Neuro-ophthalmic Manifestations Research Articles

Neuroophthalmic Manifestations and Management of Cryptococcal Meningitis in HIV-negative Pregnant Woman : A Rare Case

Abstract
 Introduction : Cryptococcal meningitis (CM) is Cryptococcus infection affecting mostly immunocompromise patients. However, immunocompetent and pregnant woman can also be affected. This condition can cause neuroophthalmic manifestations which may result in permanent vision loss or even death if not managed properly. However, literatures on neuroophthalmic manifestations and management of HIV-negative pregnant woman with CM are very scarce.
 Case Illustration : We present a rare case of 25 y.o. HIV-negative pregnant woman with neuroophthalmic manifestations associated with CM. The patient was consulted from neurology department with complaints of painless vision loss accompanied by diplopia, severe headache, nausea, and frequent vomitus. Ophthalmological examination showed best corrected visual acuity 20/40 in both eyes, ophthalmoplegia, and grade 4 papilledema. Lumbal puncture result was 89 cmH20. Cerebrospinal fluid (CSF) analysis showed mononuclear cells domination, increased protein, and decreased glucose. Indian ink stain showed Cryptococcus organism. Patient was given intravenous fluconazole, topical nonsteroid anti-inflammatory drug, and therapeutic lumbal punctures which result in clinical improvement.
 Discussion : CM can cause neuroophthalmic manifestations resulting from increase intracranial pressure (ICP) due to fungal load in arachnoid granulation which obstruct CSF outflow. The manifestations found in this patient were papilledema, ophthalmoplegia, and diplopia due to 6th nerve palsy. The management was multidisciplinary with obstetrics-gynecology and neurology department, focused in pathogen eradication and ICP management to prevent neuroophthalmic sequelae, with considerationof maternal-fetal life saving dilemma.
 Conclusion : Various neuroophthalmic manifestations can be involved in CM. Prompt and appropriate therapy in a rare case of HIV-negative pregnant woman with CM can decrease both maternal and fetal morbidity and mortality risk.

Neuro-ophthalmic manifestations in traumatic brain injury: a hospital-based study from Nepal.

Neuro-ophthalmic manifestations occur frequently in patients with a traumatic head injury which are often ignored, leading to late presentation with permanent visual disability and visual field defects. This study aimed to find the various neuro-ophthalmic manifestations of traumatic brain injury and correlate them with the neurological status and neuroimaging abnormality. A hospital-based cross-sectional study was carried out among patients admitted to our centre with a diagnosis of traumatic brain injury for a period of six months from August 2020 to January 2021. Glasgow Coma Score was used to grade the severity of the head injury. Detailed ophthalmological examinations were documented in predesigned proforma. Descriptive statistics were used to describe the outcomes. A total of 377 head injury patients underwent neuro-ophthalmic examination-271 (71.9%) were males and 106 (28.1%) were females. The mean age in our study population was 38.6 ± 16.8 years, their age ranging from 3 to 85 years. Time from injury to ophthalmologic examination ranged from 30 min to 12 days post-injury, the mean duration being 24.2 ± 34 h. Road traffic accidents were the most common cause of head injury, with 203 cases (53.8%). 38 (10.1%) patients had neuro-ophthalmic manifestations-16 (42.0%) had afferent pathway deficits and 22 (57.9%) had efferent pathway deficits. The most frequently encountered neuro-ophthalmic abnormality was optic neuropathy in 14 (36.8%) followed by trochlear, oculomotor, abducens nerve, and chiasmal injury. The presence of brain contusion was associated with traumatic optic neuropathy while skull fractures were associated with trochlear nerve palsy, these associations being statistically significant (P<0.05). Neuroimaging abnormalities, particularly brain contusions and skull fractures were significantly associated with neuro-ophthalmic deficits.

Herpes zoster in neuro-ophthalmology: a practical approach.

Herpes Zoster (HZ) or shingles is the reactivation of the Varicella Zoster Virus (VZV), usually along a single sensory nerve, but can affect both sensory and motor cranial nerves. Major risk factors for HZ include immunosuppressed status and age older than 60 years. In the United States, the lifetime risk of HZ is approximately 30%. Worldwide, the median incidence of HZ is 4-4.5 per 1000 person-years across the Americas, Eurasia, and Australia. HZ ophthalmicus, occurring in 10-20% of patients, is an ophthalmic emergency characterized by VZV reactivation along the V1 branch of the trigeminal nerve. Approximately half of this patient subgroup will go on to develop ocular manifestations, requiring prompt diagnosis and management. While anterior segment complications are more common, neuro-ophthalmic manifestations are rarer and can also occur outside the context of overt HZ ophthalmicus. Neuro-ophthalmic manifestations include optic neuropathy, acute retinal necrosis or progressive outer retinal necrosis, cranial neuropathy (isolated or multiple), orbitopathy, and CNS manifestations. Although typically a clinical diagnosis, diagnosis may be aided by neuroimaging and laboratory (e.g., PCR and serology) studies. Early antiviral therapy is indicated as soon as a presumptive diagnosis of VZV is made and the role of corticosteroids remains debated. Generally, there is wide variation of prognosis with neuro-ophthalmic involvement. Vaccine-mediated prevention is recommended. In this review, we summarize neuro-ophthalmic manifestations of VZV.

Update on Neuro-ophthalmic Manifestations of Immune Checkpoint Inhibitors.

Immune checkpoint inhibitor (ICI) use has been on the rise for treatment of many different malignancies. Subsequently, more has been learned about immune-related adverse events (irAEs) that occur up to 12months after treatment. This review summarizes the latest findings and management of neuro-ophthalmic associated irAEs. irAEs can affect the afferent and efferent neuro-ophthalmic pathways, thereby targeting central and peripheral nervous systems. As more cases are being reported, it is becoming apparent that neuro-ophthalmic irAEs often present with atypical features when compared to their spontaneous autoimmune counterparts. These neuro-ophthalmic presentations can also be signs of a more extensive inflammatory process that spans other organ systems, such as myopathies, endocrinopathies, and paraneoplastic syndromes. Awareness of neuro-ophthalmic irAEs and their atypical presentations can lead to early detection, termination of ICI treatment, and immunosuppressant therapy initiation.

Ocular Manifestations of Multiple Sclerosis: A Population-Based Study.

To evaluate the population-based frequency and severity of multiple sclerosis (MS)-related ocular diseases. Retrospective, population-based study examining patients with MS between January 1, 1998 and December 31, 2011. Patients were identified using the Rochester Epidemiology Project, which is a record-linkage system of medical records for all patient-physician encounters among Olmsted County, Minnesota residents. Diagnosis of MS was confirmed based on neuroimaging, cerebrospinal fluid studies, and serum studies for each patient according to the 2017 McDonald criteria. Patient data were obtained using the medical records and followed through April 1, 2018. Of the 116 patients with MS, 66% were female and the median age of onset was 36 years (interquartile range 27.5-43.5 years). About half (61/116, 53%) had MS-related neuro-ophthalmic manifestations during their disease course, and about one-fourth (33/116, 28%) had visual symptoms as their presenting symptom of MS, most commonly as optic neuritis (26/116, 22%). Optic neuritis was the leading MS-related ocular condition (37%), followed by internuclear ophthalmoplegia (16%) and nystagmus (13%). Optic neuritis was mostly unilateral (40/43, 93%), with 16% (6/43) having a visual acuity of 20/200 or worse at nadir but ultimately 95% (35/37) improving to a visual acuity of 20/40 or better. This study provides the population-based frequency of MS-related ocular disease, which demonstrates a high frequency of ocular manifestations in MS both at disease onset and during the disease course, emphasizing the utility of neuro-ophthalmologists, or collaboration between neurologists and ophthalmologists, in the care of patients with MS.

A review of neuro-ophthalmic sequelae following COVID-19 infection and vaccination.

It has become increasingly clear that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can affect most organs in the human body, including the neurologic and ophthalmic systems. Vaccination campaigns have been developed at rapid pace around the world to protect the population from the fast-mutating virus. This review seeks to summarise current knowledge of the neuro-ophthalmic manifestations of both COVID-19 infection and vaccination. Electronic searches for published literature were conducted using EMBASE and MEDLINE on the 30th of July 2023. The search strategy comprised of controlled vocabulary and free-text synonyms for the following terms in various combinations: "coronavirus, COVID-19, SARS-CoV-2, 2019-nCoV, vaccination, vaccine, immunisation and neuro-ophthalmology". No time range limits were set for the literature search. Published English abstracts for articles written in a different language were screened if available. A total of 54 case reports and case series were selected for use in the final report. 34 articles documenting neuro-ophthalmic manifestations following COVID-19 infection and 20 articles with neuro-ophthalmic complications following COVID-19 vaccination were included, comprising of 79 patients in total. The most commonly occurring condition was optic neuritis, with 25 cases following COVID-19 infection and 27 cases following vaccination against COVID-19. The various COVID-19 vaccines that are currently available are part of the global effort to protect the most vulnerable of the human population. The incidence of neuro-ophthalmic consequences following infection with COVID-19 is hundred-folds higher and associated with more harrowing systemic effects than vaccination against the virus.

The Optic Neuritis Developed During COVID-19 Infection - a Case Report

Background: COVID-19 affects all organ systems, including the visual organs. Clinical manifestations encompass all segments of the eye. Neuro-ophthalmic manifestations are rare and can occur during the acute phase of the illness, during recovery, or in the course of Long COVID. These clinical manifestations include optic neuritis and various forms of encephalomyelitis. Optic neuritis more commonly occurs during the recovery phase from the acute form or during Long COVID. Family physicians are in a position to first diagnose optic neuritis in the early stages. Objective: The aim of this paper is to present a case of optic neuritis occurring in the early stages of the disease, diagnosed in the prehospital period in a family medicine practice with consultations from ophthalmologists, infectologists, and neurologists. Such close collaboration enabled the early administration of corticosteroid therapy. Case report: A case of a 28-year-old woman is presented, who exhibited symptoms of COVID-19 with a confirmed positive antigen for the SARS-CoV-2 virus (PCR nasopharyngeal swab). On the fourth day of illness, sudden loss of vision in the right eye occurred along with headache. Early diagnosis and early administration of corticosteroid therapy led to significant improvement in vision. Conclusion: Clinical manifestations of eye diseases can occur in the early stages of COVID-19 as a direct consequence of viral infection. Ophthalmologist and neurologist examinations are necessary in the early stages of the disease for timely diagnosis of various ophthalmologic and neurologic disorders and adequate therapy.

Yearly Occurrence and Seasonality of Neuro-ophthalmic Manifestations of Pediatric Lyme Disease.

To review the neuro-ophthalmic manifestations of Lyme disease at a central Ohio pediatric tertiary care center. A retrospective chart review of patients diagnosed as having Lyme disease from September 2015 to September 2020 was completed. Demographic information, diagnosis dates, and manifestations of Lyme disease were recorded. Patients were excluded for age older than 18 years or lack of corroborated Lyme disease diagnosis. Descriptive statistics were performed. Of the 212 cases of pediatric Lyme disease reviewed, 50 patients had neuroborreliosis. The data showed an increase in Lyme disease and neuroborreliosis cases from 2018 to 2020, with a preponderance of diagnoses in the summer months. Twenty-four patients had meningitis, and 6 of these patients (25%) were diagnosed as having bilateral optic disc edema that was clinically consistent with intracranial hypertension. Papilledema in the setting of Lyme meningitis may be more common than previously reported in central Ohio. If Lyme disease meningitis is suspected, an opening pressure should be recorded at the time of lumbar puncture and, if elevated, an ophthalmologic evaluation for optic nerve edema is indicated. [J Pediatr Ophthalmol Strabismus. 20XX;X(X):XXX-XXX.].

Neuro-ophthalmic manifestations of autoimmune disorders: diagnostic pearls & pitfalls.

The purpose of this review is to highlight a clinical-anatomical approach to localizing neuro-ophthalmic manifestations of associated autoimmune disorders. Our understanding of autoimmune conditions has changed considerably over recent years, particularly with the emergence of novel autoantibodies. Cardinal neuro-ophthalmic signs and symptoms of antibody-mediated autoimmune disorders have been well characterized; knowledge thereof may be the first step towards an accurate diagnosis. A thorough history, further refined by a comprehensive examination are cornerstones to disease localization in clinical medicine. Taken together, these essential steps both guide investigations and facilitate early recognition of autoimmune disorders. From a neuro-ophthalmic perspective, it is important to understand heralding signs and symptoms of autoimmune syndromes, avoid cognitive errors, and remain mindful of common diagnostic pitfalls to optimize care. http://links.lww.com/COOP/A61.

Insights into Novel Choroidal and Retinal Clinical Signs in Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1) is a rare inherited neurocutaneous disorder with a major impact on the skin, nervous system and eyes. The ocular diagnostic hallmarks of this disease include iris Lisch nodules, ocular and eyelid neurofibromas, eyelid café-au-lait spots and optic pathway gliomas (OPGs). In the last years, new manifestations have been identified in the ocular district in NF1 including choroidal abnormalities (CAs), hyperpigmented spots (HSs) and retinal vascular abnormalities (RVAs). Recent advances in multi-modality imaging in ophthalmology have allowed for the improved characterization of these clinical signs. Accordingly, CAs, easily detectable as bright patchy nodules on near-infrared imaging, have recently been added to the revised diagnostic criteria for NF1 due to their high specificity and sensitivity. Furthermore, subclinical alterations of the visual pathways, regardless of the presence of OPGs, have been recently described in NF1, with a primary role of neurofibromin in the myelination process. In this paper, we reviewed the latest progress in the understanding of choroidal and retinal abnormalities in NF1 patients. The clinical significance of the recently revised diagnostic criteria for NF1 is discussed along with new updates in molecular diagnosis. New insights into NF1-related neuro-ophthalmic manifestations are also provided based on electrophysiological and optical coherence tomography (OCT) studies.

Neuro-ophthalmic manifestations of Ehlers-Danlos syndrome.

To review the neuro-ophthalmic manifestations of Ehlers-Danlos syndrome (EDS). Ehlers-Danlos syndrome (EDS) is a rare genetic disorder with an estimated prevalence of 1 in 5000 individuals, but its true prevalence may be underestimated because of variable clinical presentations and limited awareness among healthcare professionals. The neuro-ophthalmic features of EDS may be difficult to recognize in context but new molecular genetic testing is now available for identification of specific subtypes of EDS. Ophthalmic manifestations of EDS include loss of vision and double vision (strabismus), high myopia, retinal detachment, and blue sclera. The vascular subtype of EDS can present as a carotid-cavernous fistula, intracranial aneurysm, or arterial dissection.

Evaluating the Incidence and Neuro-Ophthalmic Manifestations of Carotid-Cavernous Fistulas.

To determine the incidence of carotid-cavernous fistula (CCF) and describe the neuro-ophthalmic manifestations and eventual clinical outcomes. This was a population-based, retrospective cohort study using the Rochester Epidemiology Project to identify patients aged 18 years or older residing in Olmsted County, MN, diagnosed with CCF from 1997 to 2019. The medical records were reviewed for ophthalmic signs and symptoms, including conjunctival chemosis, proptosis, orbital bruit, diplopia, ophthalmoplegia, orbital pain, ocular hypertension, and blurred vision. Also determined was the number of patients with CCF found incidentally on neuroimaging, without clinical manifestations. Ten patients were diagnosed with a CCF between 1997 and 2019 with an overall incidence rate of 0.37 per 100,000 per year (95% CI 0.20-0.68). The median age was 50.5 years (range 23-74 years), 6 (60%) were female, and 9 were White and 1 patient was Korean. Three patients (30%) were asymptomatic and found incidentally on imaging that was obtained for unrelated reasons, and one patient's ocular details were unavailable because she passed away from severe head trauma. The following neuro-ophthalmologic or ocular manifestations were identified in the remaining 6 patients: chemosis/conjunctival injection (n = 6), cranial nerve (CN) VI palsy (n = 6), CN III palsy (n = 2), proptosis (n = 4), ocular/orbital pain (n = 3), audible orbital bruit (n = 2), ocular hypertension (n = 1), and blurred vision (n = 1). Of those patients with symptomatic CCFs, all underwent treatment except for one that spontaneously resolved. None of the patients suffered a stroke or cerebral hemorrhage. The 3 patients with incidentally discovered CCFs were asymptomatic and did not require treatment. This is the first population-based study to show a low incidence rate of CCFs, supporting the notion that it is an uncommon condition. Neuro-ophthalmic manifestations are common, especially chemosis/conjunctival injection and CN VI palsy. Up to a third of patients with CCF can be asymptomatic and may be found incidentally on neuroimaging during the evaluation for unrelated symptoms.

Features of the Course and Rare Ophthalmic Manifestations of Post-COVID Syndrome

The review presents data from studies of the ophthalmological consequences of the disease caused by SARS-CoV-2, especially regarding its rare manifestations. Clinical manifestations of the post-COVID-19 syndrome disappear within about two weeks in mild cases and 3 to 12 weeks in more severe cases. 80 % of patients have more than one, often more than two, and more than 10 symptoms. In general, patients improve spontaneously and continuously over time. The ophthalmic manifestations of COVID-19 vary in nature, severity, and timing, and are more common in patients with severe systemic disease, abnormal blood counts, and inflammatory conditions. Ophthalmic manifestations can develop at any time during the course of the disease. The median time to symptom onset since COVID-19 diagnosis is 5 days for neuro-ophthalmic manifestations, 8.5 days for the ocular surface and anterior segment of the eye, and 12 days for the posterior segment and orbit. COVID-19 causes ocular manifestations in approximately 11 % of patients. The most common ocular manifestation is conjunctivitis, which affects almost 89 % of patients with eye diseases. Other much less common anterior segment anomalies caused by SARS-CoV-2 include scleritis, episcleritis, and acute anterior uveitis. Posterior segment injuries caused by SARS-CoV-2 are mainly vascular, such as hemorrhages, cotton wool spots, dilated veins, and vasculitis. Rare complications of COVID-19 include rhino-orbital-cerebral mucormycosis; retrobulbar optic neuropathy, papillitis, neuroretinitis, anterior ischemic optic neuropathy, non-arterial anterior ischemic optic neuropathy; retinal white spot syndrome (MEWDS); acute unilateral anterior uveitis, serpiginous choroiditis; bilateral central serous chorioretinopathy; bilateral acute depigmentation of the iris, bilateral acute transillumination of the iris; refraction changes; complications associated with drugs used to treat COVID-19 that have toxic effects on eye tissue; and vaccine-associated uveitis.

Collapsin response mediator protein 5-associated optic neuropathy: clinical characteristics, radiologic clues, and outcomes.

Collapsin response mediator protein 5-associated optic neuropathy (CRMP5-ON) is a rare entity of autoimmune optic neuropathy. This study aimed to review the neuro-ophthalmic findings and outcomes in a series of patients with CRMP5-ON to further characterize its clinical phenotype, radiologic clues, and outcomes. This was a retrospective case series and a single-center medical chart review of all patients with CRPM5-seropositive ON at the Department of Neurology, Beijing Tongren Hospital, from December 1, 2020, to March 31, 2023. The main outcome measures were neuro-ophthalmic manifestations, radiologic characteristics, and clinical outcomes of CRMP5-ON; coexisting neural autoantibody, paraneoplastic associations, and the impact of immunosuppressant therapy. Five patients were identified. Four (80%) were female, and the average age at onset was 59.4 years (range 53-69 years), with an average follow-up of 15.3 months (range 1.4-28.7 months). The average best-corrected visual acuity (BCVA) at nadir was 20/120 (range 20/20 to count fingers). Seven of ten affected eyes (70%) showed diffuse defects of the central field. Painless bilateral involvement and optic disk edema occurred in 100% of patients, combined with vitritis, uveitis, or retinitis in four (80%). Four patients (80%) had MRI abnormalities along the optic nerve (one patient with optic nerve enhancement and three patients had optic nerve sheath enhancement or peribulbar fat enhancement). Three patients (60%) had optic neuropathy with other neurologic symptoms. Four patients (80%) had confirmed cancer (two were small-cell lung carcinoma, one was papillary thyroid carcinoma and another was thymoma and invasive pulmonary adenocarcinoma). All cancers were identified after the presentation of the optic neuropathy. The intervention included IVIG, IVMP, surgery and chemotherapy. The average BCVA at the last follow-up was 20/50 (range 20/20 to count fingers). Three patients had surgery during the initial hospitalization, and were stable during the follow-up. Among two patients who received IVMP, both had improvement after treatment, although one patient had worsening non-ocular neurologic symptoms during the steroid taper. CRMP5-ON presented with optic disc edema, often bilateral involved and combined with vitreitis, retinitis, or uveitis. CRMP5-ON can present with MRI optic nerve or perineural optic nerve enhancement, especially in the optic nerve sheath. CRMP5-ON is closely related to paraneoplastic neurologic syndrome. Cancer screening and intervention are crucial to prognosis.

Rhino-Orbital Mucormycosis: A Case Series and Review of Literature

Mucormycosis (Zygomycosis) is an invasive fungal infection, often acute and extremely severe caused by opportunist and ubiquitous fungi belonging to the class Phygomycetes, subclass Zygomycetes, order Mucorales, family Mucoraceae. COVID-19 is an infection caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). Since the first case was detected, in December 2019 in Wuhan, China. COVID-19 has been found to have many systemic manifestations. Of these, ocular presentations associated with COVID-19 have been identified. It is acquired by the implantation of the fungal spores in the oral, nasal, and conjunctival mucosa (rhino-orbito-cerebral), by inhalation (pulmonary), or by the ingestion of contaminated food (digestive). The field of neuro-ophthalmology has been affected, due to both neuro-ophthalmic manifestations of COVID-19, as well as adaptations in the delivery of health care and treatment of pre-existing conditions.

Оценка показателей пространственной контрастной чувствительности в диагностике компьютерного зрительного синдрома у пользователей девайсами (данные пилотного исследования)

In the modern world, there is an increase in the severity of computer vision syndrome (CVS) when using digital devices. Despite numerous studies of ophthalmic symptoms of CVS, neuro-ophthalmic manifestations remain poorly understood. Evaluation of spatial contrast sensitivity (SCS) indicators can be relevant and informative in identifying functional changes in the visual system when using devices. A pilot study was conducted with the participation of 15 healthy people. The subjects were assessed the SCS in the process of habitual activity in a personal device, which was considered a visual load using the author's program «Visocontrastometry» after 15 minutes, 30 minutes, 45 minutes of use. The results were analyzed using descriptive statistics, and the nonparametric Mann – Whitney test was used to test for significance. According to the data obtained, in all time intervals, there is a decrease in SCS at the experimental stage compared with the control indicators of SCS. A statistically significant difference is observed 15 minutes after the visual load at a spatial frequency of 1.0 cycle/deg in the blue color range (p ≤ 0.05). The method for assessing the SCS has shown its adequacy for studying the neuro-ophthalmological aspects of CVS. Keywords: сomputer vision syndrome, contrast sensitivity, device

Neuro-ophthalmic Complications in Pediatric Leukemia.

Optic neuropathy in childhood leukemia occurs through multiple direct and indirect mechanisms, including leukemic infiltration of the optic nerve, infection, blood dyscrasias, or adverse effects of treatment. We aimed to characterize visual outcomes in pediatric patients with leukemia-associated neuro-ophthalmic manifestations. We retrospectively identified patients with leukemia and optic nerve pathology over 13 years by diagnostic billing codes. We collected information on demographics, presentation, treatment course, and visual outcomes directly from medical records. Of the 19 patients who met inclusion criteria, 17 (89.5%) had pseudotumor cerebri and 2 had direct optic nerve infiltration. Causes of increased intracranial pressure included central nervous system infiltration (6 of 17), hyperviscosity/leukemia (2 of 17), venous sinus thrombosis (3 of 17), medication induced (5 of 17), and bacterial meningitis (1 of 17). 47.1% (8 of 17) had papilledema at the time of leukemia diagnosis, and 94.1% (16 of 17) of patients with pseudotumor cerebri were treated with acetazolamide. At presentation, 3 patients had decreased vision secondary to macular ischemia, subhyaloid vitreous hemorrhage, or steroid induced glaucoma. Following treatment of pseudotumor cerebri, binocular visual acuity was ≥20/25 in all patients. One patient with optic nerve infiltration had a final visual acuity of count fingers in the affected eye. In our chart review, the most common mechanism of neuro-ophthalmic involvement in pediatric leukemia was elevated intracranial pressure from a myriad of causes. Visual outcomes from patients with elevated intracranial pressure were excellent. Understanding the mechanisms by which leukemia can cause optic nerve disease in pediatric patients can facilitate earlier diagnosis and treatment and potentially improve visual outcomes.

Neuro-Ophthalmic Visual Impairment in the Setting of COVID-19.

We set out to describe in detail the afferent neuro-ophthalmological complications that have been reported in association with coronavirus disease 2019 (COVID-19) infection. We describe and elaborate on mechanisms of disease, including para-infectious inflammation, hypercoagulability, endothelial damage, and direct neurotropic viral invasion. Despite global vaccination programs, new variants of COVID-19 continue to pose an international threat, and patients with rare neuro-ophthalmic complications are likely to continue to present for care.Afferent complications from COVID-19 include homonymous visual field loss, with or without higher cortical visual syndromes, resulting from stroke, intracerebral hemorrhage, or posterior reversible leukoencephalopathy. Optic neuritis has frequently been reported, sometimes along with acute disseminated encephalomyelopathy, often in association with either myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) or less commonly aquaporin-4 seropositivity or in newly diagnosed multiple sclerosis. Ischemic optic neuropathy has rarely been reported. Papilledema, resulting either from venous sinus thrombosis or idiopathic intracranial hypertension in the setting of COVID-19, has also been described.Observed afferent neuro-ophthalmic associations need to be confirmed though larger comparative studies. Meanwhile, the range of possible complications should be recognized by neurologists and ophthalmologists alike, to facilitate faster diagnosis and treatment of both COVID-19 and its neuro-ophthalmic manifestations.

Eye damage in COVID-19. Part 2: posterior segment complications, neuro-ophthalmic manifestations, vaccination and risk factors

Posterior eye segment involvement in COVID-19 has varied manifestations: vascular, inflammatory, and neuronal. All of them are triggered by SARS-CoV-2 virus but they cannot be viewed as exclusively specific to COVID-19. According to the literature, the mean age of the patients varies from 17 to 75 with the median of 50 years. The median duration between the appearance of ophthalmic symptoms and the detection of COVID-19 was 12 days. The disease affects both men and women equally. Direct exposure to the virus, immune-mediated tissue damage, activation of the coagulation system, the prothrombotic state caused by a viral infection, concomitant diseases and medications used in the treatment contribute to the development of eye pathologies. Ophthalmologists should be aware of the possible relations of posterior eye segment pathologies, orbit and neuro-ophthalmic disorders with SARS-CoV-2, as well as the possible exacerbation of chronic forms of inflammatory eye diseases and autoimmune disorders due to anti-COVID-19 vaccination.

Disturbances of Ocular Circulation in Color Doppler Imaging, Retinal Changes and Electrophysiological Tests with Neuro-Ophthalmological Clinical Symptoms in the Course of CADASIL Syndrome—A Case Report

The authors present a new paper examining the disturbances in ocular circulation and electrophysiological changes in the presence of neuro-ophthalmic manifestations in a patient with cerebral autosomal dominant arteriopathy with subcortical infracts and leucoencephalopathy (CADASIL). Symptoms reported by the patient included: transient vision loss (TVL), migraines, diplopia, bilateral peripheral visual field loss and convergence insufficiency. CADASIL was confirmed by the presence of NOTCH3 gene mutation (p.Cys212Gly), the presence of granular osmiophilic material (GOM) in cutaneous vessels in an immunohistochemistry test (IHC) and bilateral focal vasogenic lesions in the white matter of the cerebral hemisphere, with micro-focal infarct in the left external capsule on a magnetic resonance imaging test (MRI). Color Doppler imaging (CDI) confirmed decreased blood flow and increased vascular resistance in the retinal and posterior ciliary arteries, with reduced P50 wave amplitude on a pattern electroretinogram (PERG). An eye fundus examination and fluorescein angiography (FA) revealed the constriction of retinal vessels and a peripheral retinal pigment epithelium (RPE) atrophy with focal drusen. The authors suggest that the cause of TVL may be changes in the hemodynamics of the retinochoroid vessels associated with the narrowing of small vessels and the presence of druses in the retina-which is supported by a decrease in the amplitude of the P50 wave in PERG, changes in OCT correlating simultaneously with changes in MRI imaging and other neurological symptoms.