The presenting visual symptoms of optic chiasmal disease.

Recognising optic chiasmal disease early is important in order to avoid irreversible visual loss and the potential risk of mortality for patients. Yet, there is frequently a delay in the initial diagnosis. Whilst the signs of optic chiasmal disease, particularly the perimetric findings, are well documented in the recent literature, the symptoms have been less well reported. Whilst some patients with optic chiasmal disease will be asymptomatic, many will complain of visual symptoms including symptomatic field defects, problems with central vision, difficulty with near tasks, binocular visual disturbances, colour vision disturbances, photophobia, phosphenes, glare, and rarely, oscillopsia and visual hallucinations. Others may have headache or the severe and sudden visual symptoms associated with pituitary apoplexy. The visual symptoms may be vague or non-specific, even when there are significant bitemporal visual field defects. We aim in this review to describe the presenting visual symptoms of optic chiasmal disease, and to illustrate these with selected qualitative descriptions from the literature. Our hope is that this will aid clinicians in eliciting a careful history of the sometimes subtle symptoms that may be present.

Association of Amount of Weight Lost After Bariatric Surgery With Intracranial Pressure in Women With Idiopathic Intracranial Hypertension.

BACKGROUND AND OBJECTIVES: The idiopathic intracranial hypertension randomized controlled weight trial (IIH:WT) established that weight loss through bariatric surgery significantly reduced intracranial pressure when compared with a community weight management intervention. This substudy aimed to evaluate the amount of weight loss required to reduce intracranial pressure and to explore the effect of different bariatric surgical approaches. METHODS: IIH:WT was a multicenter randomized controlled trial. Adult women with active idiopathic intracranial hypertension and a body mass index ≥35 kg/m2 were randomized to bariatric surgery or a community weight management intervention (1:1). This per-protocol analysis evaluated the relationship between intracranial pressure, weight loss, and the weight loss methods. A linear hierarchical regression model was used to fit the trial outcomes, adjusted for time, treatment arm, and weight. RESULTS: Sixty-six women were included, of whom 23 had received bariatric surgery by 12 months; the mean age was 31 (SD 8.7) years in the bariatric surgery group and 33.2 (SD 7.4) years in the dietary group. Baseline weight and intracranial pressure were similar in both groups with a mean weight of 119.5 (SD 24.1) and 117.9 (SD 19.5) kg and mean lumbar puncture opening pressure of 34.4 (SD 6.3) and 34.9 (SD 5.3) cmCSF in the bariatric surgery and dietary groups, respectively. Weight loss was significantly associated with reduction in intracranial pressure (R2 = 0.4734, p ≤ 0.0001). Twenty-four percentage of weight loss (weight loss of 13.3 kg [SD 1.76]) was associated with disease remission (intracranial pressure [ICP] ≤ 25 cmCSF). Roux-en-Y gastric bypass achieved greater, more rapid, and sustained ICP reduction compared with other methods. DISCUSSION: The greater the weight loss, the greater the reduction in ICP was documented. Twenty four percentage of weight loss was associated with disease remission. Such magnitude of weight loss was unlikely to be achieved without bariatric surgery, and hence, consideration of referral to a bariatric surgery program early for those with active idiopathic intracranial hypertension may be appropriate. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02124486; ISRCTN registry number ISRCTN40152829; doi.org/10.1186/ISRCTN40152829. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that weight loss after bariatric surgery results in reduction in intracranial pressure in adult women with idiopathic intracranial hypertension. This study is Class II because of the use of a per-protocol analysis.

Update on Optic Neuritis: An International View.

Previously, optic neuritis was thought to be typical, i.e. idiopathic or multiple sclerosis (MS) related, associated with a good visual prognosis, or atypical, i.e. not associated with MS and requiring corticosteroids or plasma exchange for vision to recover. More recently, the importance of optic neuritis in neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein (MOG) antibody disease has become more appreciated. The results of the Optic Neuritis Treatment Trial (ONTT) has influenced how optic neuritis is treated around the world. For this review we surveyed the international literature on optic neuritis in adults. Our aims were first to find the reported incidence of optic neuritis in different countries and to ascertain what percentage of cases were seropositive for anti-aquaporin 4 and anti-MOG antibodies, and second, to document the presenting features, treatment, and outcomes from a first episode of the different types of optic neuritis from these countries, and to compare the results with the outcomes of the ONTT cohort. From these data we have sought to highlight where ambiguities currently lie in how to manage optic neuritis and have made recommendations as to how future treatment trials in optic neuritis should be carried out in the current antibody testing era.

Dr Gordon Plant's Festschrift Tidings.

We came together in March 2021 for a joyous celebration of Dr Gordon Plant's incredible career. This is a report of the meeting tidings.

Paraneoplastic Syndromes in Neuro-Ophthalmology.

Paraneoplastic neurological syndromes (PNS) occur in about 1 in 300 cases of cancer. The usual mechanism is that an antigen on the cancer sets off an immune response that then cross-reacts with a nervous system antigen. The presentation is usually with a subacute progressive neurological disorder. The management of these conditions is usually of both the underlying tumor and immunomodulation to suppress the autoimmune response. There are a number of these conditions that can present to the Neuro-Ophthalmology clinic, either as a discrete condition affecting vision or eye movements or as part of a more widespread neurological disorder. This article will discuss these conditions, their management and prognosis.

Neuro-Ophthalmic Complications of Vestibular Schwannoma Resection: Current Perspectives.

Vestibular schwannomas (VSs), also called acoustic neuromas, are benign intracranial neoplasms of the vestibulocochlear (VIII) cranial nerve. Management options include "wait-and-scan," stereotactic radiosurgery and surgical resection. Due to the proximity of the VIII nerve to the facial (VII) nerve in the cerebello-pontine angle, the VII nerve is particularly vulnerable to the effects of surgical resection. This can result in poor eye closure, lagophthalmos and resultant corneal exposure post VS resection. Additionally, compression from the tumor or resection can cause trigeminal (V) nerve damage and a desensate cornea. The combination of an exposed and desensate cornea puts the eye at risk of serious ocular complications including persistent epithelial defects, corneal ulceration, corneal vascularization, corneal melting and potential perforation. The abducens (VI) nerve can be affected by a large intracranial VS causing raised intracranial pressure (a false localizing sign) or as a result of damage to the VI nerve at the time of resection. Other types of neurogenic strabismus are rare and typically transient. Contralaterally beating nystagmus as a consequence of vestibular dysfunction is common post-operatively. This generally settles to pre-operative levels as central compensation occurs. Ipsilaterally beating nystagmus post-operatively should prompt investigation for post-operative cerebrovascular complications. Papilledema (and subsequent optic atrophy) can occur as a result of a large VS causing raised intracranial pressure. Where papilledema follows surgical resection of a VS, it can indicate that cerebral venous sinus thrombosis has occurred. Poor visual function following VS resection can result as a combination of all these potential complications and is more likely with larger tumors.

Effectiveness of Bariatric Surgery vs Community Weight Management Intervention for the Treatment of Idiopathic Intracranial Hypertension: A Randomized Clinical Trial.

Importance: Idiopathic intracranial hypertension (IIH) causes headaches, vision loss, and reduced quality of life. Sustained weight loss among patients with IIH is necessary to modify the disease and prevent relapse. Objective: To compare the effectiveness of bariatric surgery with that of a community weight management (CWM) intervention for the treatment of patients with active IIH. Design, Setting, and Participants: This 5-year randomized clinical trial (Idiopathic Intracranial Hypertension Weight Trial) enrolled women with active IIH and a body mass index (calculated as weight in kilograms divided by height in meters squared) of 35 or higher at 5 National Health Service hospitals in the UK between March 1, 2014, and May 25, 2017. Of 74 women assessed for eligibility, 6 did not meet study criteria and 2 declined to participate; 66 women were randomized. Data were analyzed from November 1, 2018, to May 14, 2020. Interventions: Bariatric surgery (n = 33) or CWM intervention (Weight Watchers) (n = 33). Main Outcomes and Measures: The primary outcome was change in intracranial pressure measured by lumbar puncture opening pressure at 12 months, as assessed in an intention-to-treat analysis. Secondary outcomes included lumbar puncture opening pressure at 24 months as well as visual acuity, contrast sensitivity, perimetric mean deviation, and quality of life (measured by the 36-item Short Form Health Survey) at 12 and 24 months. Because the difference in continuous outcomes between groups is presented, the null effect was at 0. Results: Of the 66 female participants (mean [SD] age, 32.0 [7.8] years), 64 (97.0%) remained in the clinical trial at 12 months and 54 women (81.8%) were included in the primary outcome analysis. Intracranial pressure was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -6.0 [1.8] cm cerebrospinal fluid [CSF]; 95% CI, -9.5 to -2.4 cm CSF; P = .001) and at 24 months (adjusted mean [SE] difference, -8.2 [2.0] cm CSF; 95% CI, -12.2 to -4.2 cm CSF; P < .001) compared with the CWM arm. In the per protocol analysis, intracranial pressure was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -7.2 [1.8] cm CSF; 95% CI, -10.6 to -3.7 cm CSF; P < .001) and at 24 months (adjusted mean [SE] difference, -8.7 [2.0] cm CSF; 95% CI, -12.7 to -4.8 cm CSF; P < .001). Weight was significantly lower in the bariatric surgery arm at 12 months (adjusted mean [SE] difference, -21.4 [5.4] kg; 95% CI, -32.1 to -10.7 kg; P < .001) and at 24 months (adjusted mean [SE] difference, -26.6 [5.6] kg; 95% CI, -37.5 to -15.7 kg; P < .001). Quality of life was significantly improved at 12 months (adjusted mean [SE] difference, 7.3 [3.6]; 95% CI, 0.2-14.4; P = .04) and 24 months (adjusted mean [SE] difference, 10.4 [3.8]; 95% CI, 3.0-17.9; P = .006) in the bariatric surgery arm. Conclusions and Relevance: In this randomized clinical trial, bariatric surgery was superior to a CWM intervention in lowering intracranial pressure. The continued improvement over the course of 2 years shows the impact of this intervention with regard to sustained disease remission. Trial Registration: ClinicalTrials.gov Identifier: NCT02124486.

Is a virtual clinic model a safe and effective way for assessing patients referred with suspiciously blurred optic discs? The blurred disc clinic.

BACKGROUND: There are increasing numbers of referrals to ophthalmology departments due to blurred optic disc margins. In light of this and the COVID-19 pandemic we aimed to assess whether these patients could be safely assessed without direct contact between the clinician and patient. METHODS: We retrospectively reviewed the records of consecutive patients seen in our 'blurred disc clinic' between August 2018 and October 2019. We then presented anonymous information from their referral letter, their visual fields and optic nerve images to two consultant neuro-ophthalmologists blinded to the outcome of the face-to-face consultation. In the simulated virtual clinic, the two consultants were asked to choose an outcome for each patient from discharge, investigate or bring in for a face-to-face assessment. RESULTS: Out of 133 patients seen in the blurred disc clinic, six (4.5%) were found to have papilloedema. All six were identified by both neuro-ophthalmologists as needing a face-to-face clinic consultation from the simulated virtual clinic. One hundred and twenty (90%) patients were discharged from the face-to-face clinic at the first consultation. The two neuro-ophthalmologists chose to discharge 114 (95%) and 99 (83%) of these respectively from the simulated virtual clinic. The virtual clinic would have potentially missed serious pathology in only one patient who had normal optic discs but reported diplopia at the previous face-to-face consultation. CONCLUSIONS: A virtual clinic model is an effective way of screening for papilloedema in patients referred to the eye clinic with suspicious optic discs. Unrelated or incidental pathology may be missed in a virtual clinic.

The Utility of Fundus Fluorescein Angiography in Neuro-Ophthalmology.

While its use is still widespread within the medical retina field, fundus fluorescein angiography (FFA) is increasingly falling out of favour in the investigation of neuro-ophthalmological disease, with the introduction of new technologies, particularly optical coherence tomography. FFA does, however, provide useful diagnostic and prognostic information in many neuro-ophthalmological diseases including papilloedema, pseudo-papilloedema, optic neuropathies and central retinal artery occlusion to name a few. We aim to summarise the main FFA findings in each of these conditions and highlight where FFA is of most use in providing complementary information to other modes of investigation.

Do Optic Canal Dimensions Measured on CT Influence the Degree of Papilloedema and Visual Dysfunction in Idiopathic Intracranial Hypertension?

A recent study found that increased optic canal area on magnetic resonance imaging was associated with worse papilloedema in idiopathic intracranial hypertension (IIH). We repeated this study using more accurate computerized tomography derived measurements. Optic canal dimensions were measured from 42 IIH patients and 24 controls.  These were compared with papilloedema grade.  There was no correlation between any of the optic canal measurements and papilloedema grade and no significant difference in optic canal measurements between patients and controls. Our results cast doubt on the existing literature regarding the association between optic canal size and the degree of papilloedema in IIH. CT delineates bony anatomy more accurately than MRI and our CT-derived optic canal measurements cast doubt on the existing literature regarding the association between optic canal size and the degree of Papilloedema in IIH.

Treatment and Outcomes in Nutritional Optic Neuropathy.

PURPOSE OF REVIEW: Nutritional optic neuropathy is a potential cause of severe visual loss; however, appropriate and timely management can result in excellent visual outcomes. The purpose of this review is to outline our current understanding of the treatment and outcomes for nutritional optic neuropathy. RECENT FINDINGS: Current understanding of nutritional optic neuropathy has been greatly aided by some well-reported and investigated epidemics of the condition, most notably the Cuban epidemic optic neuropathy of the early 1990s. More recently, there is an emerging literature surrounding nutritional deficiencies that can occur in patients who have undergone bariatric surgery. There also continues to be a stream of case reports in the literature that add to our understanding. Nutritional optic neuropathy has a great deal of overlap with toxic optic neuropathies and hereditary optic neuropathies and should not be thought of in isolation from these conditions. The mainstay of treatment for nutritional optic neuropathy involves identifying and replacing deficient nutrients as well as identifying and eliminating contributory toxins. It is also important to identify contributory genetic factors and to consider the broader social, economic and societal factors which may contribute.

A Stereotyped Syndrome with Retro-Ocular Pain, Photophobia, and Visual Disturbance Masquerading as Optic Neuritis: Case Series.

We describe here a case series of six patients referred to the Neuro-ophthalmology service in Sheffield, UK with possible acute unilateral optic neuritis. Each patient had a triad of unilateral photophobia, ipsilateral retro-ocular pain, and ipsilateral loss of vision. All patients had normal ocular examinations and investigation findings with no objective structural or functional abnormalities identified. Patients were treated by weaning-off regular analgesia and, where appropriate, commencing migraine prophylaxis. In the three patients with complete recovery of pain, there was also complete recovery of vision. We propose that this is a migraine syndrome and that the decreased visual acuity is a functional consequence of the pain and photophobia.

Idiopathic Acquired Temporal Wedge Visual Field Defects.

Our aim is to report 13 unusual cases of acquired, temporal sectoral scotomas. Such stationary "wedge" field defects have been reported previously in cases of presumed congenital nasal hypoplasia of the optic disc and as a complication of vitreoretinal surgery. To our knowledge, the literature provides no reports of similar defects occurring spontaneously. This is a descriptive clinical case series of 13 patients presenting with sub-acute monocular temporal visual field loss. All were clinically assessed and investigated with Goldmann perimetry, automated Humphrey visual fields, retinal optical coherence tomography, orbital ultrasound, and standard and multi-focal electroretinography. Cases were followed with serial perimetry for a mean of 3.9 years (range: 6 months to 10 years). Goldmann and Humphrey perimetry both demonstrated "wedge"-shaped defects extending temporally from an apex contiguous with, or lateral to, the blind spot. There was no evidence of optic disc drusen, glaucoma, disc hypoplasia, or focal retinitis. Sectoral optic disc swelling was not present in any patient at presentation. In all cases, the visual field defect remained stable. One patient developed a similar defect in the fellow eye after an interval of 5 years. Here we describe 13 cases of acquired, stationary temporal wedge scotomas, novel in the literature. Although the aetiology is uncertain, we propose damage to the nasal rim of the optic disc as a likely mechanism.

Phenytoin for neuroprotection in patients with acute optic neuritis: a randomised, placebo-controlled, phase 2 trial.

BACKGROUND: Acute demyelinating optic neuritis, a common feature of multiple sclerosis, can damage vision through neurodegeneration in the optic nerve and in its fibres in the retina. Inhibition of voltage-gated sodium channels is neuroprotective in preclinical models. In this study we aimed to establish whether sodium-channel inhibition with phenytoin is neuroprotective in patient with acute optic neuritis. METHODS: We did a randomised, placebo-controlled, double-blind phase 2 trial at two UK academic hospitals in London and Sheffield. Patients with acute optic neuritis aged 18-60 years, presenting within 2 weeks of onset, with visual acuity of 6/9 or worse, were randomly assigned (1:1) by minimisation via a web-based service to oral phenytoin (maintenance dose 4 mg/kg per day if randomised before or on July 16, 2013, and 6 mg/kg per day if randomised on or after July 17, 2013) or placebo for 3 months, stratified by time from onset, centre, previous multiple sclerosis diagnosis, use of disease-modifying treatment, and use of corticosteroids for acute optic neuritis. Participants and treating and assessing physicians were masked to group assignment. The primary outcome was retinal nerve fibre layer (RNFL) thickness in the affected eye at 6 months, adjusted for fellow-eye RNFL thickness at baseline, analysed in a modified intention-to-treat population of all randomised participants who were followed up at 6 months. Safety was analysed in the entire population, including those who were lost to follow-up. The trial is registered with ClinicalTrials.gov, number NCT 01451593. FINDINGS: We recruited 86 participants between Feb 3, 2012, and May 22, 2014 (42 assigned to phenytoin and 44 to placebo). 29 were assigned to phenytoin 4 mg/kg and 13 to phenytoin 6 mg/kg. Five participants were lost to follow-up, so the primary analysis included 81 participants (39 assigned to phenytoin and 42 to placebo). Mean 6-month RNFL thickness in the affected eye at 6 months was 81.46 µm (SD 16.27) in the phenytoin group (a mean decrease of 16.69 µm [SD 13.73] from baseline) versus 74.29 µm (15.14) in the placebo group (a mean decrease of 23.79 µm [13.97] since baseline; adjusted 6-month difference of 7.15 µm [95% CI 1.08-13.22]; p=0.021), corresponding to a 30% reduction in the extent of RNFL loss with phenytoin compared with placebo. Treatment was well tolerated, with five (12%) of 42 patients having a serious adverse event in the phenytoin group (only one, severe rash, was attributable to phenytoin) compared with two (5%) of 44 in the placebo group. INTERPRETATION: These findings support the concept of neuroprotection with phenytoin in patients with acute optic neuritis at concentrations at which it blocks voltage-gated sodium channels selectively. Further investigation in larger clinical trials in optic neuritis and in relapsing multiple sclerosis is warranted. FUNDING: US National Multiple Sclerosis Society, Multiple Sclerosis Society of Great Britain and Northern Ireland, Novartis, UK National Institute for Health Research (NIHR), and NIHR UCLH/UCL Biomedical Research Centre.

Optic Perineuritis.

Optic perineuritis (OPN) is now defined as "a form of idiopathic orbital inflammatory disease, in which the specific target tissue is the optic nerve sheath". It may be idiopathic or may occur as part of an underlying systemic inflammatory disease. It is a rare disorder and information regarding its presentation and management is only available in case reports and small case series. This review will discuss the contribution of these recent articles to what is known about idiopathic and secondary OPN. Suggestions will also be made as to how to investigate and treat a patient presenting with OPN.

An Evaluation of Educational Neurological Eye Movement Disorder Videos Posted on Internet Video Sharing Sites.

BACKGROUND: Internet video sharing sites allow the free dissemination of educational material. This study investigated the quality and educational content of videos of eye movement disorders posted on such sites. METHODS: Educational neurological eye movement videos were identified by entering the titles of the eye movement abnormality into the search boxes of the video sharing sites. Also, suggested links were followed from each video. The number of views, likes, and dislikes for each video were recorded. The videos were then rated for their picture and sound quality. Their educational value was assessed according to whether the video included a description of the eye movement abnormality, the anatomical location of the lesion (if appropriate), and the underlying diagnosis. RESULTS: Three hundred fifty-four of these videos were found on YouTube and Vimeo. There was a mean of 6,443 views per video (range, 1-195,957). One hundred nineteen (33.6%) had no form of commentary about the eye movement disorder shown apart from the title. Forty-seven (13.3%) contained errors in the title or in the text. Eighty (22.6%) had excellent educational value by describing the eye movement abnormality, the anatomical location of the lesion, and the underlying diagnosis. Of these, 30 also had good picture and sound quality. The videos with excellent educational value had a mean of 9.84 "likes" per video compared with 2.37 for those videos without a commentary (P < 0.001). The videos that combined excellent educational value with good picture and sound quality had a mean of 10.23 "likes" per video (P = 0.004 vs videos with no commentary). There was no significant difference in the mean number of "dislikes" between those videos that had no commentary or which contained errors and those with excellent educational value. CONCLUSIONS: There are a large number of eye movement videos freely available on these sites; however, due to the lack of peer review, a significant number have poor educational value due to having no commentary or containing errors. The number of "likes" can help to identify videos with excellent educational value but the number of "dislikes" does not help in discerning which videos have poor educational value.

Unilateral compressive optic neuropathy due to skull hyperostosis secondary to nutritional vitamin A deficiency.

We report a 17-year-old boy who presented with a chronic left unilateral optic neuropathy. Computerized tomography and magnetic resonance imaging demonstrated compression of the left optic nerve due to skull hyperostosis. He was found to be profoundly vitamin A deficient secondary to an unusual diet consisting predominantly of potato chips and crisps. Skull hyperostosis with cranial neuropathies and other neurological abnormalities has been described in growing animals fed vitamin A deficient diets but has not been previously reported in humans.