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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]

Abstract: Visual aura is a common presenting symptom of migraine to both neurologists and ophthalmologists. Features such as photopsia, fortification spectra, and the slow propagation of a scintillating scotoma across the visual field are usually considered diagnostic features of the visual aura of migraine. In the vast majority of cases, the diagnosis can be made without the need for further investigations. We present 9 patients and a further 31 cases from the literature who experienced visual aura fulfilling the diagnostic criteria for migraines but caused by focal occipital pathology. Key clinical features that could help to differentiate between the visual aura of migraine and those suggestive of a structural lesion are outlined. We review current scientific theories into the pathophysiology of visual aura, drawing on clinical and basic science research, including human functional imaging studies of migraine aura and advances in the genetic characterization of familial channelopathies, in order to explain the overlap which occurs in the clinical features of visual aura associated with migraine, cortical lesions, and epilepsy. We conclude that any disease process that is able to create a state of neuronal hyperexcitability can therefore increase an individual’s susceptibility to the development of cortical spreading depression, the electrophysiological correlate of the visual aura. [Copyright &y& Elsevier]

Purpose: To report abnormalities of retinal nerve fiber layer (RNFL) thickness using optical coherence tomography (OCT) in patients with trans-synaptic degeneration. Design: Observational case reports. Methods: Two patients with congenital/long standing occipital lobe lesions who had asymptomatic homonymous hemianopic field defects were examined using OCT. Results: In case 1, there was loss of RNFL in the projection of the nasal hemifovea temporally and the horizontal projection nasally in the right eye and thinning in the superior and inferior temporal arcades in the left eye corresponding to visual field abnormalities. In case 2, abnormalities in the RNFL pattern corresponded to the visual defects even though the field defect was incomplete. Conclusion: OCT measurements of the RNFL provide useful information in the diagnosis of congenital/long standing occipital lobe lesions. [Copyright &y& Elsevier]

Purpose: To describe 2 adult patients who presented with papilledema after band atrophy (i.e., twin and triple peaks papilledema). Design: Retrospective small case series. Participants: Two outpatients. Methods: Observations made on 2 patients whose cases were reviewed in the neuro-ophthalmology clinic. Results: The first patient had a pituitary tumor presenting with papilledema, causing a triple peaks clinical sign. Color photographs, optical coherence tomograms, and magnetic resonance images are shown. The second patient developed twin peaks papilledema due to a chiasmal glioma causing secondary raised intracranial pressure. Conclusion: Twin peaks papilledema is a rare clinical sign that may develop in adults as well as in children. The first report and optical coherence tomography features of triple peaks papilledema illustrate a new clinical sign. [Copyright &y& Elsevier]

Abstract: Patients with homonymous hemianopia may report the completion of forms that overlap the vertical meridian of their field defects. While previous investigations of “hemianopic completion” have variously attributed to the disorder to inattention, residual vision or unstable fixation, we believe that our investigation has controlled for such potentially confounding factors. We report patient P.O.V. who experienced hemianopic completion in everyday life following a surgical lesion of his left occipital lobe. He showed normal spatial attention and normal spatial orienting: hemianopic completion can therefore occur in the absence of inattention. His completion was retinotopic and affected partial as well as complete forms: his hemianopic completion cannot be attributed to residual visual input or poor fixation. P.O.V.''s completion was also systematically affected by varying stimulus contrast and pattern masking. We argue that while other explanations may be appropriate for different cases, P.O.V.''s hemianopic completion reflects normal “constructive” visual processes and can be attributed to the unconstrained operation of visual routines that are normally involved in the perception of partially occluded forms. As such, this disorder has the potential to shed light on some of the most basic aspects of visual perception. [Copyright &y& Elsevier]

A recent hypothesis to enter the literature suggests that glaucoma is a neurodegenerative disease. The basis for this has been the finding of central nervous system changes in glaucoma patients on histology and neuroimaging. It is known that retinal ganglion cell pathology of any cause leads to anterograde and retrograde retinal ganglion cell degeneration, as well as trans-synaptic (transneuronal) anterograde degeneration. Trans-synaptic degeneration has been demonstrated in a range of optic neuropathies including optic nerve transection, optic neuritis, and hereditary optic neuropathies. More recently, similar changes have been confirmed in glaucoma patients using the neuroimaging techniques of voxel-based morphometry and diffusion tensor imaging. Some studies have reported brain changes in glaucoma outside the retino-geniculo-cortical pathway; however, these are preliminary and exploratory in nature. Further research is required to identify whether the degenerative brain changes in glaucoma are entirely secondary to the optic neuropathy or whether there is additional primary central nervous system pathology. This has critical implications for neuroprotective and regenerative treatment strategies and our basic understanding of glaucoma. [ABSTRACT FROM AUTHOR]

Purpose Enhanced depth imaging (EDI) spectral-domain optical coherence tomography (SD OCT) has been recognized as the most sensitive tool to diagnose optic nerve head drusen (ONHD). The relationship between OCT characteristics and visual loss has not been well documented. This study compares EDI SD OCT–determined morphologic characteristics of drusen in eyes with or without visual field (VF) defects. Design Descriptive study of patients attending the neuro-ophthalmology service of Moorfields Eye Hospital between January 2013 and October 2014. Subjects Patients with diagnosed ONHD and EDI SD OCT imaging of the optic nerve head. Methods Eyes with and without VF defects were compared with regard to retinal nerve fiber layer (RNFL) thickness, drusen morphology, size, extent, visibility on funduscopy, ultrasound, and fundus autofluorescence. Main Outcome Measures Difference in OCT characteristics of ONHD between patients with or without VF defects. Results Of 38 patients, 69 eyes with ONHD were included. Thirty-three eyes had a normal VF with average mean deviation (MD) −0.96 (±1.2) dB and pattern standard deviation (PSD) 1.6 (±0.3) dB (group I), and 36 eyes had VF defects with MD −13.7 (±10.4) dB and PSD 7.2 (±3.6) dB (group II). Mean global RNFL thickness was 62 (±20.9) μm in the latter group and 99.0 (±12.9) μm in group I. In group I, the predominant drusen type was peripapillary drusen, of variable size. In group II, most eyes had confluent ( P < 0.02) and large (>500 μm; P < 0.003) drusen, and drusen were more commonly visible on funduscopy ( P = 0.001), ultrasound ( P = 0.013), and autofluorescence ( P = 0.002). Differences between the 2 groups reached statistical significance in a clustered analysis. RNFL thinning and autofluorescence showed relative sparing of the temporal sector. Sixty-four percent of patients with a VF defect in 1 eye also had a VF defect in their fellow eye. Conclusions Drusen size and drusen type as classified by OCT morphologic characteristics are significantly different in patients with or without VF defects. Confluent, large, and autofluorescent drusen were more commonly found in patients with VF defects. These findings may assist in clarifying how drusen give rise to visual loss, which is currently not known. [ABSTRACT FROM AUTHOR]

A 30-year-old woman presented with intermittent photopsia, a temporal visual field defect below the horizontal in her left eye, and flu-like symptoms. Slit-lamp and fundus examinations were unremarkable. Humphrey 30-2 threshold perimetry and 120-point screening visual field demonstrated blind spot enlargement of the left eye and a normal field in the right eye. Fundus autofluorescence, optical coherence tomography of the macula, full-field electroretinogram, electrooculogram, and multifocal electroretinogram were normal. Swept-source optical coherence tomography scan of the left optic nerve showed an intact outer retina, a remarkably thinned nerve fiber layer nasally, and peripapillary vitreous traction. Goldmann kinetic perimetry revealed a sector-shaped dense defect breaking out from the blind spot to the temporal periphery just below the horizontal in the left eye. The patient had nasal hypoplasia of the optic nerve and peripapillary vitreous traction. [ABSTRACT FROM AUTHOR]

Embolic disease of the anterior or posterior vascular territories may lead to disturbance of vision. Although death from this is uncommon, morbidity remains relatively high: Visual field loss may impair or preclude reading and driving and these are important influences on quality of life. Visual symptoms of stroke mean that patients may present to ophthalmologists with isolated visual symptoms, rather than directly to an emergency department. It is important to diagnose stroke and transient ischemic attacks accurately, as well as to manage them appropriately, as they are important harbingers of further cerebrovascular events. Ophthalmologists are therefore well placed to ensure that these patients receive appropriate acute treatment and secondary prevention. This article reviews the evidence for managing patients presenting with visual symptoms of vascular events. It reviews management of ischemic stroke in general, and compares this with management of events involving the anterior circulation by way of transient monocular visual loss or retinal artery occlusion, and posterior circulation by way of transient binocular visual loss or infarction of the visual cortex. [ABSTRACT FROM AUTHOR]

Abstract: Transient monocular blindness and amaurosis fugax are umbrella terms describing a range of patterns of transient monocular visual field loss (TMVL). The incidence rises from ≈1.5/100,000 in the third decade of life to ≈32/100,000 in the seventh decade of life. We review the vascular supply of the retina that provides an anatomical basis for the types of TMVL and discuss the importance of collaterals between the external and internal carotid artery territories and related blood flow phenomena. Next, we address the semiology of TMVL, focusing on onset, pattern, trigger factors, duration, recovery, frequency-associated features such as headaches, and on tests that help with the important differential between embolic and non-embolic etiologies [Copyright &y& Elsevier]

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]

Abstract: Purpose: To investigate the presence of an ethnicity bias within patients presenting with optic neuritis in London. Design: Observational cross-sectional study. Methods: The ethnicity profile of all patients attending a neuro-ophthalmology clinic in central London with acute optic neuritis over a 16month period (n =86) was studied. A comparison was made with the ethnicity profile of the population of London as well as patients with Multiple Sclerosis-associated optic neuritis (n =41), Neuromyelitis Optica spectrum disorder-associated optic neuritis (n =27) and patients with an atypical corticosteroid-dependent optic neuropathy (21). Results: The ethnicity profile of the patient cohort presenting to our clinic with acute optic neuritis over a 16month period closely matched the ethnicity profile of London (P =0.08). Within this cohort, patients of African or African-Caribbean heritage were found to be more likely to manifest either a pattern or aetiology of optic neuritis requiring immunosuppressive treatment in comparison with patients of a white Caucasian background (relative risk 3.47; 95% CI=1.092 to 11.007). There was a disproportionately high representation of patients from an African or African-Caribbean background within the Neuromyelitis Optica spectrum-related optic neuritis diagnostic group (P <0.00). Conclusions: Patients with acute isolated optic neuritis from African or African Caribbean backgrounds are over 3 times more likely than patients of white Caucasian backgrounds to have an ‘atypical’ pattern of optic neuritis where corticosteroid therapy may be required. Our results suggest that a patient''s ethnic background is an important factor to be taken into consideration when deciding on the diagnosis and management of acute isolated optic neuritis. [Copyright &y& Elsevier]

Abstract: Myasthenia gravis (MG) is an autoimmune disorder where patients develop autoantibodies towards skeletal muscle proteins (e.g. acetylcholine receptor and muscle specific kinase), causing weakness in striated muscles. Ocular MG (OMG) represents a subtype of (MG) affecting only the periocular muscles. The pathogenesis of this phenotype remains unclear. Heat Shock Protein 70 (Hsp70) plays a role in immune regulation. Antibodies against this protein are associated with several autoimmune diseases, and its biological significance has been shown in vivo. We have therefore examined the concentration of anti-Hsp70 antibodies in sera from 35 OMG patients and 94 patients with generalized MG (GMG) using ELISA assays. The antibody concentrations were compared to those in patients with multiple sclerosis (MS), Guillain-Barré syndrome (GBS) and to healthy controls. MG patients had significantly higher anti-Hsp70 antibody concentrations than both MS patients and healthy controls. GBS patients had higher antibody levels than all other groups. No difference in antibody levels was found when comparing OMG and GMG. Our results suggest that patients with MG and GBS have a previous or current increased exposure to Hsp70 antigens. The similarity between GMG and OMG strengthens the hypothesis that OMG represents a systemic disease, similar to GMG. [ABSTRACT FROM AUTHOR]

Background: The 2001 and 2005 McDonald criteria allow MRI evidence for dissemination in space (DIS) and dissemination in time (DIT) to be used to diagnose multiple sclerosis in patients who present with clinically isolated syndromes (CIS). In 2006, new criteria were proposed in which DIS requires at least one T2 lesion in at least two of four locations (juxtacortical, periventricular, infratentorial, and spinal-cord) and DIT requires a new T2 lesion on a follow-up scan. We applied all three criteria in a large cohort of CIS patients to assess their performance by use of conversion to clinically definite multiple sclerosis (CDMS) as the outcome.Methods: Patients who had two MRI scans within 12 months of CIS onset were identified in four centres in the Magnims European research network. The specificity and sensitivity of MRI criteria for CDMS after 3 years was assessed in 208 patients. A Cox proportional hazards model was applied in a larger cohort of 282 patients that included all patients irrespective of length of follow-up.Findings: The specificity of all criteria for CDMS was high (2001 McDonald, 91%; 2005 McDonald, 88%; new, 87%). Sensitivity of the new (72%) and 2005 McDonald (60%) criteria were higher than the 2001 McDonald criteria (47%). The Cox proportional hazards model showed a higher conversion risk for all three criteria in those with both DIS and DIT than those with either DIS or DIT alone. When all three criteria were included in the model, only the new criteria had an independent significant effect on conversion risk.Interpretation: The new criteria are simpler than the McDonald criteria without compromising specificity and accuracy. The presence of both DIS and DIT from two MRI scans has a higher specificity and risk for CDMS than either DIS or DIT alone. [ABSTRACT FROM AUTHOR]

Background: Capillary hemangioma in the cranial cavity is rare. This report describes 2 additional cases presenting shortly after pregnancy and provides a systematic review summarizing clinical experience to date. Methods: Case reports were compiled retrospectively. Patient 1 was a 28-year-old woman who presented with a simple partial seizure associated with left-sided visual distortions that progressed to a secondary generalized tonic-clonic seizure. Imaging revealed a contrast-enhancing lesion in the right temporal region adjacent to the transverse sinus, with vasogenic edema and scalloping of the inner table. The angiographic appearance suggested the correct diagnosis. Patient 2 was a 41-year-old woman who presented with progressive visual disturbance 6 months after giving birth to her second child. Subtle symptoms of headache and visual disturbance had commenced during pregnancy. Noncontrasted imaging displayed a homogenous tumor with surrounding vasogenic edema in the occipital region. PubMed and Science Citation Index were reviewed systematically for prior publications. Results: Complete surgical excision was performed in both patients. Neuropathological examination confirmed benign capillary hemangioma consisting of a meshwork of capillary-sized and larger feeder vessels in both instances. Systematic review was based on 20 publications and a total of 24 patients; their clinical, radiological, and surgical features as well as management are summarized. Conclusions: At follow-up, symptoms had resolved and magnetic resonance imaging confirmed complete removal in both patients. Intracranial capillary hemangioma is rare, and treatment is empirical. Review of limited published reports suggests that surgery is the most commonly used treatment and that complete excision seems most likely to prevent further recurrence. [ABSTRACT FROM AUTHOR]