The last decade has seen an unprecedented pace of translation and innovation in the field of neuro-ophthalmology, providing our previously diagnosable but untreatable patients with new management options that reduce morbidity and mortality. No longer are we the subspeciality that simply admires the disease, and now we have much more than steroids to offer patients.
We recognise that many general Ophthalmologists take care of neuro-ophthalmology diseases, and this was the focus for the “How to do it” opinion articles. The remit was to provide practical advice for targeted investigation, diagnosis and management starting with two common neuro-ophthalmic conditions, optic neuritis (ON) and idiopathic intracranial hypertension (IIH). There has been a dramatic shift in the way we should manage ON, as there a broad spectrum of ON pathology that needs urgent treatment to preserve vision [1]. Secondly, IIH is a condition that has become prevalent in the last decade and managing the spectrum of visual involvement can be challenging [2].
There can only be a few people who are not terrified examining a patient with nystagmus, let alone managing it. Madill and colleagues have distilled their experience and provided an aide to diagnosis to reduce unnecessary confusion and incorrect investigations [3]. With a rise in patient support groups and online forums, many patients with unusual visual phenomena are finding more information that lead them to self-diagnosis. The diagnostic criteria and biological basis for visual snow syndrome is being characterised, and while it is not vision threatening, having a good approach to people living with visual snow syndrome is helpful [4]. Another thought provoking opinion paper approaches functional visual loss from a perspective and understanding that many of us may not have had before [5].
It was important to build in authoritative reviews to keep pace with the advances in neuro-ophthalmic diagnosis, treatment, and monitoring. Conditions like non-arteritic anterior ischaemic optic neuropathy and pituitary pathology may seem relatively easy and with little change in management over many years, however there are increasing nuances to providing the best patient care [6, 7]. The world of ON is no longer as simple as Multiple Sclerosis (MS) and non-MS related disease. Ramanathan and colleagues provide clear phenotyping for myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) [8]. With travel returning to pre-COVID-19 levels and an increasing number of ex-patriots living abroad, we will all see patients with diseases classically associated with somewhere else that we may be less familiar with. The infectious optic neuropathies case based article pulls together many conditions that may require bespoke management [9]. Following on there are two more papers looking at the infectious pathologies, herpes zoster ophthalmicus and syphilis. The two articles included here have allowed our colleagues to give their invaluable insights for both [10, 11].
Keeping the issue topics global, traumatic optic neuropathy (TON) may be less frequently found in the editors’ countries. Meanwhile working in counties like Cambodia or Mongolia, Ophthalmologists might see between two to three TON cases per day. Blanch and colleagues outline the triangulated opinion that glucocorticoids should not be used for traumatic injury [12]. Corneal neuropathic pain is a debilitating condition that currently lacks translational programs to deliver therapies to meet patient’s needs: Watson and colleagues tackle this topic [13]. There is a thoughtful paper on dystrophic calcification and the relevance to optic disc drusen, while we still don’t have a treatment, understanding pathogenesis may pave the way for future therapies [14]. Chen and colleagues have laid the foundation of our knowledge to date on central retinal artery occlusion, while we await the results of three European trials evaluating intravenous thrombolysis within 4.5 h of onset of symptoms [15].
While neuro-ophthalmology is centred on history, more history and then examination, there is an increasing supportive role of diagnostic tests. These are highlighted through three clinicopathological articles. Optical coherence tomography (OCT) imaging has become a cornerstone of the neuro-ophthalmic examination [16]. Neuro-imaging has become an essential investigation; however pathology can be missed if the correct protocol is not requested and performed [17]. There are many patients the readers may have in clinic, where structural changes are just not providing the answer. The electrodiagnostic (EDTs) case review not only superbly translates the complex into simple but it will help you navigate how and when EDTs can be diagnostic and where they are helpful in follow-up [18].
In the age of “apps” to make our lives simpler, Bano et al. provided a clear review of those ophthalmology applications that are validated for clinical use [19]. Likewise purchasing a pupilometer for your clinical practice may be daunting, as not all devices sample the pupil movements in the same way [20]. With more accessible information than ever before, we have been observing the rise of big data research in neuro-ophthalmology. This holds great potential in a specialty that often deals in rare diseases [21].
Ophthalmologists interact with many other specialists as part of a multi-disciplinary team, including colleagues such as neurology, rheumatology, infectious diseases, to name a few! This interaction requires us to understand conditions that may lie, for the most part, outside our own specialty. Therefore we invited non-ophthalmologists to demystify investigation and treatment of myasthenia gravis and giant cell arteritis [22, 23].
To completely shift the neuro-ophthalmic paradigm, one of the most exciting papers in this compendium is nerve fibre organisation in the human optic nerve and chiasm: what do we really know? [24] This article challenges our traditional thinking about the anatomy of the visual pathways. In particular, they support the idea that Wilbrand’s knee does not actually exist. The authors have carefully evidenced their thoughts and conclude that the assumed preservation of retinotopy in the human optic nerve and chiasm is probably not correct [24].
The special issue closes with a short summary of the challenges for the future in the neuro-ophthalmology workforce; in particular as demand has far outstripped the increasing number of people specialising in Neuro-ophthalmology [25]. We reflect on the wealth of advancing knowledge and the excitement of new treatments in this specialty: who would not be drawn to practice in Neuro-ophthalmology today?
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We are deeply in debt to all the reviewers who have dedicated their time to the critical analysis of each paper in this special issue. They met tight timelines and provided their expertise in assuring all the papers are balanced in their viewpoints.
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Mollan S: First drafting of manuscript, collection of papers and critical review of the manuscripts in this special issue. Lee AG: Review of the manuscript, overview of the manuscripts. Fraser CL: First drafting of manuscript, collection of papers and critical review of the manuscripts in this special issue.
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SPM has received payment for consultancy work from Invex Therapeutics. She has received payment for advisory boards from Gentech and Ocular Therapeutix. Grant funding has been paid to her institution from the National Institute of Health Research (NIHR131211), UK Space Agency and IIHUK. She is also a member of the Eye editorial board. AGL, MD, is on the speaker’s bureau for Amgen and Alexion and the Advisory boards for Viridian and is a consultant for Astrazeneca, Bristol Myers Squibb, and Stoke Therapeutics. AGL is also a consultant for the National Football League, NASA, and the United States Department of Justice. AGL is also a member of the Eye editorial board. CLF received payment for the Invex Therapeutics trial steering committee.
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Mollan, S.P., Lee, A.G. & Fraser, C.L. Neuro-ophthalmology: recent advances and paradigm shifts. Eye 38, 2233–2234 (2024). https://doi.org/10.1038/s41433-024-03188-w
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DOI: https://doi.org/10.1038/s41433-024-03188-w