlation with FD-OCT and perimetry.

Purpose: To evaluate the ability of transient pattern electroretinogram (PERG) parameters to differentiate between eyes of patients with neuromyelitis optica (NMO), longitudinally extensive transverse myelitis (LETM), multiple sclerosis with optic neuritis (MS + ON), multiple sclerosis without optic neuritis (MS - ON), and controls, to compare PERG and OCT with regard to discrimination ability, and to assess the correlation between PERG, FD-OCT, and visual field measurements (VFs).

Methods: Visual field measurements and full-field stimulation PERGs based on both 48- and 14-min checks were obtained from patients with MS (n = 28), NMO (n = 20), LETM (n = 18), and controls (n = 26). In addition, FD-OCT peripapillary retinal nerve fiber layer (RNFL) and segmented macular layer measurements were obtained and their correlation coefficients were determined.

Correlation between multifocal pattern electroretinography and Fourier-domain OCT in eyes with temporal hemianopia from chiasmal compression.

Purpose: To evaluate the correlation between multifocal pattern electroretinography (mfPERG) and Fourier-domain optical coherence tomography (FD-OCT) with regard to macular and retinal nerve fiber layer (RNFL) thickness in eyes with temporal hemianopia from chiasmal compression.

Methods: Twenty-five eyes from 25 patients with permanent temporal visual field defects from chiasmal compression and 25 healthy eyes were submitted to mfPERG using a stimulus pattern of 19 rectangles, standard automated perimetry and FD-OCT measurements. The mfPERG response was determined for groups of three rectangles for the nasal and temporal hemifields and for each quadrant.