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[This corrects the article DOI: 10.3389/fnins.2023.1278626.].

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BACKGROUND AND PURPOSE: Neuromyelitis optica spectrum disorder is a demyelinating and inflammatory affliction that often leads to visual disturbance. Various imaging techniques, including free-water imaging, have been used to determine neuroinflammation and degeneration. Therefore, this study aimed at determining multimodal imaging differences between patients with neuromyelitis optica spectrum disorder, especially those with visual disturbance, and healthy controls. MATERIALS AND METHODS: Eighty-five neuromyelitis optica spectrum disorder patients and 89 age- and sex-matched healthy controls underwent 3-T magnetic resonance imaging (MRI). We analyzed adjusted brain-predicted age difference, voxel-based morphometry, and free-water-corrected diffusion tensor imaging (DTI) by tract-based spatial statistics in each patient group (MRI-positive/negative neuromyelitis optica spectrum disorder patients with or without a history of visual disturbance) compared with the healthy control group. RESULTS: MRI-positive neuromyelitis optica spectrum disorder patients exhibited reduced volumes of the bilateral thalamus. Tract-based spatial statistics showed diffuse white matter abnormalities in all DTI metrics in MRI-positive neuromyelitis optica spectrum disorder patients with a history of visual disturbance. In MRI-negative neuromyelitis optica spectrum disorder patients with a history of visual disturbance, voxel-based morphometry showed volume reduction of bilateral thalami and optic radiations, and tract-based spatial statistics revealed significantly lower free-water-corrected fractional anisotropy and higher mean diffusivity in the posterior dominant distributions, including the optic nerve radiation. CONCLUSION: Free-water-corrected DTI and voxel-based morphometry analyses may reflect symptoms of visual disturbance in neuromyelitis optica spectrum disorder.

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INTRODUCTION: Keratoconus is a progressive disorder of the cornea that causes thinning (Sedaghat et al. in Sci Rep 11(1):11971, 2021), ectasia, and irregular astigmatism, resulting in poor visual acuity that cannot be corrected with standard sphero-cylindrical spectacle lenses. One feature distinguishing keratoconic corneas is ocular aberrations, manifesting up to five or six times the amount of higher-order aberrations than a normal, healthy eye. These aberrations can cause visual disturbances even at the very early stages of the disease. METHODS: In the past, a diagnosis was derived from clinical symptoms, but technological advances have revealed multiple pre-clinical features, allowing for the differentiation between keratoconic and normal eyes at a much earlier stage. These include anterior and posterior corneal surface elevations, the corneal pachymetry profile, corneal epithelial patterns, wavefront aberration metrics, and corneal biomechanics (Sedaghat et al. in Sci Rep 11(1):11971, 2021).This review discusses the aberrations associated with keratoconus, how to measure them, and treatment methods to minimize their negative influence. CONCLUSIONS: Early diagnosis can lead to early treatment and may allow for arresting progression, thereby improving the long-term prognosis. With the acceleration of refractive surgery, it is important to identify patients with keratoconus, as they are usually contraindicated for refractive surgery.

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Tuberculum sellae meningiomas commonly present as bitemporal hemianopia and loss of visual acuity due to optic nerve compression. Two female patients (48 and 58 years old) presented with a small scotoma at the lower visual field center due to tuberculum sellae meningioma (25 and 10 mm, respectively). Despite the fact that their visual field defect was not very large, daily activities, including walking or reading were hindered. By the total removal of the tumors in both patients, the scotoma was cured and daily activities recovered. When patients exhibit visual deficits, especially in the lower center fields, surgical removal should be considered even if the tumors are small and visual deficits are limited because improvement of both vision and daily activities can be achieved.

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Retinal migraine is usually characterized by attacks of fully reversible monocular visual loss associated with migraine headache. Retinal migraine is most common in women of child-bearing age who have a history of migraine with aura. In the typical attack, monocular visual features consist of partial or complete visual loss lasting less than 1h. Although the current diagnostic criteria for retinal migraine require fully reversible visual loss, our findings suggest that irreversible visual loss is part of the retinal migraine spectrum. Nearly half of reported cases with recurrent transient monocular visual loss subsequently experienced permanent monocular visual loss.

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AIM: To compare the short-term postoperative visual acuity, visual disturbance phenomena, and spectacle independence rate in patients who underwent monocular implantation with trifocal, multifocal, or extended range of vision intraocular lens(IOL).METHODS: A retrospective analysis was conducted on 67 cataract patients(67 eyes)who underwent phacoemulsification cataract extraction combined with IOL implantation from March 2019 to December 2022. A total of 35 cases(35 eyes)received Symfony extended range of vision IOL implantation, 21 cases(21 eyes)received AcrySof IQ ReSTOR +3D multifocal IOL, and 11 cases(11 eyes)received AcrySof IQ PanOptix trifocal IOL. The preoperative uncorrected distance visual acuity(UDVA), uncorrected intermediate visual acuity(UIVA), and uncorrected near visual acuity(UNVA)and 3 mo postoperatively were documented. Moreover, defocus curves, visual disturbance phenomena, and spectacle independence rates were recorded at 3 mo postoperatively.RESULTS: At 3 mo postoperatively, no statistically significant differences were observed in UDVA among the three groups(P>0.05). A comparison of UIVA showed superior results in the Symfony and PanOptix groups compared to the ReSTOR group(all P<0.01). The UNVA of both the ReSTOR and PanOptix groups outperformed the Symfony group(all P<0.01). The defocus curves indicated that in the intermediate vision range(-1.00 to -1.50 D), the Symfony group exhibited better performance than the ReSTOR group(P<0.05); while in the near vision range(-2.50 to -3.50 D), the ReSTOR group was superior to the Symfony group(P<0.05). The PanOptix group demonstrated superior visual acuity in the near vision range(-2.00 to -3.50 D)compared to the Symfony group(P<0.05)and was also superior in the intermediate vision range(-1.00 to -2.00 D)compared to the ReSTOR group(P<0.05). No significant differences were observed in the incidence of glare or halo and binocular interference phenomena among the three groups(P>0.05). The PanOptix and ReSTOR groups exhibited a higher spectacle independence rate compared to the Symfony group(P<0.0167).CONCLUSION: Compared to Symfony extended range of vision IOL and ReSTOR multifocal IOL, PanOptix trifocal IOL offers a balanced approach to distance, intermediate, and near visual acuity, without a high incidence of glare and halo, and with a higher spectacle independence rate. Caution is still advised when considering monocular implantation with presbyopia-correcting IOLs.

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Purpose: To investigate the impact of patient-reported visual disturbance on dynamic visual acuity in myopic patients after corneal refractive surgery. Methods: This is a prospective nonrandomized study. Adult myopic patients receiving bilateral laser-assisted sub-epithelial keratomileusis (LASEK), femtosecond laser-assisted in situ keratomileusis (FS-LASIK), or small incision lenticule extraction (SMILE) with Plano target were included. Eight types of patient-reported visual disturbance were evaluated regarding frequency, severity and bothersome and dynamic visual acuity (DVA) of 40 and 80 degrees per second (dps) was measured postoperatively at 3 months. Results: The study enrolled 95 patients with an average age of 27.6 ± 6.4 years. The most frequently reported visual disturbance was the fluctuation in vision (70.5%), followed by glare (66.3%) and halo (57.4%). Postoperative DVA at 80 dps was significantly associated with the total score of haloes (p = 0.038) and difficulty in judging distance (p = 0.046). Significant worse postoperative DVA at 40 dps was observed in patients with haloes than those without (p = 0.024). The DVA at 80 dps for patients without haloes or difficulty in judging distance was significantly better than that with the symptoms (haloes, p = 0.047; difficulty in judging distance, p = 0.029). Subgroup analysis by surgical procedures demonstrated that the significant difference in DVA between patients with and without visual disturbance was only observed in patients receiving FS-LASIK. Conclusion: Postoperatively, myopic patients undergoing corneal refractive surgery with haloes or difficulty in judging distance have significantly worse low and high-speed DVA than those without the symptoms. The present study provided the basis for postoperative guidance in daily tasks involving dynamic vision when patients have visual disturbances.

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Background: Rhabdoid meningiomas (RMs) are a rare type of malignant meningioma. Here, we report a case of intracranial RM presenting with visual disturbance and prominent hyperintensity in the optic nerve (ON). Case Description: A 20-year-old female presented with a 1-year history of headache. At presentation, her visual acuity (VA) was 20/50 on the right side and 20/40 on the left, with an intraocular pressure of 17 mmHg on both sides. Cerebral magnetic resonance imaging revealed a broad-based tumor in the right frontal convexity. It measured 82 mm × 65 mm × 70 mm in diameter, accompanied by cystic components, and was inhomogeneously enhanced. The intraorbital ONs demonstrated prominent intramedullary hyperintensity on the constructive interference steady-state sequence. Gross total tumor resection was performed and the pathology was consistent with RM. Immediately after surgery, her VA and IOP were 20/17 and 10 mmHg, respectively, with a remarkable resolution of the intramedullary hyperintensity. Conclusion: Prominent hyperintensity in the ON identified in patients with chronic intracranial hypertension may be an indicator of visual disturbance. It can rapidly resolve after resolution of intracranial hypertension with functional recovery.

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A 6-year-old girl presented with moderate-intensity headache, frequent vomiting, visual disturbance, and left-sided decreased hearing for 7 months. The neurologic examination revealed a right upper motor neuron facial nerve palsy, left-sided 4-mm sluggish pupil (right: 3-mm reactive), and unsteady gait. Fundoscopy was notable for bilateral papilledema. Brain magnetic resonance imaging with contrast demonstrated a giant multiloculated suprasellar cystic lesion (9.7 × 10.5 × 7.6 cm). It extended to the left anterior cranial fossa, both middle cranial fossae, and posterior fossa prepontine region with consequent effect on the brainstem and moderate hydrocephalus. The patient underwent a right frontal external ventricular drain insertion and left frontotemporal craniotomy and tumor resection. Histopathologic sections were compatible with adamantinomatous craniopharyngioma. Giant craniopharyngiomas have rarely been reported. This article presents the clinical and radiologic outcomes of a patient with a giant craniopharyngioma.

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Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease caused by JC virus infection of oligodendrocytes. Little has been reported on iron deposits in patients with PML. Herein, we report a case of PML with massive iron deposition in the juxtacortical regions attaching white matter lesions in a 71-year-old woman who developed bilateral visual disturbance and progressive aphasia after 16 months of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone treatment for follicular lymphoma. Magnetic resonance imaging revealed white matter lesions in the left parietal and other lobes with massive iron deposition in the juxtacortical lesions. A PCR test for JC virus was positive, confirming the diagnosis of PML. Despite treatment with mefloquine and mirtazapine, the patient died six months later. At autopsy, demyelination was found dominantly in the left parietal lobe. Moreover, hemosiderin-laden macrophages and reactive astrocytes containing ferritin were abundant in the juxtacortical regions adjacent to the white matter lesions. This is a previously unreported case of PML after lymphoma, in which iron deposition was confirmed both radiologically and pathologically.

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Background Patients with neurofibromatosis type 1 (NF1) have various vascular diseases due to the vascular fragility, but no reports of case of giant thrombotic aneurysm was found. We treated a rare case of giant thrombotic aneurysm of the internal carotid artery (ICA) in a patient with NF1. Case Presentation A 60-year-old man had suffered deteriorating visual loss and homonymous hemianopia. Contrast-enhanced computed tomography showed a giant thrombosed aneurysm on the anterior wall of the ICA located in the optic chiasma. We planned and completed the external carotid artery-middle cerebral artery high-flow bypass using radial artery graft. The visual fields test was performed 14 days after surgery. Homonymous hemianopia persisted but no exacerbation of visual field impairment was observed. No complications were found at 14 days after surgery and the postoperative course was uneventful. Conclusion We consider that external carotid artery-middle cerebral artery bypass surgery using radial artery grafts is a safe and effective treatment method for giant thrombotic aneurysm associated with NF1.

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Background and objective Pituitary apoplexy (PA) is a possible life-threatening disorder due to spontaneous hemorrhage or impaired blood supply in the pituitary gland. It may present as an acute or subclinical form, and treatment options include either surgery or a conservative approach. The purpose of this study was to retrospectively analyze the clinical, imaging, and hormonal features, as well as the therapeutic outcomes, in a relatively short period of time in a series of consecutive patients with pituitary apoplexy (PA). Results Thirty-six patients were included, 50% presenting typical symptoms of PA. The presenting symptoms were headache (44.4%), visual abnormalities (44.4%), and digestive symptoms (22.2%). At diagnosis, hormonal deficiency was observed in 22 (61.1%) patients. Of the evaluated patients, 78.2% of the 23 operated cases and all unoperated cases presented tumor remnants. Vision improved in 81.8% of the operated and 100% of conservatively managed cases. Of all cases, 69.4% remained with long-term hypopituitarism. Conclusion Complex management of PA frequently leads to visual improvement but long-standing hypopituitarism.

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A 76-year-old man receiving maintenance therapy with oral steroids for immunoglobulin G4 (IgG4)-related disease presented to our hospital with the chief complaint of visual disturbance. His best corrected visual acuities of the right and left eye were 1.2 and 0.7, respectively. Humphrey visual field test revealed inferior auriculotemporal one-quarter blindness in the left eye. After detailed history-taking for IgG4-related disease, clinical diagnosis based on imaging revealed the marked pituitary/pituitary stalk enlargement with associated optic chiasm compression. Based on the history and initial evaluation findings, a diagnosis of IgG4-related ophthalmic disease was made. Intensified steroid therapy was performed, which led to symptom resolution. IgG4-related diseases are considered in the differential diagnosis when bilateral hemianopsia is observed. When unilateral visual acuity and visual field defects are present, IgG4-related diseases and other organ disorders should be considered.

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OBJECTIVES: To investigate the clinical correlates of visual symptoms in patients with migraine. METHOD: Patients with migraine that attended our headache clinics were enrolled. Headache profiles, disability, and comorbidities were acquired with structured questionnaires. A semi-structured visual phenomenon questionnaire was also used to assess the characteristics of visual symptoms, including visual aura in patients with migraine with aura and transient visual disturbance in patients with migraine without aura. Headache specialists interviewed with the participants for the ascertainment of diagnosis and verification of the questionnaires. RESULT: Migraine with aura patients with visual aura (n = 743, female/male = 2.3, mean age: 34.7 ± 12.2 years) and migraine without aura patients with non-aura transient visual disturbance (n = 1,808, female/male = 4.4, mean age: 39.4 ± 12.6 years) were enrolled. Patients with transient visual disturbance had higher headache-related disability and more psychiatric comorbidities. Chronic migraine was more common in migraine without aura than migraine with aura patients (41.9% vs. 11.8%, OR = 5.48 [95% CI: 4.33-7.02], p < 0.001). The associations remained after adjusting confounding factors. CONCLUSION: Presence of non-aura transient visual disturbance may suggest a higher migraine-related disability and is linked to higher risk of chronic migraine than typical migraine aura in migraine patients. Further studies are needed to elucidate the potential mechanism.

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The pathogenesis of new visual symptoms after flow diverter stent placement in the ophthalmic artery for internal carotid artery aneurysms remains unclear. We report two cases of patients who developed visual field disturbance and decreased visual acuity following flow diverter placement. The "doughnut sign" was found around the optic nerve on magnetic resonance imaging. The patients had progressive visual field defects and impairment on the side where the flow diverter was placed. Short tau inversion recovery coronal images showed a doughnut-shaped high-signal around the optic nerve on the affected side. Both patients were treated with steroid pulse therapy, and 1 received endovascular therapy. Their symptoms gradually improved, and the "doughnut sign" disappeared. The "doughnut sign" observed around the optic nerve on magnetic resonance imaging may be found alongside visual disturbance symptoms after paraclinoid aneurysm treatment. It is recommended that short tau inversion recovery sequences be performed preoperatively in patients presenting with visual impairment and in whom the possibility of postoperative exacerbation is suspected.

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Introduction: The present study aimed to determine the incidence of intraprocedural visual-evoked potential (VEP) changes and to identify correlations with intraprocedural ischemic complications during endovascular treatment in patients with intracranial aneurysm related to visual function. Methods: This study analyzed data from 104 consecutive patients who underwent endovascular coil embolization to treat intracranial aneurysms related to visual function under VEP and transcranial motor evoked potential (MEP) monitoring. We analyzed associations between significant changes in MEP and VEP, defined as a >50% decrease in amplitude, and both intraprocedural complications and postoperative neurological deficits. Factors associated with postoperative neurological deficits were also assessed. Results: Treated aneurysms were predominantly located in the internal carotid artery (95%). Five (5%) were located in the posterior cerebral artery (PCA). Significant decreases in intraprocedural VEP occurred in four patients (4%), although one of those four patients did not show concomitant MEP decreases during procedures. Immediate salvage procedures avoided postoperative visual disturbances. All VEP decreases were transient and not associated with postoperative visual impairment. One of three cases who underwent intraoperative balloon occlusion test showed tolerance to balloon occlusion of the proximal PCA under VEP assessment; parent artery occlusion was performed without postoperative visual disturbance in that case. Conclusion: Although significant VEP decreases occurred 4% during neuro-endovascular aneurysm treatment related to visual function, intraprocedural VEP monitoring identifies ischemic changes associated with visual pathways and facilitates prompt initiation of salvage procedures.

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Introduction: Pituitary metastases are very rare in cancer patients and often originate from lung or breast tumors. They usually occur in patients with known metastatic disease, but rarely may be the first presentation of the primary tumor. Methods: We present the case of a 58 years-old-man who reported a three-month history of polyuria-polydipsia syndrome, generalized asthenia, panhypopituitarism and bitemporal hemianopsia. Brain-MRI showed a voluminous pituitary mass causing posterior sellar enlargement and compression of the surrounding structures including pituitary stalk, optic chiasm, and optic nerves. Results: The patient underwent neurosurgical removal of the mass. Histological examination revealed a poorly differentiated adenocarcinoma of uncertain origin. A total body CT scan showed a mass in the left kidney that was subsequently removed. Histological features were consistent with a clear cell carcinoma. However, endoscopic examination of the digestive tract revealed an ulcerating and infiltrating adenocarcinoma of the gastric cardia. Total body PET/CT scan with 18F-FDG confirmed an isolated area of accumulation in the gastric cardia, with no hyperaccumulation at other sites. Conclusion: To the best of our knowledge, there are no reports of pituitary metastases from gastric cardia adenocarcinoma. Our patient presented with symptoms of sellar involvement and without evidence of other body metastases. Therefore, sudden onset of diabetes insipidus and visual deterioration should lead to the suspicion of a rapidly growing pituitary mass, which may be the presenting manifestation of a primary extracranial adenocarcinoma. Histological investigation of the pituitary mass can guide the diagnostic workup, which must however be complete.

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Aim: By reviewing the existing clinical studies about visual snow (VS) as a symptom or as part of visual snow syndrome (VSS), we aim at improving our understanding of VSS being a network disorder. Background: Patients with VSS suffer from a continuous visual disturbance resembling the view of a badly tuned analog television (i.e., VS) and other visual, as well as non-visual symptoms. These symptoms can persist over years and often strongly impact the quality of life. The exact prevalence is still unknown, but up to 2.2% of the population could be affected. Presently, there is no established treatment, and the underlying pathophysiology is unknown. In recent years, there have been several approaches to identify the brain areas involved and their interplay to explain the complex presentation. Methods: We collected the clinical and paraclinical evidence from the currently published original studies on VS and its syndrome by searching PubMed and Google Scholar for the term visual snow. We included original studies in English or German and excluded all reviews, case reports that did not add new information to the topic of this review, and articles that were not retrievable in PubMed or Google Scholar. We grouped the studies according to the methods that were used. Results: Fifty-three studies were found for this review. In VSS, the clinical spectrum includes additional visual disturbances such as excessive floaters, palinopsia, nyctalopia, photophobia, and entoptic phenomena. There is also an association with other perceptual and affective disorders as well as cognitive symptoms. The studies that have been included in this review demonstrate structural, functional, and metabolic alterations in the primary and/or secondary visual areas of the brain. Beyond that, results indicate a disruption in the pre-cortical visual pathways and large-scale networks including the default mode network and the salience network. Discussion: The combination of the clinical picture and widespread functional and structural alterations in visual and extra-visual areas indicates that the VSS is a network disorder. The involvement of pre-cortical visual structures and attentional networks might result in an impairment of "filtering" and prioritizing stimuli as top-down process with subsequent excessive activation of the visual cortices when exposed to irrelevant external and internal stimuli. Limitations of the existing literature are that not all authors used the ICHD-3 definition of the VSS. Some were referring to the symptom VS, and in many cases, the control groups were not matched for migraine or migraine aura.

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Background and Purpose: Visual Snow (VS) is a disorder characterised by the subjective perception of black-and-white visual static. The aetiology of this condition is not known. In our previous work we suggested that there is a link between short-wave (S or "blue" cone) signals and severity of visual snow symptoms. Therefore we aimed to further characterise this potential link. Methods: Patients (n = 22) with classic VS based on the diagnostic criteria and healthy controls (n = 12), underwent Intuitive Colorimetry (IC) testing (Cerium Visual Technologies). Twelve hue directions (expressed as angle in CIE 1976 LUV space relative to D65) were rated on a five-point scale from preferred (relieving, positive score) to non-preferred (exacerbating, negative score), and overall preferred and non-preferred angles were chosen. Results: A non-preferred violet region near the tritanopic confusion line / S-cone axis (267 deg.) was strongly associated with exacerbation of VS symptoms (range 250-310 deg, mean 276 ± 16, n = 20, Rayleigh p < 0.001). Two subjects with non-preferred region > 90 deg from mean were considered as outliers. Median rank at hue angle 270 deg was significantly lower than at angle 90 (-1.5 vs. 0.0, p < 0.001, Wilcoxon non-parametric rank-sum test). Patients showed preference for one of two spectral regions which relieved VS symptoms: orange-yellow (range 50-110 deg., mean 79 ± 24, n = 14) and turquoise-blue (range (210-250 deg., mean 234 ± 27, n = 8). Conclusion: Our results show that visual snow symptoms are exacerbated by colour modulation that selectively increased levels of S-cone excitation. Because S-cone signals travel on primordial brain pathways that regulate cortical rhythms (koniocellular pathways) we hypothesis that these pathways contribute to the pathogenesis of this disorder.

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BACKGROUND: Patients with neurohypophyseal germ cell tumors (GCTs) typically present with visual problems. Hence, this study aimed to assess optic pathway involvement based on clinical and radiological findings and to validate the outcome of visual function. METHODS: A total of 16 patients with newly diagnosed neurohypophyseal GCTs who were treated at the University of Tsukuba Hospital between 2000 and 2020 were included in this study. RESULTS: The median interval from symptom onset to diagnosis was 173.5 days (range, 33-1588 days). Patients with visual disturbance at diagnosis had a longer time to diagnosis compared with those without. Ophthalmologic abnormalities were frequently observed, with an incidence rate of 69%. Fifty percent of patients exhibited optic pathway involvement detected via magnetic resonance imaging (MRI). Visual impairment was more severe in the patients with optic pathway involvement (p = 0.002). Post-treatment visual impairment was improved but was still significantly severe in patients with optic pathway involvement than in those without involvement (p = 0.010). Visual field deficit more likely remained with an improvement rate of 50%, whereas the improvement rate of visual acuity was 78%. Further, none developed late-onset visual deterioration during the follow-up period. CONCLUSIONS: Visual disturbance and optic pathway involvement are common in neurohypophyseal GCTs. Visual impairment particularly in patients with optic pathway involvement on MRI is more likely to remain at follow-up, although the outcome of visual function is acceptable in most cases.

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