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Stephanie J Chiu,1,2 Simon J Hickman,2,3 Irene M Pepper,1,2 Jennifer HY Tan,1,2 John Yianni,2,4 Joanna M Jefferis1,2,5 1Department of Ophthalmology, Royal Hallamshire Hospital, Sheffield, UK; 2Department of Medical Education, University of Sheffield, Sheffield, UK; 3Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK; 4Department of Neurosurgery, Royal Hallamshire Hospital, Sheffield, UK; 5Department of Neuro-Ophthalmology, Manchester Royal Eye Hospital, Manchester, UKCorrespondence: Joanna M JefferisManchester Royal Eye Hospital, Oxford Road, Manchester, M13 9WL, UKTel +44 0161 276 1234Email jojefferis@doctors.org.ukAbstract: 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.Keywords: vestibular schwannoma, post-operative complications, papilledema, facial nerve paralysis, neurotrophic keratopathy, nystagmus

Acute promyelocytic leukemia (APML) can lead to pseudotumour cerebri (PTC), either due to direct effects of the disease or as a complication of its treatment with the vitamin A derivative all-trans-retinoic acid or arsenic. We report the case of a patient with sight-threatening papilledema secondary to APML-induced PTC who was treated with bilateral optic nerve sheath fenestration. [ABSTRACT FROM AUTHOR]

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.

Background/aims: Optic nerve sheath fenestration (ONSF) is a surgical intervention in the management of idiopathic intracranial hypertension (IIH) infrequently performed in the United Kingdom. Numerous surgical approaches have been described, including medial transconjunctival, lateral and endoscopic. We describe our outcomes and complications from ONSF via a supero-medial eyelid skin crease incision in patients with IIH., Methods: We performed a retrospective review of consecutive patients undergoing ONSF for IIH between January 2011 and December 2017 by a single surgeon., Results: Thirty patients were included in the analysis with a median follow-up of 14.5 months. Bilateral ONSFs were undertaken in 27 (90%). The data from one eye per patient were analysed. The mean kinetic perimetry score in mean radial degrees of the I4e isopter improved from 27.3° to 35.7°, p = 0.04. After removing cases with optic atrophy, the median modified Frisén grade of papilloedema improved from 2.5 to 1.0, p = 0.007. A total of 5/30 (17%) patients had complications: two (7%) had recurrence/late failure (one managed medically and one with cerebrospinal fluid [CSF] diversion surgery), one had transient cotton wool spots post-operatively, one had transient retinal haemorrhages and one patient had a transiently oval pupil. No patients had repeat ONSF, but CSF diversion surgery was subsequently carried out in 4/30 (13%) patients., Conclusions: ONSF via a supero-medial eyelid skin crease approach is effective at improving visual function in patients with IIH. The complication rates are low when compared with CSF diversion surgery and other surgical approaches for ONSF.

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.

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.

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.

Sarcoidosis can affect the optic nerves by means of optic disc oedema secondary to posterior uveitis, optic disc oedema secondary to raised intracranial pressure, optic neuritis, optic atrophy secondary to compression or infiltration from a primary central nervous system lesion, and primary granuloma of the optic nerve head. The authors report the use of optical coherence tomography in assessing the response to immunosuppression in a 57-year-old woman with an optic nerve head granuloma.

A retrospective notes review was conducted for 50 consecutive patients who underwent shunt surgery for idiopathic intracranial hypertension (IIH). The decimal visual acuity and the mean radial degrees (MRD) of the I4e isopter of the Goldmann visual field were measured pre-operatively and after a mean follow-up period of 1123 days (range: 13-3551 days). A ventriculo-peritoneal shunt was the first procedure in 38 patients and a lumbo-peritoneal shunt in 12. The mean decimal visual acuity of the worse affected eye improved from 0.75 to 0.84, p  = 0.011. The MRD score of the worse affected eye improved on average from 25.6° to 35.5°, p  < 0.0001. In those with significant pre-operative visual impairment in their worse affected eye (defined as an MRD score ≤30°), the MRD score improved on average from 10.3° to 26.5°, p  = 0.0008. The mean number of surgical procedures for each patient was 2.8 (range: 1-15). Taking all surgical procedures into account, post-operative complications were experienced by 30 patients. At last follow-up, 28 patients still complained of headache, 8 of whom had the intervention performed primarily for headache. Shunting can improve visual function in patients with IIH. There is significant post-operative morbidity and often the need for repeated procedures. Headache also commonly remains in these patients. There is a need for a randomised controlled trial of operative interventions in IIH. Sample size calculations for such a trial to treat significant vision loss are presented.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can lead to prominent nerve hypertrophy, which can mimic other forms of neuropathy radiologically. Neuro-ophthalmological complications can also occur in CIDP, either at presentation or chronically in the disorder. This can also cause diagnostic difficulties. We report three cases of neuro-ophthalmological complications of CIDP: two cases of papilloedema and one case of proptosis. In all three cases cranial nerve hypertrophy was present. CIDP should be considered in neuro-ophthalmological presentations associated with cranial/spinal nerve root hypertrophy.

Background and Purpose: The purpose of this study was to identify the incidence and prevalence of idiopathic intracranial hypertension (IIH) in Sheffield, UK., Methods: A retrospective review of case notes was conducted to identify cases of IIH seen between 1 January 2007 and 31 December 2008., Results: Sixteen (15 women and 1 man) new patients were identified to give an incidence within Sheffield of 1.56/100,000/year and 2.86/100,000/year for women. The incidence of IIH in obese women was 11.9/100,000/year. The prevalence of IIH was calculated as 10.9/100,000, and 85.7/100,000 in obese women., Conclusion: A higher incidence of IIH than previously reported UK data was found, which may be because of increasing obesity within the population, or improved case ascertainment., (© 2011 The Author(s). European Journal of Neurology © 2011 EFNS.)

Myasthenia gravis is a disease in which antibodies directed at nicotinic acetylcholine receptors are produced, leading to a deficiency of acetylcholine receptors at the neuromuscular junction. This results in impairment of muscular excitation, which appears clinically as fatigable muscle weakness. Weakness of the extraocular muscles occurs in nearly 90% of all myasthenics at disease onset, with ptosis being the most common presenting feature. Myasthenia gravis affecting one or a combination of the extraocular muscles without ptosis is less common; however, cases such as bilateral internuclear ophthalmoplegia without ptosis have been described in the literature. The authors present a case in which decompensating esophoria was the presenting feature of myasthenia gravis.

Aims: 1. To assess the prevalence of visual impairment in those patients who sustain proximal hip fracture after a simple fall. 2. To test the validity of a simple screening test to identify patients with visual impairment., Methods: Patients on the orthopaedic rehabilitation ward recuperating from proximal hip fracture were recruited. The nurse screener and examining Ophthalmologist independently assessed the patients' distance visual acuity and visual field to confrontation. In addition, the nurse screener assessed for the presence of cataract in the red reflex and the examining Ophthalmologist performed a dilated slit-lamp examination. On completion of the examination, the Ophthalmologist documented the cause(s) of any visual impairment found., Results: A total of 89 patients were assessed. In all, 29 patients (33%) could be classified as visually impaired using the United States criteria and 52 patients (58%) had a distance visual acuity of 6/18 or worse in at least one eye. The test reliably identified those patients with visual impairment (sensitivity 94% (+/-5%), specificity 92% (+/-6%)), but was less reliable at identifying those patients with potentially remedial visual impairment (sensitivity 70% (+/-10%), specificity 92% (+/-6%))., Conclusion: The level of visual impairment in this group of patients is high and screening for visual impairment in the elderly with a history of falls is justified. We have demonstrated that a suitably trained member of the rehabilitation team can identify over 94% of those patients with impaired vision. We believe this simple test should now be incorporated into the assessment of all patients requiring rehabilitation after a proximal hip fracture.