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To describe the frequency and characteristics of intraretinal and subretinal fluid in nonarteritic anterior ischemic optic neuropathy (NAAION) and to assess the influence on the visual deficit and optic nerve fiber/ganglion cell loss. A retrospective, single-center study. Thirty-two patients with NAAION referred to our Neuro-ophthalmology Department between 2014 and 2021. The study was carried out at the University Hospital of Liège, Belgium. For participants in whom subretinal fluid was identified on standard OCT (Carl Zeiss Meditec) an additional macular OCT (Spectralis Heidelberg) had been performed. The pattern and the maximal height of the retinal fluid were determined manually, and thicknesses of retinal layers were obtained using the OCT protocol analysis. The mean age of the cohort was 60 years (standard deviation, ±12.5; range, 22–88 years), and 65.6% were male. In the 21 eyes (46.7%) in which retinal fluid was observed, macular OCT findings were categorized according to fluid localization: 19 cases had parafoveal fluid (of whom 9 also had subfoveal fluid). One patient had subfoveal fluid alone, and 1 patient had peripapillary subretinal fluid alone. Specific patterns of optic disc (OD) swelling were associated with the occurrence and distribution of retinal edema. Visual acuity, visual field loss, and foveal thresholds were stable over the period of observation (P = 0.74, P = 0.42, and P = 0.36, respectively). No difference was found in visual function at 6 months between patients with retinal fluid treated (n = 10) or not treated (n = 11) with corticosteroids (visual acuity, P = 0.13; foveal threshold, P = 0.59; mean deviation, P = 0.66). Subretinal fluid is found in a high proportion of cases of NAAION. Visual function remained largely stable from presentation in this cohort. Corticosteroid intake at presentation did not influence visual recovery or timing of the resorption of tissue edema. Our findings do not support treatment of NAAION with corticosteroids with or without evidence of subretinal fluid acutely. With regard to pathogenesis, we propose that the volume of transudate generated at the OD is the critical factor rather than dysfunction of retinal mechanisms subserving reabsorption. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article. [ABSTRACT FROM AUTHOR]

Abstract: The spectrum of autoimmune optic neuropathies (ON) is extending. The phenotypic spectrum includes single isolated optic neuritis (SION), relapsing isolated optic neuritis (RION), chronic relapsing inflammatory optic neuropathy (CRION), the neuromyelitis optica (NMO) spectrum disorder, multiple sclerosis associated optic neuritis (MSON) and unclassified optic neuritis (UCON) forms. Epidemiological data suggests a slight female predominance. The ethnic heritage is relevant as Caucasian patients are more likely to suffer from MSON, whilst SION, RION, CRION and NMO are more frequent in non-Caucasian patients. Importantly, prognosis for recovery of visual function is good in MSON, but poorer in NMO and CRION which also have a high chance for recurrent episodes. Testing for serum anti-AQP4 autoantibodies is advised in all patients with severe, atypical or recurrent ON because of the high diagnostic specificity. The diagnostic specificity may be aided by testing for glial biomarkers in the CSF and prognostic accuracy by testing for biomarkers for neuroaxonal degeneration. Optical coherence tomography is a highly accurate tool to document the final outcome. The current clinical classification criteria rely on the phenotype, response to treatment and presence of anti-AQP4 autoantibodies. [Copyright &y& Elsevier]

Optic neuritis is an inflammatory optic neuropathy that affects many patients with multiple sclerosis (MS) at some point during their disease course. Differentiation of acute episodes of MS-associated optic neuritis from other autoimmune and inflammatory optic neuropathies is vital for treatment choice and further patient management, but is not always straightforward. Over the past decade, a number of new imaging, laboratory and electrophysiological techniques have entered the clinical arena. To date, however, no consensus guidelines have been devised to specify how and when these techniques can be most rationally applied for the diagnostic work-up of patients with acute optic neuritis. In this article, we review the literature and attempt to formulate a consensus for the investigation of patients with acute optic neuritis, both in standard care and in research with relevance to clinical treatment trials. [ABSTRACT FROM AUTHOR]

Bilateral optic neuropathy in Dar es Salaam is now considered endemic and is estimated to affect 0.3–2.4% of young adults. The condition is characterized by a subacute bilateral loss of central vision of unknown aetiology. Findings of spectral domain optical coherence tomography have not previously been reported for these patients. All patients diagnosed with endemic optic neuropathy over a 2-year period at the Muhimbili National Hospital underwent spectral domain optical coherence tomography macular imaging. Scans were graded qualitatively for severity of retinal nerve fibre layer loss as well as the presence of microcystic macular changes, which have not previously been described in this condition. Of the 128 patients included (54.7% male; median age 20 years), severe retinal nerve fibre layer loss was found in 185 eyes (74.0%). There was full concordance in retinal nerve fibre layer thickness between the two eyes in 113 (91.1%) patients. Microcystic macular spaces were found in 16 (12.5%) patients and were bilateral in nine (7.0%) individuals. These changes were typically more prominent in the nasal than the temporal macula, predominantly involving the inner nuclear layer, and often occurred in an annular configuration that was evident on en face infra-red imaging, though not discernible on colour fundus photography or clinically. All patients with microcystic macular changes had severe thinning of the retinal nerve fibre layer (P = 0.02). Four patients in whom cystic spaces were demonstrated had sequential scans, and there was no detectable alteration in the configuration of these changes over a period of up to 16 months. This is the first study to document optical coherence tomography findings in endemic optic neuropathy. We have observed symmetrical severe loss of the caeco-central projection (papillomacular bundle) with otherwise well-preserved macular architecture. Also, we have observed microcystic retinal changes in a significant proportion of patients, which were associated with severe retinal nerve fibre layer loss. Similar changes have recently been reported from optical coherence tomography images of patients with multiple sclerosis, relapsing isolated optic neuritis, dominant optic atrophy, Leber’s hereditary optic neuropathy and a patient with a chronic compressive optic neuropathy, supporting the hypothesis that this may be a non-specific phenomenon secondary to ganglion cell death. The correspondence of the changes to an annulus discernible on infra-red en face imaging, but not using other conventional retinal imaging techniques highlights the potential usefulness of this modality. [ABSTRACT FROM PUBLISHER]

Abstract: Background: Complement mediated autoimmunity against aquaporin-4 results in astrocytic damage in neuromyelitis optica (NMO). There is evidence for increased CSF glial fibrillary acidic protein (GFAP) and S100B levels in acute NMO. Here we tested whether the CSF finding also holds true for the diagnostic value of serum GFAP and S100B levels in NMO. Methods: A multicentre study included 322 patients from London (n=160), Nijmegen (n=95), Pecs (n=44), and Lyon (n=24). Patients were classified into the following diagnostic categories: neurological control patients (n=45), MS optic neuritis (MSON, n=38), isolated optic neuritis (ION, n=11), relapsing isolated optic neuritis (RION, n=48), chronic relapsing isolated optic neuropathy (CRION, n=18), unclassified optic neuritis (UCON, n=39), NMO (n=77) and relapsing remitting multiple sclerosis (RRMS, n=47). Serum GFAP and S100B levels were quantified using ELISA. Results: Median serum GFAP but not S100B levels were significantly higher (p<0.0001, general linear model) in patients with NMO (4.83pg/mL) if compared to MSON (1.5pg/mL, p=0.0001), UCON (1.92pg/mL, p<0.01), ION (0.0ng/mL, p<0.05), RION (1.3pg/mL, p<0.0001) and CRION (2.2pg/mL, p=0.01). Serum GFAP levels in the control cohort (3.6pg/mL) were not significantly different to NMO. There was no relationship between serum GFAP levels and any other clinical or demographic parameter. Serum S100B concentrations correlated with the number of relapses in MSON (R=0.83, p=0.005). Conclusion: In contrast to the CSF, neither serum GFAP nor S100B levels were of major diagnostic value for the laboratory supported differential diagnosis between optic neuritis in the context of NMO and other optic neuropathies. [Copyright &y& Elsevier]

Background Non-arteritic anterior ischaemic optic neuropathy (NA-AION) can cause disabling visual loss and traditionally, visual prognosis has been considered poor, although recent studies have demonstrated improvements in visual acuity in about 30% of patients over time. The aim of the study was to determine whether there was significant cortical reorganisation with functional MRI (fMRI) after acute NA-AION by comparing affected individuals with healthy controls. Methods 9 patients with NA-AION were studied acutely and then after 1, 2, 3 and 6 months. 23 healthy volunteers underwent scanning at least twice. At each time point, patients were assessed clinically and with fMRI. For the fMRI experiments, subjects underwent monocular visual stimulation (wearing goggles with flashing LED displays). Results When stimulating the affected eye, occipital activation was reduced in patients compared with controls. Also, within the NA-AION group, activation in the right Brodmann areas (BA) 44 and 45 was seen during the early phase of the condition. The same areas were activated within the NA-AION group several months later for fellow eye stimulation. When the NA-AION and healthy control groups were formally compared however, these areas (BA 44/45) were not significantly different. NA-AION subjects did show greater activation in visual related areas compared with controls when stimulating the fellow eye. Visual acuity was correlated with more occipital cortex activation when stimulating the affected eye. Conclusions There is cortical re-organisation of the fMRI response in extra-visual areas, seen when both affected and fellow eyes are stimulated after NA-AION. [ABSTRACT FROM AUTHOR]

Three patients on haemodialysis presented with acute painless visual loss. From each, a comprehensive medical and ophthalmic history was taken and each underwent a complete ophthalmic examination. Visual evaluation was carried out by recording Snellen visual acuities, Ishihara colour test scores, the presence of a relative afferent pupillary defect (RAPD) and visual fields with either Goldmann or Humphrey perimetry. Review of renal case notes was performed and appropriate laboratory investigations including imaging were carried out to investigate the cause of disc swelling in all cases. All cases presented with acute painless visual loss, a RAPD, disc swelling, impaired colour vision and a visual field defect. All cases had a history of post-dialysis hypotension. In 2 cases, post-dialysis hypotension was documented just prior to the development of anterior ischaemic optic neuropathy (AION) suggesting a causal relationship. Falls in mean arterial pressure (MAP) of 31 and 22 mmHg were recorded. None of the cases had other well documented risk factors for AION such as diabetes or hypercholesterolaemia. One case had crowded discs. Two cases had bilateral involvement. Two cases were treated with corticosteroids and 1 with aspirin. Visual improvement was noted in 1 case coincident with corticosteroid treatment. Common risk factors in all 3 cases were anaemia and post-dialysis hypotension. In conclusion, patients undergoing haemodialysis may be at risk of AION particularly if they are anaemic and develop post-dialysis hypotension. They should be informed of a small and unpredictable risk of visual loss. [ABSTRACT FROM AUTHOR]