Abstract: Primary vitreoretinal lymphoma (PVRL), as a subset of primary central nervous system lymphoma (PCNSL), is a rare and fatal ocular malignancy. Most PVRL masquerades as chronic posterior uveitis, which makes the clinical diagnosis challenging. Vitreous cells, subretinal lesions and imaging techniques are essential for clinical diagnosis. Importantly, cytopathology/histopathology identification of malignant cells is the gold standard for the diagnosis of PVRL. In addition, molecular detection of immunoglobulin heavy chain (IgH) or T cell receptor (TCR) gene rearrangements, immunophenotyping for cell markers, and cytokine analysis of interleukine-10 elevation are often used as adjunct procedures. Current management of PVRL involves local radiation, intravitreal chemotherapy (methotrexate and rituximab), with or without systemic chemotherapy depending on the involvement of non-ocular tissues. In cases with concomitant PCNSL, systemic high-dose methotrexate/rituximab based therapy in conjunction with local therapy, whole brain radiotherapy and/or autologous stem cell transplantation is considered. Although PVRL normally responds well to initial treatment, high rates of relapse and CNS involvement usually lead to poor prognosis and limited survival. A professional team of medical experts in ophthalmologists, ocular pathologists, neuro-oncologists and hemato-oncologists is essential for optimizing patient management.
Abstract: The Guangzhou Twin Eye Study (GTES) is a population-based study of young twins residing in Guangzhou City. The major aim of GTES is to explore the impact of genes, environmental factors and gene-environment interactions on common eye diseases. From 2006, for more than 1,300 twin pairs, age 7–26 years old, progressive ocular phenotypes, such as refraction, ocular biometrics, weight, and height were collected annually, while non-progressive phenotypes such as parental refraction, corneal thickness, retinal fundus, intraocular pressure and DNA only collected at baseline. In the current study, we summarize the major findings on the etiology of myopia in recent decades.
Abstract: This submission will briefly review the anatomy and physiology of the optic nerve, and highlight various ischemic optic neuropathies including anterior ischemic optic neuropathies (non-arteritis and arteritic), diabetic papillopathy, posterior ischemic optic neuropathies, and ischemic optic neuropathies in the setting of hemodynamic compromise.
Abstract: Optical coherence tomography (OCT) is an ocular imaging technique that can complement the neuro-ophthalmic assessment, and inform our understanding regarding functional consequences of neuroaxonal injury in the afferent visual pathway. Indeed, OCT has emerged as a surrogate end-point in the diagnosis and follow up of several demyelinating syndromes of the central nervous system (CNS), including optic neuritis (ON) associated with: multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Recent advancements in enhanced depth imaging (EDI) OCT have distinguished this technique as a new gold standard in the diagnosis of optic disc drusen (ODD). Moreover, OCT may enhance our ability to distinguish cases of papilledema from pseudopapilledema caused by ODD. In the setting of idiopathic intracranial hypertension (IIH), OCT has shown benefit in tracking responses to treatment, with respect to reduced retinal nerve fiber layer (RNFL) measures and morphological changes in the angling of Bruch’s membrane. Longitudinal follow up of OCT measured ganglion cell-inner plexiform layer thickness may be of particular value in managing IIH patients who have secondary optic atrophy. Causes of compressive optic neuropathies may be readily diagnosed with OCT, even in the absence of overt visual field defects. Furthermore, OCT values may offer some prognostic value in predicting post-operative outcomes in these patients. Finally, OCT can be indispensable in differentiating optic neuropathies from retinal diseases in patients presenting with vision loss, and an unrevealing fundus examination. In this review, our over-arching goal is to highlight the potential role of OCT, as an ancillary investigation, in the diagnosis and management of various optic nerve disorders.
Background: The perception of visual forms is crucial for effective interactions with our environment and for the recognition of visual objects. Thus, to determine the codes underlying this function is a fundamental theoretical objective in the study of the visual forms perception. The vast majority of research in the field is based on a hypothetico-deductive approach. Thus, we first begin by formulating a theory, then we make predictions and finally we conduct experimental tests. After decades of application of this approach, the field remains far from having a consensus as to the traits underlying the representation of visual form. Our goal is to determine, without theoretical a priori or any bias whatsoever, the information underlying the discrimination and recognition of 3D visual forms in normal human adults.
Methods: To this end, the adaptive bubble technique developed by Wang et al. [2011] is applied on six 3D synthetic objects under varying views from one test to another. This technique is based on the presentation of stimuli that are partially revealed through Gaussian windows, the location of which is random and the number of which is established in such a way as to maintain an established performance criterion. Gradually, the experimental program uses participants’ performance to determine the stimulus regions that participants use to recognize objects. The synthetic objects used in this study are unfamiliar and were generated from a program produced at C. Edward Connor’s lab, Johns Hopkins University School of Medicine.
Results: The results were integrated across participants to establish regions of presented stimuli that determine the observers’ ability to recognize them—i.e., diagnostic attributes. The results will be reported in graphical form with a Z scores mapping that will be superimposed on silhouettes of the objects presented during the experiment. This mapping makes it possible to quantify the importance of the different regions on the visible surface of an object for its recognition by the participants.
Conclusions: The diagnostic attributes that have been identified are the best described in terms of surface fragments. Some of these fragments are located on or near the outer edge of the stimulus while others are relatively distant. The overlap is minimal between the effective attributes for the different points of view of the same object. This suggests that the traits underlying the recognition of objects are specific to the point of view. In other words, they do not generalize through the points of view.
Abstract: Pediatric neuro-ophthalmology is a subspecialty within neuro-ophthalmology. Pediatric neuro-ophthalmic diseases must be considered separate from their adult counterparts, due to the distinctive nature of the examination, clinical presentations, and management choices. This manuscript will highlight four common pediatric neuro-ophthalmic disorders by describing common clinical presentations, recommended management, and highlighting recent developments. Diseases discussed include pediatric idiopathic intracranial hypertension (IIH), pseudopapilledema, optic neuritis (ON) and optic pathway gliomas (OPG). The demographics, diagnosis and management of common pediatric neuro-ophthalmic disease require a working knowledge of the current research presented herein. Special attention should be placed on the differences between pediatric and adult entities such that children can be appropriately diagnosed and treated.
