Hereditary Optic Neuropathies: A Systematic Review on the Interplay between Biomaterials and Induced Pluripotent Stem Cells.

Hereditary optic neuropathies (HONs) such as dominant optic atrophy (DOA) and Leber Hereditary Optic Neuropathy (LHON) are mitochondrial diseases characterized by a degenerative loss of retinal ganglion cells (RGCs) and are a cause of blindness worldwide. To date, there are only limited disease-modifying treatments for these disorders. The discovery of induced pluripotent stem cell (iPSC) technology has opened several promising opportunities in the field of HON research and the search for therapeutic approaches. This systematic review is focused on the two most frequent HONs (LHON and DOA) and on the recent studies related to the application of human iPSC technology in combination with biomaterials technology for their potential use in the development of RGC replacement therapies with the final aim of the improvement or even the restoration of the vision of HON patients. To this purpose, the combination of natural and synthetic biomaterials modified with peptides, neurotrophic factors, and other low- to medium-molecular weight compounds, mimicking the ocular extracellular matrices, with human iPSC or iPSC-derived cell retinal progenitors holds enormous potential to be exploited in the near future for the generation of transplantable RGC populations.

Post COVID-19 Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis.

Neurological manifestations of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are relatively common. Although some may be consequences of direct cellular viral invasion (neurotropism), many represent post-infectious inflammation mediated by autoimmune mechanisms. We herein report the case of a 69-year-old diabetic male who presented with bilateral sub-acute, progressive loss of vision 45 days after suffering a presumed SARS-CoV-2 related pneumonia. He had bilateral optic disc oedema. Magnetic resonance imaging showed uniform contrast enhancement of both optic nerves without spinal cord involvement. He tested positive for SARS-CoV-2 IgG and myelin oligodendrocyte glycoprotein (MOG) IgG antibodies. He was treated with intravenous methylprednisolone for 5 days. The optic disc oedema resolved within 6 weeks with improvement in visual acuity, although optic atrophy developed by week 16. The MOG-IgG antibody test turned negative after 24 weeks.

Papillophlebitis in a COVID-19 patient: Inflammation and hypercoagulable state.

INTRODUCTION: Papillophlebitis is a rare condition characterized by venous congestion and optic disc edema, which has been suggested to occur as a consequence of inflammation of the retinal veins or, possibly, the capillaries of the optic disc, leading to venous insufficiency and compression of the central retina vein. The disease affects healthy young adults and commonly has a benign course, however, if complications such as macular edema or ischemia appears, treatment should be instituted immediately to avoid poor prognosis. CASE REPORT: A 40-year old white male patient consulted for a slight decrease in the sensitivity of the visual field in his left eye (OS). Visual acuities (VA) were 20/20 in both eyes. OS fundus examination showed dilated and tortuous retinal vessels, disc edema, and retinal hemorrhages. The patient was diagnosed with papillophlebitis. OS VA decreased to 20/200 due to macular edema, and he was treated with a intravitreal dexamethasone implant. An exhaustive and interdisciplinary exploration process was performed, identifying a recent disease and recovery of Covid-19 as the only factor of inflammation and coagulation alteration. Other systemic diseases were excluded. We also describe a rapid decrease in disc and macular edema after intravitreal dexametasone injection, which could support the inflammatory hypothesis. CONCLUSION: The importance of this case lies in the possible association of papillophlebitis with the new Covid-19 disease. We believe that the inflammatory reaction and the coagulation alteration present in our patient due to Sars-Cov2 coronavirus may have acted as risk factors for the development of papillophlebitis.

Oclusión de la arteria central de la retina como forma de presentación de arteritis de Takayasu / Central retinal artery occlusion as an onset form of Takayasu's arteritis

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Valoración de la formación de postimágenes mediante una nueva aplicación informática en pacientes con neuropatías ópticas desmielinizantes / Appraisal of the formation of afterimages by means of a new computer application in patients with demyelinating optic neuropathies

Introducción. En las neuritis ópticas se afecta, entre otras funciones visuales, la percepción del color. La mayoría de las pruebas existentes para evaluar discromatopsias se basa en evaluar el matiz, pero no se ha estudiado clínicamente la formación de postimágenes en la retina en estos pacientes. Objetivo. Evaluar la discromatopsia adquirida en las neuritis ópticas desmielinizantes mediante la formación de postimágenes en la retina. Sujetos y métodos. Estudio observacional, transversal, de casos y controles. Los casos son pacientes con al menos un episodio de neuritis óptica y esclerosis múltiple confirmada. Se seleccionó un control sano por cada caso, emparejado por edad y sexo. La variable principal es la capacidad de ver postimágenes tras saturar los fotorreceptores retinianos. Para evaluar dicho fenómeno se desarrolló una aplicación informática específica. Resultados. La muestra comprende 30 casos y 30 controles (63% mujeres; edad media: 33 años; rango: 18-48 años). Los casos mostraron menor probabilidad de ver la postimagen (el 36,6% de los casos y ningún control no veían postimagen) y, en caso de verla, una menor permanencia de ella. La curva ROC muestra una sensibilidad del 86,3% y una especificidad del 83,3%. La odds ratio era de 5 (intervalo de confianza al 95%: 2,21-11,3) para la probabilidad de ver la postimagen en los controles frente a los casos. Conclusiones. Los pacientes con al menos un episodio de neuritis óptica presentan una menor capacidad de observar postimágenes, por lo que la prueba es útil para la evaluación y el seguimiento del daño funcional en neuropatías ópticas desmielinizantes (AU)

Introduction. The perception of colour is one of the visual functions affected by optic neuritis. Most of the tests currently available for evaluating dichromatism are based on assessing the hue, but no clinical studies have been conducted to investigate the formation of afterimages on the retina of these patients. Aims. To evaluate the dichromatism acquired in demyelinating optic neuritis by means of the formation of afterimages on the retina. Subjects and methods. This is an observation-based, cross-sectional, case-control study. The cases are patients with at least one bout of optic neuritis and confirmed multiple sclerosis. A healthy age- and sex-paired control was selected for each case. The main variable is the capacity to see afterimages after saturation of the retinal photoreceptor cells. A specific computer application was developed to evaluate this phenomenon. Results. The sample consisted of 30 cases and 30 controls (63% females; mean age: 33 years; range: 18-48 years). The cases showed less probability of seeing the afterimage (36.6% of the cases, while none of the controls failed to see an afterimage) and, if it was seen, it remained for less time. The ROC curve shows a sensitivity of 86.3% and a specificity of 83.3%. The odds ratio was 5 (95% confidence interval: 2.21-11.3) for the probability of seeing the afterimage in controls versus cases. Conclusions. Patients with at least one episode of optic neuritis presented a lower capacity to observe afterimages. The test is therefore useful in the assessment and follow-up of functional damage in demyelinating optic neuropathies (AU)