High diagnostic accuracy of T2FLAIR at 3 T in the detection of optic nerve head edema in acute optic neuritis.

OBJECTIVES: Optic nerve head edema (ONHE) detected by fundoscopy is observed in one-third of patients presenting optic neuritis (ON). While ONHE is an important semiological feature, the correlation between ONHE and optic nerve head MRI abnormalities (ONHMA), sometimes called "optic nerve head swelling," remains unknown. Our study aimed to assess the diagnostic accuracy of T2 fluid-attenuated inversion recovery (FLAIR) MRI sequence in detecting ONHE in patients with acute ON. METHODS: In the present single-center study, data were extracted from two prospective cohort studies that consecutively included adults with a first episode of acute ON treated between 2015 and 2020. Two experienced readers blinded to study data independently analyzed imaging. A senior neuroradiologist resolved any discrepancies. The primary judgment criterion of ONHMA was assessed as optic nerve head high signal intensity on gadolinium-enhanced T2FLAIR MRI sequence. Its diagnostic accuracy was evaluated with both the gold standard of ONHE on fundus photography (FP) and peripapillary retinal nerve fiber layer thickening on optic coherence tomography (OCT). RESULTS: A total of 102 patients were included, providing 110 affected and 94 unaffected optic nerves. Agreement was high between the different modalities: 92% between MRI and FP (k = 0.77, 95% CI: 0.67-0.88) and 93% between MRI and OCT (k = 0.77, 95% CI: 0.67-0.87). MRI sensitivity was 0.84 (95% CI: 0.70-0.93) and specificity was 0.94 (95% CI: 0.89-0.97) when compared with the FP. CONCLUSION: Optic nerve head high T2FLAIR signal intensity corresponds indeed to the optic nerve head edema diagnosed by the ophthalmologists. MRI is a sensitive tool for detecting ONHE in patients presenting acute ON. CLINICAL RELEVANCE STATEMENT: In patients with optic neuritis the high T2FLAIR (fluid-attenuated inversion recovery) signal intensity of the optic nerve head corresponds indeed to optic nerve head edema, which is a useful feature in optic neuritis etiological evaluation and treatment. KEY POINTS: Optic nerve head edema is a prominent clinical feature of acute optic neuritis and is usually diagnosed during dilated or non-dilated eye fundus examination. Agreement was high between magnetic resonance imaging, fundus photography, and optical coherence tomography. Optic nerve head high T2 fluid attenuation inversion recovery signal intensity is a promising detection tool for optic nerve head edema in patients presenting acute optic neuritis.

Cranial nerve involvement, visual complications and headache syndromes in Lyme disease.

PURPOSE OF REVIEW: To provide a summary of the visual manifestations and cranial neuropathies seen in Lyme disease. RECENT FINDINGS: Lyme facial palsy remains the most common manifestation of Lyme neuroborreliosis. Recent investigations show likely evidence of vagal involvement in Lyme disease. SUMMARY: The literature on Lyme neuroborreliosis continues to evolve. Lyme disease can affect nearly any cranial nerve in addition to causing various headache syndromes. The most common manifestation is Lyme disease facial palsy, occurring in up to 5-10% of patients with documented Lyme disease. Headache syndromes are common in the context of facial palsy but can occur in isolation, and more specific headache syndromes including trigeminal and geniculate neuralgias can occur rarely. Signs and symptoms indicative of vestibulocochlear nerve involvement are relatively common, although it could be that these represent other vestibular involvement rather than a specific cranial neuropathy. Optic neuritis is a controversial entity within Lyme disease and is likely overdiagnosed, but convincing cases do exist. Physicians who see any cranial neuropathy, including optic neuritis, in an endemic area can consider Lyme disease as a possible cause.

[An analysis of the etiology composition and clinical characteristics of bilateral optic disc swelling].

Objective: To investigate the etiology composition and clinical characteristics of bilateral optic disc swelling(ODS). Methods: The medical records of all newly diagnosed bilateral ODS patients admitted to the neurology ward of Beijing Tongren Hospital from January 2017 to June 2021 were retrospectively searched to classify the etiology, obtain demographic and clinical information, and compare the differences in clinical characteristics. Results: A total of 131 patients with bilateral ODS were included, including 56 males and 75 females, aged 15-73 (39±14) years. The most common cause of the bilateral ODS was increased intracranial pressure (ICP)(56/131, 42.7%), followed by optic neuritis (ON)(40/131, 30.5%). Other causes included vascular optic neuropathy (13/131, 9.9%), pseudopilledema (9/131, 6.9%); uveitis (6/131, 4.6%), toxic optic neuropathy (3/131, 2.3%) and unknown causes (4/131, 3.1%). Idiopathic intracranial hypertension (IIH) (43/56, 76.8%) was the most common etiology for papilledema. In the comparison of the two main causes of intracranial hypertension and the clinical characteristics of ON in ODS, there was no statistically significant difference in the incidence of age, gender, complaints of ocular pain or headache, and hemorrhage of optic disc(P>0.05). Visual acuity abnormalities and low vision were more common in ON group than the increased ICP group[36/40(90%) vs 33/56(58.9%), P=0.001; 35/80(43.8%) vs 22/112(19.6%), P<0.001], while severe papilledema was more common in increased ICP group[38/112(33.9%)vs 9/80(11.3%), P<0.001]. Conclusions: The most common cause for bilateral ODS is increased ICP, but it can also be triggered by a variety of other causes. Optic neuritis(ON) is the most important differentiating disease in the study of Chinese patients.

Longitudinal Retinal Changes in MOGAD.

OBJECTIVE: Patients with myelin oligodendrocyte glycoprotein antibody (MOG-IgG)-associated disease (MOGAD) suffer from severe optic neuritis (ON) leading to retinal neuro-axonal loss, which can be quantified by optical coherence tomography (OCT). We assessed whether ON-independent retinal atrophy can be detected in MOGAD. METHODS: Eighty patients with MOGAD and 139 healthy controls (HCs) were included. OCT data was acquired with (1) Spectralis spectral domain OCT (MOGAD: N = 66 and HCs: N = 103) and (2) Cirrus high-definition OCT (MOGAD: N = 14 and HCs: N = 36). Macular combined ganglion cell and inner plexiform layer (GCIPL) and peripapillary retinal nerve fiber layer (pRNFL) were quantified. RESULTS: At baseline, GCIPL and pRNFL were lower in MOGAD eyes with a history of ON (MOGAD-ON) compared with MOGAD eyes without a history of ON (MOGAD-NON) and HCs (p < 0.001). MOGAD-NON eyes had lower GCIPL volume compared to HCs (p < 0.001) in the Spectralis, but not in the Cirrus cohort. Longitudinally (follow-up up to 3 years), MOGAD-ON with ON within the last 6-12 months before baseline exhibited greater pRNFL thinning than MOGAD-ON with an ON greater than 12 months ago (p < 0.001). The overall MOGAD cohort did not exhibit faster GCIPL thinning compared with the HC cohort. INTERPRETATION: Our study suggests the absence of attack-independent retinal damage in patients with MOGAD. Yet, ongoing neuroaxonal damage or edema resolution seems to occur for up to 12 months after ON, which is longer than what has been reported with other ON forms. These findings support that the pathomechanisms underlying optic nerve involvement and the evolution of OCT retinal changes after ON is distinct in patients with MOGAD. ANN NEUROL 2022;92:476-485.

Co-occurrence of polyneuritis crainials and visual impairment: a case report and literature review.

BACKGROUND: Polyneuritis cranialis (PNC) with the disease characteristics of Guillain-Barré syndrome (GBS) in addition to both ocular and bulbar weakness in the absence of limb paralysis or ataxia is defined as an unusual variant of GBS. As evidence of central nervous system (CNS) involvement, visual impairment is an unusual finding complicating with GBS spectrum disorders and has never been reported in patients with PNC. METHODS: We describe a very rare case who clinically presented with progressive multiple cranial nerve palsy and visual impairment. Furthermore, a literature search of concurrent GBS and optic neuritis (ON) as well as PNC attributed to GBS was conducted. RESULTS: A diagnosis of PNC was considered due to the typical clinical characteristics as well as the presence of cerebrospinal fluid cytoalbumin dissociation and serum antibodies against gangliosides. The clinical manifestations and the bilateral optic nerve involvement in brain magnetic resonance imaging further suggested possible optic neuritis (ON). The patient received treatment with intravenous immunoglobulin followed by short-term use of corticosteroids and finally achieved a full recovery. Thirty-two previously reported cases (17 women, mean age 40) of concurrent GBS and ON and 20 cases of PNC (5 women, mean age 40) were analyzed. We further provided a comprehensive discussion on the potential etiologies, clinical features, therapeutic strategies, and prognosis. CONCLUSIONS: This rare case with the co-occurrence of PNC and visual impairment and the related literature review may help clinicians advance the understanding of GBS spectrum disorders and make appropriate diagnoses and treatment decisions for the rare variants and CNS complications of GBS.

MRI Signal Intensity Varies Along the Course of the Normal Optic Nerve.

BACKGROUND: MRI can help distinguish various causes of optic neuropathy including optic neuritis. Importantly, neuromyelitis optica spectrum disorder (NMOSD) has a propensity to cause enhancement of the prechiasmatic optic nerves. To determine whether the prechiasmatic optic nerve (PC-ON) demonstrates a different intensity from the midorbital optic nerve (MO-ON) on MRI among patients without optic neuropathy. METHODS: Data were retrospectively obtained from 75 patients who underwent brain MRI for an ocular motor nerve palsy between January 2005 and April 2021. Inclusion criteria were patients aged 18 years or older with visual acuities of at least 20/25 and no evidence of optic neuropathy on neuro-ophthalmic examination. A total of 67 right eyes and 68 left eyes were assessed. A neuroradiologist performed quantitative intensity measurements of the MO-ON and PC-ON on precontrast and postcontrast T1 axial images. Normal-appearing temporalis muscle intensity was also measured and used as a reference to calculate an intensity ratio to calibrate across images. RESULTS: The mean PC-ON intensity ratio was significantly higher than the MO-ON intensity ratio on both precontrast (19.6%, P < 0.01) and postcontrast images (14.2%, P < 0.01). Age, gender, and laterality did not independently affect measurements. CONCLUSIONS: The prechiasmatic optic nerve shows brighter intensity ratios on both precontrast and postcontrast T1 images than the midorbital optic nerve among normal optic nerves. Clinicians should recognize this subtle signal discrepancy when assessing patients with presumed optic neuropathy.

Can volumetric analysis of the brain help diagnose isolated optic neuritis?

Isolated optic neuritis is a single episode inflammatory optic neuropathy. This condition, which affects the optimal function of the optic nerve, is not associated with neurological or systemic diseases. Our study aimed to compare patients with isolated optic neuritis and normal healthy individuals in terms of the cerebrum, cerebellum and hippocampus volumes by using the "volBrain Online MRI Brain Volumetry System" program. Persons diagnosed with isolated optic neuritis (n = 16) and persons without any disease (n = 16) were included in the study. VolBrain was used to process the MRI data and, the findings were compared with Mann-Whitney U test. Values with a p-value <0.05 were considered statistically significant. The cerebrum white matter volumes in the total brain and in the right-left hemispheres of the brain were statistically significantly lower in the optic neuritis group (p = 0.029; p = 0.050; p = 0.029, respectively). In the segmental cerebellum analysis, the left side lobule VIIIB, the total and right-left side lobule IX volumes were statistically significantly higher (p = 0.022; p = 0.014; p = 0.029; p = 0.018, respectively). In total, lobule I-II volume was statistically significantly lower in the optic neuritis group (p = 0.046). In the segmental hippocampus analysis, the right side CA2-CA3, the total and right-left side SR-SL-SM volumes were statistically significantly lower in the optic neuritis group (p = 0.039; p = 0.050; p = 0.016, respectively). There are neurodegenerative changes in brain volume in patients with isolated optic neuritis. Although volBrain alone is not sufficient to diagnose isolated optic neuritis, it provides quantitative data that can be used as a complementary diagnostic method.

SUBCLINICAL OPTIC NEURITIS.

Acute optic neuritis is often associated with multiple sclerosis. It is considered to be the most common ocular symptom of multiple sclerosis. In addition to acute optic neuritis, in patients with multiple sclerosis, subclinical optic neuritis is also described. It is characterized by slow progression and bilateral involvement, thus being unnoticed by the patient. The purpose of the present study was to assess vision impairment in multiple sclerosis patients without a history of acute optic neuritis, using a number of functional tests including visual field testing by Octopus 101 perimetry N1 program, contrast sensitivity testing by Pelli Robson chart, and color vision by Ishihara pseudoisochromatic plates. The study included 35 multiple sclerosis patients aged 18-50 years, without subjective signs of vision impairment and visual acuity 1.0 according to Snellen. Visual field defects were found in 28 patients. The most common defects of visual fields were retinal sensitivity depression in peripheral zone and nerve fiber bundle defect. Reduced contrast sensitivity was found in 30 (86%) patients. Study results indicated multiple sclerosis patients free from signs of optic neuritis to suffer vision function impairment, as demonstrated by Octopus perimetry and contrast sensitivity testing with Pelli Robson charts.

Anatomical and functional visual network patterns in progressive multiple sclerosis.

The gradual accrual of disability over time in progressive multiple sclerosis is believed to be driven by widespread degeneration. Yet another facet of the problem may reside in the loss of the brain's ability to adapt to the damage incurred as the disease progresses. In this study, we attempted to examine whether changes associated with optic neuritis in the structural and functional visual networks can still be discerned in progressive patients even years after the acute insult. Forty-eight progressive multiple sclerosis patients, 21 with and 27 without prior optic neuritis, underwent structural and functional MRI, including DTI and resting state fMRI. Anatomical and functional visual networks were analyzed using graph theory-based methods. While no functional metrics were significantly different between the two groups, anatomical global efficiency and density were significantly lower in the optic neuritis group, despite no significant difference in lesion load between the groups. We conclude that long-standing distal damage to the optic nerve causes trans-synaptic effects and the early ability of the cortex to adapt may be altered, or possibly nullified. We suggest that this limited ability of the brain to compensate should be considered when attempting to explain the accumulation of disability in progressive multiple sclerosis patients.

An overview of peripapillary hyperreflective ovoid mass-like structures.

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the ophthalmic findings associated with peripapillary hyperreflective ovoid mass-like structures (PHOMS) in both adult and pediatric patients. RECENT FINDINGS: PHOMS have recently been identified in a number of different ophthalmic disease entities ranging from nonpathologic to pathologic, including but not limited to anatomic abnormalities (tilting in myopia), optic nerve head drusen, optic disc edema from inflammation (optic neuritis, white dot syndromes), vascular insults (ischemic optic neuropathy, retinal vascular occlusion), and papilledema. The mechanism underlying the formation of PHOMS has not been fully elucidated although it has been hypothesized that PHOMS occur secondary to axoplasmic stasis from crowding at the optic nerve head. SUMMARY: Although the clinical significance of the presence of PHOMS remains unclear, PHOMS are associated with several disease processes. Understanding the mechanism behind their formation and their impact on optic nerve head structure and visual function may be relevant in patients with optic nerve head pathology. The presence of PHOMS may also correlate with disease severity and duration. Future studies to evaluate whether the formation of PHOMS may be useful as an early indicator of disease or a prognostic tool are warranted.

Approach to the diagnosis and management of nutritional optic neuropathies.

PURPOSE OF REVIEW: Nutritional deficiency is an under-recognized cause of optic neuropathy. The purpose of this review is to discuss how to identify, diagnose, and appropriately manage patients with nutritional optic neuropathy. RECENT FINDINGS: Nutritional deficiencies have long been thought to be more prevalent in the developing countries. However, with the advent of bariatric surgery, restrictive/selective diets, and the increase in alcohol dependence, it is not uncommon to see nutritional optic neuropathies in the developed world. SUMMARY: Although nutritional optic neuropathy can cause severe and debilitating vision loss, it is often reversible when it is diagnosed and treated in a timely manner.

Visual Function and Brief Cognitive Assessment for Multiple Sclerosis in Optic Neuritis Clinically Isolated Syndrome Patients.

BACKGROUND: In this study, we hypothesized that clinically isolated syndrome-optic neuritis patients may have disturbances in neuropsychological functions related to visual processes. METHODS: Forty-two patients with optic neuritis within 3 months from onset and 13 healthy controls were assessed at baseline and 6 months with MRI (brain volumes, lesion load, and optic radiation lesion volume) and optical coherence tomography (OCT) (peripapillary retinal nerve fiber layer [RNFL], ganglion cell and inner plexiform layers [GCIPLs], and inner nuclear layer). Patients underwent the brief cognitive assessment for multiple sclerosis, high-contrast and low-contrast letter acuity, and color vision. RESULTS: At baseline, patients had impaired visual function, had GCIPL thinning in both eyes, and performed below the normative average in the visual-related tests: Symbol Digit Modalities Test and Brief Visuospatial Memory Test-Revised (BVMT-R). Over time, improvement in visual function in the affected eye was predicted by baseline GCIPL (P = 0.015), RNFL decreased, and the BVMT-R improved (P = 0.001). Improvement in BVMT-R was associated with improvement in the high-contrast letter acuity of the affected eye (P = 0.03), independently of OCT and MRI metrics. CONCLUSION: Cognitive testing, assessed binocularly, of visuospatial processing is affected after unilateral optic neuritis and improves over time with visual recovery. This is not related to structural markers of the visual or central nervous system.

Optical Coherence Tomography Is Associated With Cognitive Impairment in Multiple Sclerosis.

BACKGROUND: Optical coherence tomography (OCT) is a sensitive method for quantifying retinal neuronal and axonal structures. Reductions in retinal nerve fiber layer (RNFL) and ganglion cell inner plexiform layer (GCIPL) thicknesses have a reported association with white and grey matter atrophy in multiple sclerosis (MS). We hypothesized that the thinning of intraretinal layer measurements associates with cognitive decline in MS patients with no prior event of optic neuritis (ON). METHODS: OCT and NeuroTrax computerized cognitive assessments were performed in 204 relapsing remitting MS patients with no history of ON or other conditions affecting the eye. Data were collected between 2010 and 2020 and retrospectively analyzed. Correlations were examined between cognitive performance and a lower RNFL or GCIPL thickness. A multilinear regression model was generated to assess the significance of these correlations regarding the disability score and disease duration. RESULTS: The 204 study participants had a mean age of 40.52 ± 11.8 years (mean ± SD) and disease duration of 9.80 ± 9.40 years. The mean RNFL thickness in this whole cohort was 82.22 ± 10.85 µm and the global cognitive score was 95.32 ± 12.32. The mean GCIPL thickness measured in a subgroup of 104 patients was 74.27 ± 10.37 µm. The RNFL and GCIPL both correlated with the global cognitive score (r = 0.174, P = 0.013 and r = 0.29, P = 0.03, respectively), and with various cognitive domains. However, the GCIPL showed stronger correlations than RNFL, particularly with executive function (r = 0.29, P = 0.003), attention (r = 0.332, P = 0.001), and the information processing speed (r = 0.25, P = 0.012). These correlations remained significant after correcting for confounders. CONCLUSION: OCT measurements correlate with cognitive performance in MS patients. OCT can thus be used to evaluate central nervous system neurodegeneration in MS, as reflected by cognitive decline.

Optic Perineuritis Presenting as the Initial Manifestation of Central Nervous System Involvement in Rai Stage 0 Chronic Lymphocytic Leukemia.

BACKGROUND: To describe the first case of optic perineuritis because of meningeal involvement of early stage chronic lymphocytic leukemia (CLL). METHODS: A case report and review of the literature. RESULTS: A case of unilateral optic neuropathy associated with enhancement of the optic nerve sheath is described in a patient with a prior 2-year history of Rai Stage 0 CLL. Lumbar puncture revealed a lymphocytic pleocytosis. Cerebrospinal fluid flow cytometry revealed a monoclonal expansion of CD5+ B cells compatible with CLL, matching the flow cytometry characteristics of his peripheral blood. CONCLUSIONS: Optic perineuritis is often initially diagnosed as optic neuritis, yet the 2 have different etiologies and follow a different clinical course. Orbital MRI with contrast structurally separates the 2, revealing a characteristic pattern of peripheral optic nerve sheath rather than primary optic nerve enhancement. Etiologies of optic perineuritis are varied and include inflammatory, infectious, neoplastic, and toxic entities. Central nervous system (CNS) involvement by chronic lymphocytic leukemia is unusual, but cranial nerve and meningeal involvement have been reported. This case adds central nervous system chronic lymphocytic leukemia to the list of differential diagnostic possibilities for optic perineuritis. It also alerts clinicians to consider optic perineuritis as a potential presenting feature of CNS involvement in otherwise asymptomatic and stable CLL.

No Light Perception Vision in Neuro-Ophthalmology Practice.

BACKGROUND: To determine differential diagnosis and visual outcomes of patients with no light perception (NLP) vision related to neuro-ophthalmic conditions. METHODS: Retrospective case series of patients seen at tertiary neuro-ophthalmology practices. Patients were included if they had NLP vision any time during their clinical course. Outcome measures were final diagnosis, treatment, and visual outcome. RESULTS: Seventy-two eyes of 65 patients were included. The average age was 57.6 (range 18-93) years, and 58% were women. The Most common diagnosis (21 patients) was compressive optic neuropathy (CON) with meningioma being the most common culprit (12). Other diagnoses included optic neuritis (ON) (11 patients), infiltrative optic neuropathies (8), posterior ischemic optic neuropathy (7), nonarteritic anterior ischemic optic neuropathy (4), arteritic anterior ischemic optic neuropathy (3), ophthalmic artery occlusion (3), nonorganic vision loss (3), radiation-induced optic neuropathy (2), cortical vision loss (1), retinitis pigmentosa with optic disc drusen (1), and infectious optic neuropathy (1). Ten patients recovered vision: 7 ON, 2 infiltrative optic neuropathy, and 1 CON. Corticosteroids accelerated vision recovery in 7 of the 11 patients with ON to mean 20/60 (0.48 logMAR) over 9.0 ± 8.6 follow-up months. Eleven patients deteriorated to NLP after presenting with at least LP; their diagnoses included CON (3), ophthalmic artery occlusion (2), infiltration (2), ON (1), posterior ischemic optic neuropathy (1), arteritic anterior ischemic optic neuropathy (1), and radiation-induced optic neuropathy (1). CONCLUSIONS: NLP vision may occur because of various diagnoses. Vision recovery was mainly seen in patients with ON. Serious systemic conditions may present or relapse with NLP vision, which clinicians should consider as an alarming sign in patients with known malignancies.

ABCB1 gene polymorphisms impact the effect of high-dose intravenous methylprednisolone therapy on optic neuritis associated with AQP4-IgG-positive neuromyelitis optica spectrum disorder.

WHAT IS KNOWN AND OBJECTIVE: Patients with optic neuritis (ON) have significant individual differences in their response to high-dose intravenous methylprednisolone (HIMP) therapy. This study aims to evaluate the association between gene polymorphisms and the efficacy of HIMP therapy in Chinese Han patients with ON mediated by aquaporin-4 immunoglobulin G antibody (AQP4-IgG) -positive neuromyelitis optica spectrum disorder (NMOSD) or multiple sclerosis (MS). METHODS: Chinese Han patients with AQP4-IgG+ NMOSD-ON or MS-ON were genotyped for four candidate genes: ABCB1 (rs1045642, rs1128503, rs2032582), NR3C1 (rs41423247), TBX21 (rs9910408, rs16947078) and VDR (rs731236, rs1544410, rs7975232, rs2228570). Patients were divided into glucocorticoid resistance (GR) and glucocorticoid sensitivity (GS) groups based on vision acuity (VA) improvement after HIMP treatment. Intergroup comparisons were performed on clinical characteristics, allele and genotype frequencies and haplotype distributions. RESULTS: A total of 267 patients completed the follow-up, including 120 patients with AQP4-IgG+ NMOSD-ON and 147 patients with MS-ON. We observed a significant association between the ABCB1 G2677T/A (rs2032582) polymorphism and glucocorticoid response in AQP4-IgG+ NMOSD-ON patients. Changes in VA scores in patients with the GG genotype were significantly lower than those in patients with the T/A T/A genotype (1.07 ± 1.20 vs. 1.77 ± 1.31, p = 0.026). In the GS group, the G allele had a lower frequency than the T/A allele (32.03% vs. 60.16%, p = 0.001). Logistic regression analysis showed that the G2677T/A GG and G T/A genotypes could increase the GR risk 3.53 and 2.67 times compared with the T/A T/A genotype, respectively (OR = 3.534, 95% CI: 1.186-10.527, p = 0.023; OR = 2.675, 95% CI: 1.005-7.123, p = 0.049). In addition, haplotype analysis showed that AQP4-IgG+ NMOSD-ON patients with the TAT/TTT haplotype (ABCB1 C3435T-G2677T/A-C1236T) were only 0.54 times more likely to develop GR than those with other haplotypes (OR = 0.542, 95% CI: 0.315-0.932, p = 0.026). However, we did not observe intergroup differences in the MS-ON population. WHAT IS NEW AND CONCLUSION: Our findings suggest that the G > T/A polymorphism of ABCB1 G2677T/A and the TAT/TTT haplotype played a protective role in HIMP treatment of AQP4-IgG+ NMOSD-ON but not MS-ON.

Sarcoidosis presenting as bilateral optic neuritis after ChAdOx1 nCoV-19 vaccination.

Sarcoidosis is an idiopathic granulomatous disease and can virtually affect any organ system. Multiple factors, including tubercular antigens organic and environmental exposures, have been implicated in its pathogenesis. In addition to drugs, sarcoid-like reactions have been reported following varicella and influenza vaccination. Few reports of erythema nodosum and Lofgren syndrome have been reported after the COVID19 vaccination, though no histologic diagnosis was pursued in these cases. We herein report a case of sarcoidosis presenting with bilateral acute onset vision loss with a temporal association with COVID19 vaccination (ChadOx-1 n-COV, COVISHIELDTM). Symptoms started within two weeks of receiving the vaccine. Alternate causes for optic neuritis were excluded. Transbronchial lung biopsy showed the presence of non-caseating epithelioid cell granulomas. The patient received high-dose corticosteroids immediately after diagnosis, albeit with incomplete clinical improvement in vision on a three-month follow-up. In conclusion, we report a novel case of sarcoidosis-related optic neuritis following COVID19 vaccination.

Retinal imaging with optical coherence tomography in multiple sclerosis: novel aspects.

Optical coherence tomography (OCT) is of increasing interest in the clinical assessment of multiple sclerosis (MS) patients beyond the scope of clinical studies. In this narrative review, we discuss novel changes of OCT parameters during acute optic neuritis and the disease course of MS patients. OCT images document the changes of retinal layers during an episode of acute optic neuritis and can therefore provide valuable insights into the pathophysiology. Moreover, MS patients show progredient thinning of retinal layers throughout the disease. The thinning is accelerated through relapses as well as disease progression without relapse. The OCT parameters are also associated with clinical outcome parameters, including disability, cognitive function, and brain atrophy. The impact of disease-modifying therapies on OCT parameters is the subject of ongoing research and depends on the agent used. Additional data are still necessary before OCT parameters can be implemented in the clinical standard of care of MS patients.

Inner and outer retina findings determined by optical coherence tomography in different subtypes of multiple sclerosis.

Background: Integrity of outer retinal bands among multiple sclerosis (MS) subtypes remains unclear, however alterations of thickness in retinal layers is well described. Aim: The objective of the study was to determine the alterations in the thickness of the inner and outer layers of the retina and the findings in both layers detected by optical coherence tomography (OCT) among patients with relapsing-remitting multiple sclerosis (RRMS) and secondary progressive multiple sclerosis (SPMS). Patients and Methods: A total of 132 eyes from 66 patients with multiple sclerosis (MS) (70 eyes from 35 patients with RRMS and 62 eyes from 31 patients with SPMS) and 72 eyes from 36 healthy controls were included in the study. The external structures of the retina, including the outer limiting membrane (ELM), ellipsoid zone (EZ), and interdigitation zone (IZ), were examined using OCT in RRMS, SPMS, and healthy control groups. The correlation of neurological disability expressed by the Expanded Disability Study Scale (EDSS) score, best-corrected visual acuity, and duration of disease among OCT parameters was also analyzed. Results: In eyes, with no history of previous optic neuritis (ON), the macular nerve fiber layer, the internal plexiform layer of ganglion cells (GCIPL), and the total thickness of the retinal layer were thinner in the SPMS group than in the RRMS group (P < 0.05, in each comparison). EZ was more vulnerable among the three hyperreflective external retinal zones in the retina of patients with SPMS than in patients with RRMS (P = 0.016). Conclusions: Alterations in retinal thickness in MS are not limited to the inner layers of the retina and also occur in the outer structures of the retina.

Age-dependent favorable visual recovery despite significant retinal atrophy in pediatric MOGAD: how much retina do you really need to see well?

BACKGROUND: To investigate age-related severity, patterns of retinal structural damage, and functional visual recovery in pediatric and adult cohorts of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) optic neuritis (ON). METHODS: All MOGAD patients from the 5 participating centers were included. Patients with initial manifestation <18 years were included in the pediatric (MOGADped) cohort and patients with ≥18 years in the adult (MOGADadult) cohort. For patients with MOGAD ON, examinations at least ≥6 months after ON onset were included in the analyses. Using spectral domain optical coherence tomography (SD-OCT), we acquired peripapillary retinal nerve fiber layer thickness (pRNFL) and volumes of combined ganglion cell and inner plexiform layer (GCIPL). High- and 2.5% low-contrast visual acuity (HCVA, LCVA) and visual-evoked potentials (VEP) were obtained. RESULTS: Twenty MOGADped (10.3±3.7 years, 30 MOGAD ON eyes) and 39 MOGADadult (34.9±11.6 years, 42 MOGAD ON eyes) patients were included. The average number of ON episodes per ON eye was similar in both groups (1.8±1.3 and 2.0±1.7). In both pediatric and adult MOGAD, ON led to pronounced neuroaxonal retinal atrophy (pRNFL: 63.1±18.7 and 64.3±22.9 µm; GCIPL: 0.42±0.09 and 0.44±0.13 mm3, respectively) and moderate delay of the VEP latencies (117.9±10.7 and 118.0±14.5 ms). In contrast, visual acuity was substantially better in children (HCVA: 51.4±9.3 vs. 35.0±20.6 raw letters, p=0.001; LCVA: 22.8±14.6 vs. 13.5±16.4, p=0.028). Complete visual recovery (HCVA-logMAR 0.0) occurred in 73.3% of MOGADped and 31% MOGADadults ON eyes, while 3.3% and 31% demonstrated moderate to severe (logMAR > 0.5) visual impairment. Independent of retinal atrophy, age at ON onset significantly correlated with visual outcome. CONCLUSION: Pediatric MOGAD ON showed better visual recovery than adult MOGAD ON despite profound and almost identical neuroaxonal retinal atrophy. Age-related cortical neuroplasticity may account for the substantial discrepancy between structural changes and functional outcomes.