Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common cause of acute optic neuropathy in the elderly population and is strongly associated with vascular and microcirculatory abnormalities. We read with interest the study investigating retinal vascular changes in chronic NAION by Dynamic Vessel Analyzer (DVA) measurements. Although the results provide valuable information on the changes of retinal vasculature in NAION, some methodological issues need to be addressed. Among them, we find the possible violation of statistical independence in the case of bilateral disease, the lack of adjustment for important vascular comorbidities, the cautious interpretation of the arteriovenous ratio (AVR) as a surrogate marker of cerebrovascular risk, and the limitations of the retrospective cross-sectional design in establishing predictive value. Future prospective studies with adequate statistical modeling and extensive adjustment for vascular risk factors are necessary to confirm these associations and clarify their clinical significance.
To evaluate and compare peripapillary retinal nerve fiber layer (pRNFL) thickness and optic nerve head (ONH) structural parameters using spectral-domain optical coherence tomography (SD-OCT) in non-optic neuritis (NON) eyes of patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), multiple sclerosis (MS), and healthy controls (HC). This retrospective observational study included three groups: patients with MOGAD, patients with MS, and age- and sex-matched healthy controls. Only NON eyes - those without clinical or radiological evidence of optic neuritis and with negative visual evoked potentials - were included for MOGAD and MS patients. OCT imaging (Cirrus HD-OCT, Carl Zeiss Meditec) was performed to measure average and quadrant-specific pRNFL thickness, along with ONH parameters. A total of 63 participants (45 females, 71.4%) were included: 21 in each group (MOGAD, MS, and HC). The mean age was 37.5 +/- 9.8 years. Average pRNFL thickness differed significantly among the groups (p = 0.001), being lower in MS compared to MOGAD and HC. Optic disc area was significantly larger in MOGAD (p = 0.03). No significant differences were noted in rim area or cup-to-disc ratios, but cup volume was significantly greater in HC (p = 0.002). MOGAD patients without optic neuritis exhibited preserved pRNFL thickness comparable to healthy controls, suggesting an absence of subclinical axonal loss. MS patients showed reduced pRNFL thickness despite no clinical ON, indicating subclinical neurodegeneration. The larger optic disc area in MOGAD may represent a disease-specific anatomical trait warranting further study.
Vitamin B12-deficiency nutritional optic neuropathy (NON) is a potentially preventable cause of permanent visual loss that may occur in individuals following prolonged unsupplemented vegan diets. This cross-sectional observational study assessed peripapillary retinal nerve fiber layer (pRNFL) and ganglion cell complex (GCC) thickness using optical coherence tomography (OCT) in vegan patients with permanent visual loss secondary to vitamin B12-deficiency NON and compared the findings with those of age-matched healthy controls. All participants underwent OCT and standard automated perimetry, and clinical data, dietary history, duration of diet and symptoms, serum vitamin B12 levels, best-corrected visual acuity, mean deviation values, and pRNFL and GCC measurements were evaluated. A total of 22 eyes from 11 vegan patients and 22 eyes from 11 healthy controls were included. Vegan patients showed significant temporal pRNFL thinning and diffuse GCC thinning compared with controls. Significant correlations were observed between the duration of the vegan diet and symptoms, the severity of visual field loss, and several OCT parameters, particularly GCC thickness. Patients with permanent visual loss due to vitamin B12-deficiency nutritional optic neuropathy associated with prolonged unsupplemented vegan diets demonstrated marked temporal pRNFL thinning and diffuse macular GCC loss on OCT, with GCC measurements correlating with symptom duration. These findings support the usefulness of OCT for documenting structural damage in established nutritional optic neuropathy. However, larger longitudinal studies including asymptomatic or early symptomatic vegan individuals are needed to determine whether OCT can detect early neuroaxonal loss or predict visual outcomes.
Leber hereditary optic neuropathy (LHON), toxic optic neuropathy, and nutritional optic neuropathy are three conditions that preferentially affect the metabolically active cells of the papillomacular bundle, causing bilateral optic neuropathy with central vision loss. Herein, we sought to evaluate the visual field index (VFI) as a useful tool for quantifying visual field loss in these optic neuropathies, through comparison with the mean deviation (MD). This was a single center, retrospective, cross-sectional study. Patients were identified using ICD-10 codes, and charts were manually reviewed to confirm the diagnosis. The toxic and nutritional groups were combined into a single cohort due to low numbers and similar presentations, toxic-nutritional optic neuropathy (TNON). Inclusion criteria required automated visual fields. The primary outcome was the correlation between VFI and MD. Fifty-three patients met inclusion criteria: 30 LHON and 23 TNON. Among patients with LHON, there was a strong correlation (r = 0.988 OD, r = 0.975 OS) between the VFI and MD in both eyes. Each one decibel decrease in MD correlated with a 3% decrease in VFI (p < .001 OD, p < .001 OS). In patients with TNON, there also was a strong correlation (r = 0.980 OD, r = 0.985 OS) between the VFI and MD. A one decibel decrease in MD correlated with a 3% decrease in VFI (95%CI: 2.6, 3.2, p < .001 OD, p < .001 OS). VFI demonstrates a strong correlation to MD in both LHON and TNON groups. These findings support the use of VFI to evaluate and explain vision loss in these patient groups and warrant further research into monitoring progression.
Papilledema has long been recognised as a cardinal sign of elevated intracranial pressure, yet its structural underpinnings and dynamic behaviour have only recently become accessible through modern ocular imaging. This review traces the historical development of papilledema understanding, from early anatomical descriptions and the introduction of the ophthalmoscope to contemporary high-resolution imaging, and synthesises current evidence on the structural deformation of the optic nerve head in intracranial hypertension. Particular emphasis is placed on anterior displacement of the optic disc, a parameter that complements peripapillary retinal nerve fibre layer thickness assessment and offers unique insight into pressure-related biomechanics. We summarise key studies evaluating optic disc morphology across different aetiologies, treatment modalities, and time courses, highlighting the emergence of deformation of the optic nerve head patterns and their potential diagnostic and prognostic relevance. By integrating historical context, methodological advances, and contemporary evidence, this review outlines how structural imaging has reshaped current understanding and may further refine clinical assessment of papilledema.
Identification of the trochlear nerve on magnetic resonance (MR) imaging is challenging because it is the thinnest cranial nerve and courses horizontally parallel to the transverse imaging plane. These anatomical features lead to difficulties in detecting small trochlear nerve schwannomas. We identified four cases of presumed small trochlear schwannomas which had been underdiagnosed at outside hospitals due to suboptimal imaging protocols - specifically the absence of gadolinium administration or the lack of high-resolution imaging tailored to the trochlear nerve. In our repeat MR imaging, we employed a high-resolution imaging (voxel size: 0.3 × 0.3 × 0.25 mm), enabling visualization of the entire nerve path within the perimesencephalic cistern. Additional sequences included thin-section postcontrast T1-weighted imaging and orbital coronal T2-weighted imaging. All tumors exhibited clear contrast enhancement and were accurately localized to the trochlear nerve on high-resolution imaging. Orbital coronal images showed ipsilateral atrophy of the superior oblique in all patients. Imaging and clinical findings were reviewed and compared with previously reported cases in the literature. Trochlear nerve schwannomas are rare and often overlooked due to their small size and inconspicuous imaging findings. High-resolution MR imaging tailored for the trochlear nerve is a robust technique to diagnose small trochlear nerve schwannomas.
A comprehensive narrative review of the superficial extracranial artery and orbital features on magnetic resonance imaging (MRI) in giant cell arteritis (GCA) is presented. A search on PubMed, EMBASE and Web of Science for English-language publications detailing MRI head/orbital imaging in GCA was conducted. Inclusion criteria were patients with positive histopathology and/or meeting the 2022 American college of Rheumatology (ACR) criteria who had MRI depicting significant abnormalities of the superficial extracranial vessels and/or orbit. 51 studies were identified for inclusion: 12 studies detailing analysis of superficial extracranial vessels, and 39 studies detailing orbital abnormalities. Articles included ranged from case-reports, retrospective case-series and prospective cohort studies, with variable patient-level data available for extraction and analysis. MRI of the extracranial arteries may demonstrate similar diagnostic power to that of ultrasound and demonstrates promising sensitivity and specificity when compared to biopsy-proven cases. The orbital features on MRI may occasionally be overlooked, however key structures include the intraconal orbital fat, optic nerve and nerve sheath and ophthalmic arteries. Subtle changes may exist in these clinically significant structures and represent important features which need to be identified to facilitate expedient diagnosis or guide further investigation and appropriate treatment. GCA should be a differential consideration in patients with non-specific intraconal fat inflammation and/or optic peri-neuritis, particularly if bilateral. Additionally, the concurrent presence of orbital inflammation (e.g.: optic peri-neuritis, intraconal orbital inflammation) and extracranial vessel wall inflammation should indicate a consideration of vasculitic pathology such as GCA.
We highlight key diagnostic gaps in a reported case of toluene-related toxic optic neuropathy, including the absence of anion gap calculation, lack of explicit methanol exclusion, and incomplete temporal characterization of visual symptoms. A comprehensive toxicological workup - including serum methanol levels, osmolar gap, and metabolic profiling - is essential to distinguish toluene from methanol or mixed solvent toxicity in cases of acute bilateral visual loss.
Meningiomas are the most frequent primary intracranial tumors. Neuroimaging remains a pillar for the diagnosis and the clinical decisions that follow. Magnetic Resonance Imaging (MRI) has been the gold standard tool for evaluating meningiomas, alongside CT scans. However, many obstacles persist, hence the need for new technologies providing functional and molecular information on these tumors. This literature review aims to evaluate the role of 68Gallium DOTATATE PET scan in the diagnosis and management of meningiomas, specifically optic nerve sheath meningiomas (ONSM). Studies on this imaging technique found that the 68Ga-DOTATATE PET scan was more efficient than MRI in differentiating meningiomas from normal tissues, with a sensitivity of 90.1% compared to 79%. The scan was superior in distinguishing scar tissues from tumoral tissues and could detect a disease recurrence with a better sensitivity than the MRI. The addition of 68Ga-DOTATATE to the clinical management of meningiomas with neuro-ophthalmic implications turned out to be beneficial for the diagnosis, surgical planning, post-surgical radiation, and surveillance. This technology could allow to overcome certain limitations found with MRIs and CT scans, such as delineating tumor spread in anatomically challenging areas as well as attributing smaller and more precise radiation volumes.
Diplopia in thyroid eye disease (TED) results from restrictive myopathy and can be disabling. Teprotumumab, an insulin-like growth factor-1 (IGF-1) antagonist, reduces proptosis and improves diplopia as measured by a subjective Gorman diplopia score without assessing ocular misalignment. This study evaluated whether there was observed variability between subjective versus objective measures of diplopia assessment in patients with TED treated with teprotumumab. This retrospective case series included patients with TED-related diplopia treated with teprotumumab at a single institution. Gorman diplopia scores and strabismus measurements in primary and cardinal gaze positions were recorded before and after treatment. Patients without diplopia, with unrelated strabismus, or with <3 months of post-treatment follow-up were excluded. The primary endpoint was change in ocular alignment and Gorman score before and after teprotumumab and the secondary endpoint was examining the difference between the two outcomes of diplopia assessment. Of 67 patients treated, 17 met inclusion criteria. Teprotumumab improved ocular alignment in 71% (12/17) and Gorman score in 59% (10/17). Changes in alignment and Gorman score were not in alignment in 41% (7/17): 12% (2/17) showed Gorman improvement despite worsened alignment, while 30% (5/17) had alignment improvement without change in Gorman score. Teprotumumab improves both ocular alignment and diplopia in most TED patients; however, differences were observed between subjective and objective outcome measures. Validated tools are needed to assess therapeutic outcomes more accurately in TED-related strabismus.
A 68-year-old lady presented with sudden onset painless binocular diplopia, which was worse with distance vision. She was diagnosed with an isolated right abducens nerve palsy which resolved fully after 2 months. Between 2014 and 2025, she experienced 11 similar episodes. Initial neuroimaging was reported as normal, and other causes, such as myasthenia, were ruled out. She was discussed at a multidisciplinary neuroradiology meeting where the compression of the abducens nerve by an aberrant anterior inferior cerebellar artery (AICA) was identified as the likely cause of these recurrent episodes.
Previous studies have highlighted problems with the sight impairment registration process due to lack of consistency in applying criteria, inaccuracies in epidemiological data and delays. We carried out a service evaluation to examine the spectrum of conditions leading to sight impaired (SI) or severely sight impaired (SSI) registration in an adult neuro-ophthalmology service, to assess adherence to official guidance, whether there were delays in registration and to identify potentially preventable causes. Clinical records of all individuals registered as SI or SSI in our adult neuro-ophthalmology clinic between June 2017 and June 2023 were reviewed. Seventy individuals were registered as SI and 50 as SSI. The commonest causes affecting both eyes were non-arteritic anterior ischemic optic neuropathy (n = 22), stroke (n = 18) and idiopathic intracranial hypertension (IIH) (n = 8). Fifteen individuals were registered as SI without strictly meeting criteria, including eight with quadrantanopias. One was classified as SI despite fulfilling SSI criteria. There was diagnostic classification disagreement in 21 cases. The median delay to registration was 0 days (interquartile range 46.75 days). Potentially preventable causes included IIH (n = 6), giant cell arteritis (n = 3), pituitary adenoma (n = 3), and bilateral optic neuritis secondary to multiple sclerosis (n = 1). Our study demonstrated that although most registrations were accurate, sources of inconsistency persisted, including variable interpretation of eligibility criteria, disagreement over diagnostic categorization, and some delays in registration. These issues mirror longstanding concerns about variability in practice and its impact on equitable access to support and the accuracy of epidemiological data.
Paranasal sinus mucoceles can present with various ophthalmic symptoms because of their capacity to expand and impinge on nearby nerves and vasculature. We present a 64-year-old white female with blurry vision OD, binocular oblique diplopia, headaches, and pupillary trace escape OD that started in 2023. The patient has a significant past medical history for multiple sinus diseases and surgeries and chronic immunosuppression. Despite several visits with ophthalmology, optometry, and neurosurgery, no definitive diagnosis was made due to the variability in imaging and clinical features. Finally, a right total ethmoidectomy, right sphenoidotomy, bilateral inferior turbinate lateralization, and image guided endoscopic sinus surgery biopsy were conducted, revealing a skull base mucocele and an MSSA infection.
This retrospective cross-sectional study aimed to determine the prevalence and significance of MRI intracranial hypertension signs in children undergoing imaging for any clinical indication and investigate their correlation with papilledema findings. Data were analyzed from 160 children under 15 years who underwent both head MRI and ophthalmologic examination within a one-month interval at two tertiary medical centers in Israel over a two-year period. Ten established radiological markers associated with intracranial hypertension were evaluated by two specialized neuroradiologists, and ophthalmologic examinations were performed to assess for papilledema. Papilledema was confirmed in 19 patients (11.9%). Six MRI signs showed significant association with papilledema (p < .05), with optic nerve head protrusion demonstrating the highest odds ratio (OR = 30.2; 95% CI, 8.5-107.4; p < .001). Patients with papilledema had a significantly higher mean number of radiological signs than those without (3.1 vs 0.7, p < .001). The presence of at least one positive MRI sign increased papilledema odds (OR = 8.2), while more than three positive signs substantially elevated this risk (OR = 14.9). Both individual radiological markers and their cumulative presence significantly increase the likelihood of papilledema in children. Even when discovered incidentally, multiple MRI signs of intracranial hypertension warrant careful ophthalmological evaluation, as the presence of three or more radiological indicators is associated with nearly 15-fold increased odds of papilledema.
