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.

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.

Relapsing-Remitting Sixth Nerve Palsy in Association with Ollier's Disease.

Relapsing-remitting sixth nerve palsy is usually due to ophthalmoplegic migraine (recurrent cranial nerve palsy) in younger patients and microvascular disease in older patients. There have been isolated reports, however, of it occurring in the presence of a skull base tumour. We report a 20-year-old woman with Ollier's disease who presented with a relapsing-remitting sixth nerve palsy. Neuro-imaging revealed a skull base enchondroma.

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.

Bilateral Homonymous Hemianopia with Sparing of the Vertical Meridian.

A 19-year-old woman, who was 16 weeks post partum, collapsed with loss of consciousness. Following awakening she suffered abrupt loss of vision. Partial recovery occurred, although she has been left with bilateral homonymous hemianopia with sparing of vision just to the left of the vertical meridian. Magnetic resonance imaging demonstrated bilateral occipital infarcts, sparing the lips of the calcarine sulcus on the right. This is compatible with our expectation that the vertical meridian of the retinotopic map is represented at the superior and inferior lips of the calcarine sulcus.

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.

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.

Decompensating esophoria as the presenting feature of myasthenia gravis.

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.