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Purpose of ReviewTo highlight potential avenues to reduce preventable diagnostic error of neuro-ophthalmic conditions and avoid patient harm.Recent FindingsRecent prospective studies and studies of patient harm have advanced our understanding. Additionally, recent studies of fundus photography, telemedicine, and artificial intelligence highlight potential avenues for diagnostic improvement.SummaryDiagnostic error of neuro-ophthalmic conditions can often be traced to failure to gather an adequate history, perform a complete physical exam, obtain adequate/appropriate neuroimaging, and generate a complete, appropriate differential diagnosis. Improving triage and identification of neuro-ophthalmic conditions by other providers and increasing access to subspecialty neuro-ophthalmology evaluation are essential avenues to reduce diagnostic error. Further research should evaluate the relationship between misdiagnosis and patient harm, and help identify the most impactful potential targets for improvement.

Purposeof ReviewTo outline the current landscape of treatments for Leber hereditary optic neuropathy (LHON) along the therapeutic delivery pipeline, exploring the mechanisms of action and evidence for these therapeutic approaches.Recent FindingsTreatments for LHON can be broadly classified as either mutation-specific or mutation-independent. Mutation-specific therapies aim to correct the underlying mutation through the use of a gene-editing platform or replace the faulty mitochondrial DNA-encoded protein by delivering the wild-type gene using a suitable vector. Recent gene therapy clinical trials assessing the efficacy of allotopically expressed MT-ND4 for the treatment of LHON due to the m.11778G > A mutation in MT-ND4 have shown positive results when treated within 12 months of symptom onset. Mutation-independent therapies can have various downstream targets that aim to improve mitochondrial respiration, reduce mitochondrial stress, inhibit or delay retinal ganglion cell apoptosis, and/or promote retinal ganglion cell survival. Idebenone, a synthetic hydrosoluble analogue of co-enzyme Q10 (ubiquinone), is the only approved treatment for LHON. Mutation-independent approaches to gene therapy under pre-clinical investigation for other neurodegenerative disorders may have the potential to benefit patients with LHON.SummaryAlthough approved treatments are presently limited, innovations in gene therapy and editing are driving the expansion of the therapeutic delivery pipeline for LHON.

Purpose of ReviewUntil the last 5 years, there was very little in the literature about the phenomenon now known as visual snow syndrome. This review will examine the current thinking on the pathology of visual snow and how that thinking has evolved.Recent FindingsWhile migraine is a common comorbidity to visual snow syndrome, evidence points to these conditions being distinct clinical entities, with some overlapping pathophysiological processes. There is increasing structural and functional evidence that visual snow syndrome is due to a widespread cortical dysfunction. Cortical hyperexcitability coupled with changes in thalamocortical pathways and higher-level salience network controls have all shown differences in patients with visual snow syndrome compared to controls.SummaryFurther work is needed to clarify the exact mechanisms of visual snow syndrome. Until that time, treatment options will remain limited. Clinicians having a clearer understanding of the basis for visual snow syndrome can appropriately discuss the diagnosis with their patients and steer them towards appropriate management options.

Purpose of ReviewThe article reviews the recent findings on the use of optical coherence tomography angiography (OCTA) in neurology.Recent FindingsOCTA is a new addition to the powerful and complementary technology of the OCT. Due to its noninvasiveness, and reproducibility, it is possible to obtain high-resolution 3D images of the vessels of the human eye. As the vessels of the retina with the presence of endothelial cell’s tight junctions resemble the brain vessels, it was hypothesized that the imaging of the retinal vessels might bring insight into brain vessels. OCTA has been effectively used to predict retinal vessel abnormalities in dementia, demyelization, optic disc neuropathies, and inherited degenerative diseases. Most common findings were decrease of vascular density and flow and an increase of avascular zones.SummaryAlthough OCTA is a relative new technology, recent studies show that it can be successfully applied in neurology.

Purpose of ReviewA pressure difference between the intraocular and intracranial compartments at the site of the lamina cribrosa has been hypothesized to have a pathophysiological role in several optic nerve head diseases. This paper reviews the current literature on the translamina cribrosa pressure difference (TLCPD), the associated pressure gradient, and its potential pathophysiological role, as well as the methodology to assess TLCPD.Recent FindingsFor normal-tension glaucoma (NTG), initial studies indicated low intracranial pressure (ICP) while recent findings indicate that a reduced ICP is not mandatory.SummaryData from studies on the elevated TLCPD as a pathophysiological factor of NTG are equivocal. From the identification of potential postural effects on the cerebrospinal fluid (CSF) communication between the intracranial and retrolaminar space, we hypothesize that the missing link could be a dysfunction of an occlusion mechanism of the optic nerve sheath around the optic nerve. In upright posture, this could cause an elevated TLCPD even with normal ICP and we suggest that this should be investigated as a pathophysiological component in NTG patients.

Purpose of ReviewAntibodies against myelin oligodendrocyte glycoprotein (MOG) are associated with a unique acquired central nervous system demyelinating disease—termed MOG-IgG-associated disorder (MOGAD)—which has a variety of clinical manifestations, including optic neuritis, transverse myelitis, acute disseminating encephalomyelitis, and brainstem encephalitis. In this review, we summarize the current knowledge of the clinical characteristics, neuroimaging, treatments, and outcomes of MOGAD, with a focus on optic neuritis.Recent FindingsThe recent development of a reproducible, live cell–based assay for MOG-IgG, has improved our ability to identify and study this disease. Based on contemporary studies, it has become increasingly evident that MOGAD is distinct from multiple sclerosis and aquaporin-4-positive neuromyelitis optica spectrum disorder with different clinical features and treatment outcomes.SummaryThere is now sufficient evidence to separate MOGAD from other inflammatory central nervous system demyelinating disorders, which will allow focused research on understanding the pathophysiology of the disease. Prospective treatment trials are needed to determine the best course of treatment, and until then, treatment plans must be individualized to the clinical manifestations and severity of disease.

Purpose of ReviewThe goal of this review is to describe the presenting features of fulminant idiopathic intracranial hypertension (IIH) and outline the multimodal approach to its treatment.Recent FindingsVenous sinus stenting may be an appropriate alternative to optic nerve sheath fenestration or cerebrospinal fluid shunting in select patients with fulminant IIH. Prompt surgical intervention maximizes the chance of visual recovery in patients with fulminant IIH.Summary“Fulminant IIH” is defined as intracranial hypertension with no secondary cause, severe vision loss within 4 weeks of symptom onset, and progressive vision loss over days. Rapid recognition of the fulminant phenotype of IIH by emergency department physicians, neurologists, and ophthalmologists is critical. Without appropriate triage and rapid medical and surgical intervention, patients with fulminant IIH are at high risk for profound, permanent vision loss. Prompt surgical intervention with optic nerve sheath fenestration, cerebrospinal fluid shunting, or venous sinus stenting minimizes the chance of poor visual outcome. If a delay is anticipated, serial lumbar punctures or temporary cerebrospinal fluid drainage and medical therapy may forestall irreversible vision loss.

The classic view of sensorineural hearing loss has been that the primary damage targets are hair cells and that auditory nerve loss is typically secondary to hair cell degeneration. Recent work has challenged that view. In noise-induced hearing loss, exposures causing only reversible threshold shifts (and no hair cell loss) nevertheless cause permanent loss of >50% of the synaptic connections between hair cells and the auditory nerve. Similarly, in age-related hearing loss, degeneration of cochlear synapses precedes both hair cell loss and threshold elevation. This primary neural degeneration has remained a \"hidden hearing loss\" for two reasons: 1) the neuronal cell bodies survive for years despite loss of synaptic connection with hair cells, and 2) the degeneration is selective for auditory nerve fibers with high thresholds. Although not required for threshold detection when quiet, these high-threshold fibers are critical for hearing in noisy environments. Research suggests that primary neural degeneration is an important contributor to the perceptual handicap in sensorineural hearing loss, and it may be key to the generation of tinnitus and other associated perceptual anomalies. In cases where the hair cells survive, neurotrophin therapies can elicit neurite outgrowth from surviving auditory neurons and re-establishment of their peripheral synapses; thus, treatments may be on the horizon.

Purpose of ReviewIdiopathic intracranial hypertension (IIH) is a disorder characterized by long-standing elevated intracranial pressure (ICP). As the name applies, no uniform cause has been identified. IIH is typically characterized by headaches, pulsatile tinnitus, and visual deterioration.Recent FindingsAnomalies in cerebrospinal fluid (CSF) absorption are implicated in the pathophysiology of IIH. Non-invasive imaging of the brain parenchyma and the cerebral venous sinus has improved, and research has gained a better understanding of the role of cerebral venous sinus stenosis. Both have led to a better delineation of the role of arachnoid granulations (AG) and the glymphatic system in the development of IIH.SummaryIIH may occur as a result of restrictions of CSF absorption from the venous system, and or the congestion and overflow of the glymphatic system. Elucidating these mechanisms will lead to greater understanding of its underlying pathophysiologic mechanisms.

Purpose of ReviewIn this review, we will describe current methods for visual field testing in neuro-ophthalmic clinical practice and research, develop terminology that accurately describes patterns of field deficits, and discuss recent advances such as augmented or virtual reality-based perimetry and the use of artificial intelligence in visual field interpretation.Recent FindingsNew testing strategies that reduce testing times, improve patient comfort, and increase sensitivity for detecting small central or paracentral scotomas have been developed for static automated perimetry. Various forms of machine learning-based tools such as archetypal analysis are being tested to quantitatively depict and monitor visual field abnormalities in optic neuropathies. Studies show that the combined use of optical coherence tomography and standard automated perimetry to determine the structure-function relationship improves clinical care in neuro-ophthalmic disorders.SummaryVisual field assessment must be performed in all patients with neuro-ophthalmic disorders affecting the afferent visual pathway. Quantitative visual field analysis using standard automated perimetry is critical in initial diagnosis, monitoring disease progression, and guidance of therapeutic plans. Visual field defects can adversely impact activities of daily living such as reading, navigation, and driving and thus impact quality of life. Visual field testing can direct appropriate occupational low vision rehabilitation in affected individuals.