Purpose: To assess the diagnostic accuracy of visual field results generated by the newly developed software (CU-VF) and the standard automated perimetry (SAP) for detecting hemianopia.
Patients and methods: Forty-three subjects with hemianopia and 33 controls were tested with the CU-VF software on a personal computer and SAP. Hemianopia was defined as the presence of a hemianopic field respecting the vertical meridian on SAP with the corresponding neuroimaging pathology as evaluated by 2 neuro-ophthalmologists. Results of CU-VF were independently evaluated by 2 neuro-ophthalmologists, 1 general ophthalmologist, and 1 general practitioner in terms of the presence of hemianopia. Sensitivity, specificity, and kappa coefficient for inter-observer reliability were calculated. Satisfaction and ease of use were evaluated with a visual analog-scale questionnaire and analyzed using paired t-test.
Results: The sensitivity (95% CI) and specificity (95% CI) of the CU-VF to detect hemianopia was 74.42% (58.53-85.96) and 93.94% (78.38-99.94). Kappa coefficient between neuro-ophthalmologists versus general ophthalmologist and general practitioner were 0.71 and 0.84, respectively. The mean (SD) test duration was 2.25 (0.002) minutes for the CU-VF and 5.38 (1.34) minutes for SAP (p < 0.001). Subjects reported significantly higher satisfaction and comfort using the CU-VF software compared to SAP.
Conclusion: The CU-VF screening software showed good validity and reliability to detect hemianopia, with shorter test duration and higher subject satisfaction compared to SAP.
Purpose: Management of optic nerve sheath meningiomas (ONSM) remains challenging. Photon radiation therapy (PhRT) is the most common treatment for sight-threatening ONSM. Proton beam therapy (PBT) is less commonly used because it is more expensive and because there are questions about its efficacy specifically in relation to ONSM. PBT has the theoretical advantage of reducing radiation exposure to adjacent structures. We report the visual outcome of patients with primary ONSM managed at the Fondation Ophtalmologique Adolphe de Rothschild, Paris, France, and treated with PBT at the Centre de Protonthérapie, Institut Curie, Orsay, France.
Methods: We conducted a retrospective review of all patients with primary ONSM who received PBT (either by itself or following surgery) between January 2006 and January 2019. Neuro-ophthalmic examinations were performed at presentation and after radiotherapy, and, when applicable, after surgery. Meningiomas were measured at the time of diagnosis and at each follow-up MRI examination.
Results: Sixty patients (50 women, 10 men; mean age, 45.2±11.1y) were included, of whom 29 underwent surgery. At presentation, 52 (87%) of them had decreased vision (average visual acuity: 0.6 logMAR). Fundus examination showed optic disc swelling (n=27; 46.5%), optic disc pallor (n=22; 37.9%), optic disc cupping (n=2; 3.4%), opto-ciliary shunt (n=8; 13.8%), or choroidal folds (n=5; 8.6%). Otherwise, it was unremarkable (n=7; 12.1%). After treatment, visual function was stable overall. Fundus examination showed pallor (n=47; 83.9%), swelling (n=3; 5.4%), or cupping (n=2; 3.4%) of the optic disc, or was unremarkable (n=5; 8.9%). The visual field of 8 patients worsened, while 3 developed asymptomatic retinal hemorrhages. Tumor shrunk significantly in 8 patients at 1 year after PBT and remained stable in size in all others. Patients with opto-ciliary shunts had significantly worse visual outcome than other patients. Retinal abnormalities were observed in 11 patients during follow-up.
Conclusion: PBT alone or in association with surgery appears to be a safe and efficient treatment for ONSM, reducing the tumor size and stabilizing visual function. The risk of developing radiation retinopathy seems to be higher when patients had upfront surgery.
Purpose: Schizophrenia is associated with alterations in neural structure and function of the retina that are similar to changes seen in the retina and brain in multiple neurodegenerative disorders. Preliminary evidence suggests that retinal microvasculature may also be compromised in schizophrenia. The goal of this study was to determine, using optical coherence tomography angiography (OCTA), whether 1) schizophrenia is associated with alterations in retinal microvasculature density; and 2) microvasculature reductions are associated with retinal neural layer thinning and performance on a measure of verbal IQ.
Patients and methods: Twenty-eight outpatients with schizophrenia or schizoaffective disorder and 37 psychiatrically healthy control subjects completed OCT and OCTA exams, and the Wechsler Test of Adult Reading.
Results: Schizophrenia patients were characterized by retinal microvasculature density reductions, and enlarged foveal avascular zones, in both eyes. These microvascular abnormalities were generally associated with thinning of retinal neural (macular and peripapillary nerve fiber layer) tissue (but the data were stronger for the left than the right eye) and lower scores on a proxy measure of verbal IQ. First- and later-episode patients did not differ significantly on OCTA findings.
Conclusion: The retinal microvasculature impairments seen in schizophrenia appear to be a biomarker of overall brain health, as is the case for multiple neurological conditions. Additional research is needed, however, to clarify contributions of social disadvantage and medical comorbidities to the findings.
Down syndrome, caused by an extra copy of all or part of chromosome 21, is the most prevalent intellectual disability of genetic origin. Among numerous comorbidities which are part of the phenotype of individuals with Down syndrome, ocular problems appear to be highly prevalent. Neuro-ophthalmological manifestations, such as ocular alignment and motility disturbances, amblyopia, hypoaccommodation or optic nerve abnormalities, and other organic ocular anomalies frequently reported in Down syndrome, may lead to an overall decrease in visual acuity. Although numerous studies have reported ocular anomalies related to Down syndrome, it remains challenging to determine the impact of each anomaly upon the decreased visual acuity, as most such individuals have more than one ocular problem. Even in children with Down syndrome and no apparent ocular defect, visual acuity has been found to be reduced compared with typically developing children. Pediatric ophthalmological examination is a critical component of a multidisciplinary approach to prevent and treat ocular complications and improve the visual outcome in children with Down syndrome. This narrative review aims to provide a better understanding of the neuro-ophthalmological manifestations and discuss the current ophthalmological management in children with Down syndrome.
Purpose: Electrical stimulation of the human central nervous system via surface electrodes has been used for both learning enhancement and the amelioration of neurodegenerative or psychiatric disorders. However, data are sparse on how such electrical stimulation affects neural circuits at the cellular level. This study assessed the effects of tACS-like currents at 10 Hz on On-center retinal ganglion cell responsiveness, using the rabbit retina eyecup preparation as a model for central nervous system effects.
Methods: We made extracellular recordings of light-evoked spike responses in different classes of On-center retinal ganglion cells before, during and after brief applications of 1 microampere alternating currents using single electrodes and microelectrode arrays.
Results: tACS-like currents (tACS) of 1 microampere produced effects on On-center ganglion cell response profiles immediately after initiation or cessation of tACS, without driving phase-locked firing in the absence of light stimuli. tACS affected the initial transient responses to light stimulation for all cells, sustained response components (if any) more strongly for sustained cells, and the center-surround balance more strongly for transient cells.
Conclusion: tACS sculpted light-evoked responses that lasted for one or more hours after cessation of current without, itself, directly inducing significant firing changes. Functionally, tACS effects could result in effects on contrast thresholds for both broad classes of cells, but because tACs differentially affects the center-surround balance of transient On-center cells, there may be greater effects on the spatial resolution and gain. The isolated retina appears to be a useful model to understand tACS actions at the neuronal level.
Glaucoma and macular degeneration are leading causes of irreversible blindness, significantly compromising the quality of life and having a high economic and social impact. Promising therapeutic approaches aimed at regenerating or bypassing the damaged anatomical-functional components are currently under development: these approaches have generated great expectations, but to be effective require a visual network that, despite the pathology, maintains its integrity up to the higher brain areas. In the light of this, the existing findings concerning how the central nervous system modifies its connections following the pathological damage caused by glaucoma and macular degeneration acquire great interest. This review aims to examine the scientific literature concerning the morphological and functional changes affecting the central nervous system in these pathological conditions, summarizing the evidence in an analytical way, discussing their possible causes and highlighting the potential repercussions on the current therapeutic perspectives.
Purpose: To investigate the prevalence of retinal vein occlusions (RVOs) and associated factors in a Chinese population.
Patients and methods: The cross-sectional community-based Kailuan Eye Study included individuals who participated in the Kailuan Study. RVOs were diagnosed on the fundus photographs. Estimated cerebrospinal fluid pressure (eCSFP) was calculated as "eCSFP=0.44*Body Mass Index+0.16*Diastolic Blood Pressure-0.18*Age".
Results: The study included 12,499 participants with a mean age of 52.9±13.1 years. The overall prevalence of RVO was 120/12,499 or 0.96%, with branch RVOs observed in 116/12,499 individuals and central RVOs in 4/12,499 individuals. RVOs started at the optic disc in 19 participants (15.8% of all RVOs), and in 101 (84.2%) individuals arterio-venous crossings outside the optic disc. In multivariable analysis, a higher RVO prevalence was associated with older age (P<0.001), higher eCSFP (P<0.001), and higher fasting serum glucose concentration (P<0.001). Differentiating between RVOs at arterio-venous crossings and RVOs at the optic disc revealed that the prevalence of both RVO types was associated with higher eCSFP (P<0.001 and P=0.004, respectively) after adjusting for age and fasting serum glucose concentration.
Conclusion: In this adult Chinese population recruited on a community basis, the prevalence of any RVO (mean: 0.96) was associated with older age, higher eCSFP and higher fasting serum glucose concentration. Higher eCSFP may play an etiologic role in RVOs.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease resulting in a gradual loss of motor neuron function. Although ophthalmic complaints are not presently considered a classic symptom of ALS, retinal changes such as thinning, axonal degeneration and inclusion bodies have been found in many patients. Retinal abnormalities observed in postmortem human tissues and animal models are similar to spinal cord changes in ALS. These findings are not dramatically unexpected because retina shares an ontogenetic relationship with the brain, and many genes are associated both with neurodegeneration and retinal diseases. Experimental studies have demonstrated that ALS affects many "vulnerable points" of the retina. Aggregate deposition, impaired nuclear protein import, endoplasmic reticulum stress, glutamate excitotoxicity, vascular regression, and mitochondrial dysfunction are factors suspected as being the main cause of motor neuron damage in ALS. Herein, we show that all of these pathways can affect retinal cells in the same way as motor neurons. Furthermore, we suppose that understanding the patterns of neuro-ophthalmic interaction in ALS can help in the diagnosis and treatment of this disease.