Optic Nerve Swelling Research Articles

Transient binocular vision loss and pain insensitivity in Klippel–Feil syndrome: a case report

BackgroundKlippel–Feil syndrome is a rare congenital bone disorder characterized by an abnormal fusion of two or more cervical spine vertebrae. Individuals with Klippel–Feil syndrome exhibit diverse clinical manifestations, including skeletal irregularities, visual and hearing impairments, orofacial anomalies, and anomalies in various internal organs, such as the heart, kidneys, genitourinary system, and nervous system.Case presentationThis case report describes a 12-year-old Pashtun female patient who presented with acute bilateral visual loss. The patient had Klippel–Feil syndrome, with the typical clinical triad symptoms of Klippel–Feil syndrome, along with Sprengel’s deformity. She also exhibited generalized hypoalgesia, which had previously resulted in widespread burn-related injuries. Upon examination, bilateral optic disc swelling was observed, but intracranial pressure was found to be normal. Extensive investigations yielded normal results, except for hypocalcemia and low vitamin D levels, while parathyroid function remained within the normal range. Visual acuity improved following 2 months of calcium and vitamin D supplementation, suggesting that the visual loss and optic nerve swelling were attributed to hypocalcemia. Given the normal parathyroid function, it is possible that hypocalcemia resulted from low vitamin D levels, which can occur after severe burn scarring. Furthermore, the patient received a provisional diagnosis of congenital insensitivity to pain on the basis of the detailed medical history and the findings of severe and widespread loss of the ability to perceive painful stimuli, as well as impaired temperature sensation. However, due to limitations in genetic testing, confirmation of the congenital insensitivity to pain diagnosis could not be obtained.ConclusionThis case highlights a rare presentation of transient binocular vision loss and pain insensitivity in a patient with Klippel–Feil syndrome, emphasizing the importance of considering unusual associations in symptom interpretation.

Orbital Tumor Misdiagnosed as Optic Neuritis in Rheumatoid Arthritis Patient: A Case Report

Abstract
 Introduction : Unilateral blurred vision and optic nerve swelling are the two most common signs of optic neuritis (ON). However, ON is rarely reported in patients with rheumatoid arthritis (RA), thus necessitating further diagnostic workup.
 Case Illustration : A 42-year-old woman visited our hospital with strabismus and progressive loss of vision in her left eye since three months ago. She had been treated with pulse dose steroid for suspected ON. She had been diagnosed with RA over five years and treated with immunomodulatory medication. Left eye examination revealed best corrected visual acuity of 20/50, proptosis, esotropia with painless limitation in temporal movement. Afferent pupillary defect (RAPD) and optic nerve swelling were present. OCT assessment revealed reduction in RNFL thickness. Further magnetic resonance imaging (MRI) investigation revealed a 2.35 x 2.6 x 1.8 cm mass in the left intraconal retrobulbar, in proximity to the medial optic nerve, which suggests a case of orbital Schwanoma. Excisional biopsy was performed and revealed a benign tumor.
 Discussion : Intraconal tumor may falsely be diagnosed as an optic neuritis in autoimmune patient. Neuro- ophthalmologic symptoms of systemic inflammatory in the optic nerve includes unilateral acute visual loss and pain in eye movement. Signs of ON includes RAPD, disc edema, and corresponding visual field defect. MRI of the optic nerve can assist diagnostic workup.
 Conclusion : The exact diagnosis of rheumatoid arthritis-associated ocular manifestations is challenging. Imaging using MRI might be needed to provide clarity in the diagnosis.

Influence of sex and disease etiology on the development of papilledema and optic nerve sheath extension in the setting of intracranial pressure elevation in children

IntroductionDilatation of the optic nerve sheath diameter and swelling of the optic disc are known phenomena associated with intracranial pressure elevation. Research questionDo sex and disease etiology have an impact on the development of optic disc elevation and optic nerve sheath extension in children in the setting of ICP elevation? Fundoscopic papilledema and point-of-care-ultrasound techniques-optic nerve sheath diameter (US-ONSD) and optic disc elevation (US-ODE) - were compared in this regard. Material and methods72 children were included in this prospective study; 50 with proven pathology (e.g. pseudotumor cerebri, tumor), 22 with pathology excluded. Bilateral US-ONSD and US-ODE were quantified by US using a 12-MHz-linear-array-transducer. This was compared with fundoscopic optic disc findings and in 28 patients with invasive ICP values, stratified for sex and etiology. ResultsIn patients with proven disease, significant more girls (69%) had fundoscopic papilledema compared with boys (37%, p < 0.05). Girls had also larger US-ODE values (0.86 ± 0.36 mm vs. 0.65 ± 0.40 mm in boys). 80% of tumor patients had initial papilledema (100% girls, 79% boys), compared with 50% in pseudotumor cerebri (PTC) (83% girls, 30% boys). US-ONSD had no sex- and no etiology-specificity. Discussion and conclusionPresence of papilledema appears to be influenced by sex and etiology, whereas US-ONSD is not. Girls seem more likely to develop papilledema under similar conditions. Male sex and PTC appear as risk factors for being undetected by fundoscopic findings. US-ONSD and US-ODE seem useful tools to identify pathologies with potentially increased ICP requiring treatment in children regardless of sex and etiology.

Neuroprotective effect of 1,25‑dihydroxyvitamin D3 against hyperoxia‑induced brain injury in premature rats.

Studies have shown that vitamin D plays a crucial role in brain development, brain metabolism and neuroprotection. There is little evidence for the neuroprotective effect of 1, 25‑dihydroxyvitamin D3 (1,25‑(OH)2D3) on various brain injury models. The aim of this study was to investigate the neuroprotection effect of 1,25‑(OH)2D3 against hyperoxia‑induced brain injury in premature rats. Sprague‑Dawley rats were exposed to 95% oxygen or room air for 24 h and treated with 1,25‑(OH)2D3 or normal saline for 14 consecutive days. The histopathological changes of optic chiasma tissue were observed by hematoxylin‑eosin staining. Immunohistochemistry, qRT‑PCR, and western blot were performed to detect the expression of integrin‑β1 and yes‑associated protein (YAP) in the organization of the optic chiasm. Histopathological sections of optic chiasma showed visible optic nerve swelling, expanded nerve fiber space, uneven staining, obvious oligodendrocyte proliferation and disordered cell arrangement accompanied by inflammatory cell infiltration and exudation after 7 days and 14 days of hyperoxia exposure. The hyperoxia group treated with 1,25‑(OH)2D3 were showed improvement of brain injury with reduced inflammatory exudation, uniform nerve fiber staining and less obvious oligodendrocyte proliferation. Immunohistochemical staining, qRT‑PCR and western blot indicated that 1,25‑(OH)2D3 treatment upregulated the expression of integrin‑β1 and YAP in the hyperoxia group on day 7. However, the expression of YAP was significantly increased compared with control group and treatment with 1,25‑(OH)2D3 reduced the expression of YAP in the hyperoxic group on day 14. 1,25‑(OH)2D3 may regulate the expression of integrin‑β1 and YAP to alleviate hyperoxia‑induced brain injury in premature rats.

Modifications in ocular microperfusion after transcatheter aortic valve implantation

Cerebral embolization is a known complication of transcatheter aortic valve implantation (TAVI) but the effect of the procedure on the ocular perfusion is currently unclear. Thus, we investigated post-procedural morphologic and perfusion changes of the retina and choroid, using optical coherence tomography angiography (OCTA) and color fundus photography (CFP) in a prospective cohort study. Ophthalmic examinations were conducted pre- and post-TAVI. OCTA images were analyzed quantitatively based on vessel density and skeleton density of the superficial and deep retinal plexus as well as the signal intensity and flow deficits in the choriocapillaris. CFP images were assessed for presence of acute retinal ischemia, optic nerve swelling, vessel emboli, hemorrhages and cotton wool spots. Data was analyzed using linear mixed models. Twenty patients (9 women; 11 men) at a mean age of 81 ± 6 years were included. Pre- and post-interventional ocular imaging data were available for 32 eyes. The analysis revealed a significant impairment of the choriocapillaris perfusion after TAVI with an increased proportion of flow deficits (p = 0.044). When controlling for blood pressure, the average size of choriocapillaris flow voids was significantly increased (systolic and diastolic, p = 0.039 and 0.029). Qualitatively, focal areas of retinal ischemia were detected on OCTA in 33% of participants. Silent emboli or cotton wool spots were identified on CFP in 21%. Our findings indicate a reduced choroidal perfusion as well as areas of retinal ischemia and embolization in a considerable proportion of patients following TAVI. Pending confirmation in a larger sample, these complications merit monitoring as well as inclusion in consent procedures for TAVI.

Bilateral optic nerve infiltration in high-grade systemic lymphoma with secondary combined central retinal artery and vein occlusions.

In Brief Bilateral lymphomatous optic nerve infiltration initially presented with mild unilateral optic nerve swelling without signs of infiltration, which then rapidly progressed into bilateral severe optic nerve swelling and infiltration with combined central retinal artery and vein occlusion resulting in complete blindness despite urgent high-dose corticosteroids infusions, radiotherapy and chemotherapy.

Clinical and pathological characterization of ophthalmic disease in a canine model of mucopolysaccharidosis type I.

Mucopolysaccharidosis type I (MPS I) is a rare lysosomal storage disease caused by α-L-iduronidase enzyme deficiency, resulting in glycosaminoglycan (GAG) accumulation in various cell types, including ocular tissues. Ocular manifestations in humans are common with significant pathological changes including corneal opacification, retinopathy, optic nerve swelling and atrophy, and glaucoma. Available treatments for MPS I are suboptimal and there is limited to no effect in treating the ocular disease. The goal of this study was to characterize the clinical and pathological features of ocular disease in a line of MPS I affected dogs, including changes not previously reported. A total of 22 dogs were studied; 12 MPS I were affected and 10 were unaffected. A subset of each underwent complete ophthalmic examination including slit lamp biomicroscopy, indirect ophthalmoscopy, rebound tonometry, and ultrasonic pachymetry. Globes were evaluated microscopically for morphological changes and GAG accumulation. Clinical corneal abnormalities in affected dogs included edema, neovascularization, fibrosis, and marked stromal thickening. Intraocular pressures were within reference interval for affected and unaffected dogs. Microscopically, vacuolated cells containing alcian blue positive inclusions were detected within the corneal stroma, iris, ciliary body, sclera, and optic nerve meninges of affected dogs. Ganglioside accumulation was identified by luxol fast blue staining in rare retinal ganglion cells. Increased lysosomal integral membrane protein-2 expression was demonstrated within the retina of affected animals when compared to unaffected controls. Results of this study further characterize ocular pathology in the canine model of MPS I and provide foundational data for future therapeutic efficacy studies.

ROSAH syndrome mimicking chronic uveitis.

To suggest a unique missense variant candidate based on long-term ophthalmological changes and associated systemic signs described in five patients from two unrelated families affected by an autosomal dominant multi-systemic disorder including Retinal dystrophy, Optic nerve oedema, Splenomegaly, Anhidrosis and migraine Headaches, called ROSAH syndrome, related to a unique missense variant in ALPK1 gene. Observational longitudinal follow-up study of unrelated families. Clinical analysis of ophthalmological and systemic examinations was performed, followed by genetic analysis, including targeted Next Generation Sequencing (NGS) and Whole-Genome Sequencing (WGS). The ophthalmological phenotype showed extensive optic nerve swelling associated with early macular oedema and vascular leakage. The main associated systemic manifestations were recurrent fever, splenomegaly, anhidrosis, mild cytopenia, anicocytosis and hypersegmented polynuclear cells. WGS, shortened in the second family by the gene candidate suggestion, revealed in all patients the heterozygous missense variant c.710C>T; p.(Thr237Met) in ALPK1. The primary morbidity in ROSAH syndrome in this cohort appeared ophthalmological. Comprehensive, detailed phenotype changes aided by the advancement in genetic testing could allow an early genetic diagnosis of ROSAH syndrome and targeted treatment. The unique missense variant may be suggested as a target of gene correction therapy.

Evaluation of visual evoked potentials in dysthyroid optic neuropathy

ABSTRACT Purpose To evaluate the findings of visual evoked potentials (VEP) in patients with dysthyroid optic neuropathy (DON). Methods In this observational, cross-sectional study 40 eyes (22 patients) with a diagnosis of DON were included. Results We discovered that in 16 out of 37 eyes with pattern-VEP (p-VEP), the latency of P100 wave was normal in spite of having a diagnosis of DON. The same pattern was also observed in the measurement of the amplitude of P100 wave: in 28 out of 37 eyes with p-VEP the amplitudes were observed as normal. In 3 eyes of 3 patients p-VEP showed no response, therefore a flash-VEP (f-VEP) was performed. Flash-VEPs of those patients indicated a prolonged P100 latency with a reduced amplitude. The sensitivity of abnormal P100 latency was 56.8% (95%CI 39.5–72.9%); and that of reduced P100 amplitude was 24.3% (95%CI 11.8–41.2%). Also, in 40 eyes color vision test by Arden was performed. In 36 eyes (20 patients) the tritan value was pathological (based on a threshold of ≥8%). Conclusion According our data, VEP seems to have a limited potential especially in patients with a good best-corrected visual acuity (BCVA ≤0.2 LogMAR) for identifying the optic nerve involvement. The fact that P100 latency and amplitude were normal even in cases with an optic nerve swelling makes us question the usefulness of the VEP for diagnosing cases of DON in daily clinical life.

Severe Chorioretinal Folds after Optical Neuritis

A 40-year-old man had an isolated episode of optic neuritis of the right eye. Fundoscopy revealed exuberant optic disc swelling with chorioretinal folds (Fig A). Magnetic resonance imaging revealed discrete optic nerve swelling and contrast enhancement. No etiologic cause of his optic neuritis was identified. He was treated with systemic corticosteroids and had complete recovery of visual acuity, color vision, and visual field. However, he experiences severe metamorphopsias due to persistent chorioretinal folds, visible in fundus fluorescein angiography and OCT (Fig B).

Intraoperative Mounted Optical Coherence Tomography Findings Following Reversal of Optic Nerve Head Cupping in Childhood Glaucoma

To examine the structural changes occurring in the optic nerve head (ONH) and macula in infants with childhood glaucoma and clinically observed ONH cupping reversal following intraocular pressure (IOP)-reducing glaucoma surgery, as captured by intra-operative spectral-domain optical coherence tomography (SD-OCT). Retrospective observational case series from an ongoing prospective cohort study. Included were 18 eyes of 14 patients with childhood glaucoma. All eyes had SD-OCT imaging pre- and post-glaucoma intervention and clinically identified ONH cupping reversal. Patients with poor quality images or persistent optic nerve swelling following IOP reduction were excluded. Outcome measurements included IOP, cup-to-disc ratio, axial length and SD-OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), transverse horizontal diameter of Bruch membrane opening (BMO-D), cup depth, and macula. Mean age at surgery was 1.14±0.93 years and mean interval between pre- and post-operative imaging was 127 days (range 35-595). Following intervention, mean IOP reduction was 45%, accompanied by significant reductions in the cup-to-disc ratio (0.30±0.12, p<0.001), axial length (0.43±0.28mm, p<0.001) and cup depth (46%, p<0.001). Mean global pRNFL thickness pre- vs. post-treatment was 93.1±14.7µm vs. 93.1±17.1µm, respectively, p=1.0. There was no significant difference in pre- and post-treatment global or sectoral pRNFL, 3mm macular total and segmented retinal layer volumes, or the BMO-D. Clinical ONH cupping reversal after IOP-lowering surgery was associated with axial length reduction and decrease in cup depth, but no significant change in the pRNFL or macular volume measures. ONH cupping reversal likely marks stabilization but any pre-intervention ONH damage persists.

Anti-NOGO Antibody Neuroprotection in a Rat Model of NAION.

PurposeTo evaluate the efficacy and mechanisms of anti-NOGO receptor monoclonal antibody 11C7mAb in a rat model of nonarteritic anterior ischemic optic neuropathy (rNAION).MethodsThe rNAION was induced in one eye of 20 Long-Evans rats, which were studied in 10 groups of two rats, each group containing a sham rat receiving intravitreal injections of vehicle and a treatment rat receiving intravitreal injections of 11C7mAb. Fellow eyes served as naïve controls. The rats were tested using flash electroretinograms (ERGs), flash visual evoked potentials (VEPs), and optical coherence tomography (OCT). Thirty days after induction, they were euthanized, and the eyes were prepared for immunohistochemistry (two groups), hematoxylin and eosin staining (two groups) or transmission electron microscopy (TEM; six groups).ResultsNinety-five percent of the VEP amplitude was preserved in eyes treated with 11C7mAb, versus 69% in the control eyes. Immunohistochemistry revealed a large reduction in microglia and extrinsic macrophages with axon sparing. In addition to axon preservation, TEM also showed reduced extracellular debris and only slight myelin damage compared with the vehicle-treated animals. There were no significant differences in retinal ganglion cell counts, nor was there a difference in optic nerve swelling as measured by OCT. ERGs were used to exclude eyes with retinal vascular occlusions, an occasional complication of the induction technique.ConclusionsThe 11C7mAb preserves optic nerve integrity and reduces inflammation in rNAION.Translational RelevanceThis study evaluates the efficacy of an anti-NOGO receptor antibody in a rat model of NAION, a disorder that currently has no universally-acknowledged treatment.

An ultrasound vibro-elastography technique for assessing papilledema

Papilledema is optic nerve swelling caused by increased intracranial hypertension, which has the potential to cause significant vision loss. Papilledema from idiopathic intracranial hypertension (IIH) is typically bilateral and symmetric, but can sometimes be asymmetric and even unilateral. The cause for this asymmetry is unknown. The purpose of this study was to develop ultrasound vibro-elastography (UVE) for assessing patients with papilledema to evaluate for biomechanical differences in these eyes. Nine patients with papilledema from IIH and 9 age-matched healthy control subjects were enrolled. A local harmonic vibration was used to generate wave propagation through the eyelid. Three excitation frequencies of 100, 150, and 200 Hz were used. A 6.4 MHz ultrasound probe was used to measure wave propagation in the eye structures. Wave speeds were measured in the posterior sclera of the maculae. The magnitudes of the wave speed of the IIH patients’ posterior sclera were significantly higher than those of healthy subjects. In patients with unilateral papilledema, the wave speed of the posterior sclera were higher in the eyes with papilledema than in the contralateral eyes without papilledema. UVE provides a noninvasive technique to measure the wave speed of posterior sclera, which is stiffer in eyes with papilledema.

BRAIN IN SPACE. HOW DOES SPACE AFFECT THE HUMAN BRAIN?

Introduction Nowadays more and more people and international companies are interested in Human Spaceflights. Aim and method In this review, the negative effects from space radiation, microgravity and the factor of isolation on the central nervous system will be described in relation to space neuroscience and the relevant studies examined. Results Space radiation can damage neuronal connections with both acute and chronic effects, manifested as altered cognitive function, reduced motor function, and behavioral changes. Moreover, some astronauts report a condition known as Spaceflight Associated Neuro-ocular Syndrome (SANS). The brain scans performed upon those astronauts, who came back from space travel suggest that due to reduced gravity conditions the brain and the fluids in the human body shift upwards, which increases pressure in the skull and may result in opticnerve swelling that causes blurred vision. Another interesting part of space neuroscience is the research of structural neuroplasticity. A study conducted on cosmonauts revealed an increase in the neuronal tissue of sensorimotor structures responsible for movement coordination. In addition to the space radiation and microgravity, long-term confinement also affects the microstructure of the brain white matter, which was proven in the study that used DTI (Diffusion Tensor Imaging). Conclusions To conclude, to continue understanding the risks posed by spaceflight to astronauts’ health research in the field of space neuroscience is important. In addition, the acquired insight could be relevant for terrestrial vestibular patients, patients with neurodegenerative disorders, as well as the elderly population, coping with neurological deficits. Keywords: space medicine, brain, space neuroscience, SANS, space radiation.

Endovascular Dural Venous Sinus Stenting for the Treatment of Papilledema in Idiopathic Intracranial Hypertension

Abstract Introduction: Recently a subset of patients with Benign Intracranial hypertension has been identified that have cerebral dural venous stenosis. Recent studies have demonstrated that venous sinus stenting can result in normalization of these pressures and resultant resolution of ophthalmologic findings. Methods: Twenty-six patients were referred to a single institution for optic nerve swelling due to Benign Intracranial hypertension and found to have cerebral dural venous sinus stenosis. These patients subsequently underwent cerebral venous sinus stenting. Ophthalmic manifestations were evaluated pre and post procedure as well as intravascular pressure measurements and pressure gradients before and after the stenting procedure. The pre and postoperative ophthalmologic findings were tabulated as well as the pre and post procedure pressure measurements before and after stenting. Results: The average preprocedural pressure gradient measured 13.8 mmHg and was reduced to 1.3 mmHg after the stenting procedure. 25 of 26 patients had improvement in their papilledema after the stenting procedure. No complications occurred during or after the procedure. Conclusion: Endovascular Dural Venous sinus stenting is a safe and effective treatment modality for the ophthalmic manifestations of Benign Intracranial Hypertension in those patients found to have cerebral venous sinus stenosis.

Drusen de papila. Análisis descriptivo

INTRODUCCIÓN: Los drusen de papila son depósitos de material hialino calcificado a nivel de la cabeza delnervio óptico. Pueden ser difíciles de distinguir de la verdadera inflamación del nervio óptico y su identificacióncorrecta es relevante debido a la morbilidad visual y sistémica que conlleva el diagnóstico de un verdaderoedema de papila.OBJETIVO: Describir los hallazgos clínicos en un grupo de pacientes con drusen de papila.MATERIAL Y MÉTODO: Estudio descriptivo retrospectivo de 39 casos de pseudoedema de papila secundarioa drusen de papila. Se recuperaron datos de la historia clínica y se analizaron características oftalmológicas yde los métodos complementarios (campo visual computarizado –CVC-, tomografía de coherencia óptica –OCT-, ecografía y neuroimágenes).RESULTADOS: Se estudiaron 39 ojos. La edad promedio al diagnóstico fue de 9 años. La mayoría no reportoantecedentes patológicos ni heredofamiliares. El principal motivo de consulta fue control de rutina y el segundofue cefalea. El 56% tenían una excelente agudeza visual, 20% buena, 2.5% regular y 5% mala agudeza visual.El 36% de los ojos fueron emétropes, 41% hipermétropes y 23% miopes. Los hallazgos más representativos enel fondo de ojos fue la sobreelevación papilar (71,8% IC95% 57,9-86), papila de bordes borrados (43% IC95%27,4-58,5) y tortuosidad vascular (43,6% IC95% 27,4-58,5). En todos los casos la ecografía fue diagnóstica,mostrando imágenes hiperecoicas dentro del nervio óptico. En el CVC no se encontraron hallazgos relevantes.CONCLUSIONES: Los drusen de papila son una alteración que puede encontrarse con frecuencia en un examende fondo de ojo de rutina, y que un oftalmólogo debe conocer para evitar errores diagnósticos.

Clinical characteristics, treatment and prognosis of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis in children

Objective: To investigate the clinical characteristics, treatment and prognosis of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) in pediatric patients. Methods: Clinical data, laboratory examination, the initial best corrected visual acuity (BCVA), fundus, neuroelectrophysiological results, MRI imaging, treatment and prognosis of children diagnosed with MOG-ON from 2016 to 2019 were retrospectively analyzed. Results: A total of 29 eyes from 16 children were involved, with a male/female ratio of 1∶1, onset age of (7.0±2.9) years. Seven of 16 patients had prodromal infection, with a unilateral/bilateral ratio of 3∶13, and 2 cases had recurrent optic neuritis. Before treatment, BCVA of 19 eyes (65.5%) was ≤0.1, among them, 4 had no sense of light, 5 had light sense, 5 with sense of hands in front of eyes, and 5 with sense of fingers in front of eyes. There were 10 eyes (34.5%) with BCVA of 0.1-0.5. After treatment, there were 4 eyes (13.8%), 5 eyes (17.2%) and 20 eyes (69.0%) in groups with BCVA of 0.1-0.5, 0.5-1.0, and>1.0, respectively. Twelve of 16 patients had optic papillitis in fundus examination during acute phase. The latency was prolonged and the amplitude was decreased in P100 wave of all the children. Thirteen out of 16 children showed swelling and thickening of optic nerve in MRI T2WI. MRI images exhibited intracranial demyelinating lesions in 12 of 16 children and long segment spinal cord lesions in 3 of 16 children. Thirteen of 16 patients showed effective results after intravenous methylprednisolone (IVMP) and intravenous immunoglobulin (IVIG) treatment. There was no relapse after administration of mycophenolate mofetil in 2 recurrent children. No progression after administration of rituximab was found in 1 child with corticosteroid insensitivity. The average follow-up time was (16±9) months and no recurrence occurred. Ten of 16 patients had full recovery, 4 had significant improvement, and 2 showed no significant improvement. Conclusions: There is no significant gender difference in the incidence of pediatric MOG-ON. Bilateral involvement and severe visual impairment are common in acute phase. Most patients have good response to IVMP combined with IVIG treatment and hence have a good prognosis. Only a few of them have neurological sequelae.

Feasibility of a Nonmydriatic Ocular Fundus Camera in an Outpatient Neurology Clinic.

To determine the feasibility of nonmydriatic fundus photography in the neurology outpatient setting and to record frequency of clinically relevant fundus findings. Over 5 weeks, fundus photographs were obtained using a nonmydriatic fundus camera in both eyes of adult patients attending our general neurology and headache clinics. A neurologist, who had received 15 minutes of training on the use of the camera, took the photographs. Quality of photographs was graded. Photographs were reviewed by 2 neuro-ophthalmologists. Treating neurologists completed a survey on the use of this technology in the neurology clinic. Feasibility parameters including ease, comfort, speed, quality, and clinical relevance of nonmydriatic fundus photography was assessed. We obtained 505 fundus photographs of 206 patients. Median time to completion of photographs per patient was 2.12 minutes. Mean rating for ease, comfort, and speed was 9.7 out of 10. Among these, 160 had normal and 44 had abnormal findings. In 114 of 206 patients, neurologists relied on photographs for ocular fundus assessment. In the remaining 92 patients, 18 patients had abnormal photographs, of which neurologists missed the abnormality in 14 (78%). All neurologists preferred nonmydriatic fundus photography over direct ophthalmoscopy. Using nonmydriatic fundus photography in an outpatient neurology clinic is feasible without disrupting patient flow or causing patient discomfort. Findings of optic nerve pallor, optic nerve swelling, or normal optic nerves were particularly relevant to these patients seen for headaches or demyelinating disease and helped inform immediate diagnosis and management.

UNILATERAL NEURORETINITIS AS A LATE-ONSET MANIFESTATION OF THE CHIKUNGUNYA FEVER: A CASE SERIES

To report 3 cases of unilateral neuroretinitis associated with the chikungunya fever (CHIKV). Retrospective noncomparative case series. Three female patients with similar complains of blurry vision and decreased visual acuity that started several weeks after experiencing symptoms of the CHIKV. All patients had decreased best-corrected visual acuity and distortion on the Amsler grid in the affected eye. Evidence of unilateral optic nerve swelling and macular exudates was noted in all patients. No pharmacologic therapy was given. At 2-month follow-up evaluation, all patients had regained at least 3 lines in the Snellen chart, and subretinal fluid and optic nerve swelling had resolved. Unilateral neuroretinitis can present as a late-onset manifestation of CHIKV. Three patients with diagnosis of CHIKV developed unilateral neuroretinitis weeks after the acute viral syndrome, suggesting a possible immunological etiology for this manifestation. To our knowledge, this is the first case series of CHIKV leading to neuroretinitis in the Western World.

Resurgence of ocular syphilis in British Columbia between 2013–2016: a retrospective chart review

ObjectiveTo characterize the trends and explore the demographics, ophthalmic manifestations, and outcomes of ocular syphilis cases in British Columbia. DesignRetrospective chart review. ParticipantsOcular syphilis cases reported to the British Columbia Centre for Disease Control (BCCDC) between January 2013 and December 2016. MethodsThe demographic and clinical data were extracted from the BCCDC's centralized sexually transmitted infection database and the ophthalmologists’ clinical charts. ResultsThere was a steady increase in the rate of syphilis infection per 100 000 population, from 3.4 in 2010 to 18.4 in 2018. There were 39 ocular syphilis cases identified from January 2013 to December 2016. The median age was 50 years (interquartile range: 40–59.5 years); 82.1% were male and 51.3% were HIV positive. The clinical charts belonging to 32 patients were available for review, 14 of which (43.8%) presented with bilateral ocular complaints (46 affected eyes). The most commonly noted ocular presentations were uveitis (93.5%), including retinal vasculitis in 54.3%, and optic nerve involvement in 65.2% (which included papillitis, optic nerve swelling, or pallor). Panuveitis was the most frequent type of uveitis (52.2% of all eyes); 77.8% of affected eyes with best-corrected visual acuity (BCVA) ≤20/50 on presentation had an improvement of 2 or more Snellen lines of visual acuity at their final assessment. At presentation, 37.0% of eyes had BCVA ≤20/200, which decreased to 17.1% at final assessment. ConclusionOcular syphilis, although rare, is on the rise globally and can result in serious ocular sequelae. A high index of suspicion is required for proper diagnosis and treatment.