Neuro-Ophthalmology最新文献

To report on the occurrence and characteristics of eye manifestations and determine the predictors of permanent vision loss (PVL) in patients with giant cell arteritis. Case-control study. Retrospective cohort study of 258 patients diagnosed with giant cell arteritis (GCA) over a 20- year period at a single institution. Eighty nine of 258 patients (34.5%) with GCA had vision symptoms and 56 (21.7%) developed PVL. Acute loss of vision with no preceding ocular symptoms occurred in 28.5%; for those with symptoms, median time to vision loss was 4.5 days. Bilateral clinical eye involvement was present in 38%, mostly anterior ischemic optic neuropathy (AION), rarely vascular occlusions. Vision loss was severe with acuity of count fingers or less or mean deficit -20 decibels or less in at least one eye in 80% of patients with PVL. Following initiation of GC treatment, 23% of patients continued to have further vision loss and only 5% showed improvement. We found predictors of PVL by multivariate analysis to be increased age (OR 1.05, p0.040), jaw claudication (OR 2.29, p = .040), diplopia (OR 2.74, p = .039), increased platelets (OR 2.98, p = .020) and lower CRP (OR 0.63, p = .004). We found there is a 27.3% decrease in the odds of having PVL when CRP value is doubled, keeping all other variables fixed. Permanent vision loss was 3.07 times more likely in the absence of polymyalgia rheumatica and 4.25 times more likely in patients without headaches. Ocular involvement in GCA is common, usually severe with little chance of recovery. Detailed analysis of visual acuity and visual field loss in our PVL cohort adds valuable data to the literature which lacks this information. Older patients with jaw claudication, diplopia, and increased platelets are at higher risk for permanent vision loss, especially when PMR and headache are absent and acute phase reactants lower.

The 46th meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG), held on July 26, 2024, and hosted by Northwestern University at Chicago, brought together a group of neuro-ophthalmologists, researchers, and trainees. This conference served as a forum for presenting research findings, illustrating challenging cases in clinical practice, and discussing them from an expert viewpoint. Key topics included visual outcomes following the treatment of various optic neuropathies, and advancements in diagnostic techniques, including the integration of machine learning. Case demonstrations covered a wide range of afferent and efferent neuro-ophthalmological conditions, primarily related to systemic illnesses and novel treatments. The proceedings aimed to disseminate valuable knowledge and foster further research, offering comprehensive insights into the discussion held during the meeting.

The 16th biennial European Neuro-ophthalmology Society (EUNOS) meeting took place in Rotterdam, Netherlands, from June 3-6, 2024. This landmark event attracted nearly 400 scientists and clinicians worldwide, featuring groundbreaking presentations, symposia, and posters. Notably, it hosted the world's first symposium on Artificial Intelligence (AI) in Neuro-ophthalmology and introduced the inaugural Young Investigators' platform session. Beyond scientific sessions, the event featured social activities that fostered collaboration and community building. These included a memorable dinner at the Rotterdam Zoo and an inaugural morning run. The unforgettable three-day conference concluded with the announcement of EUNOS 2026 in Milan, promising continued advancement and collaboration in neuro-ophthalmology. Overall, EUNOS 2024 set high standards for future meetings , showcasing scientific rigor and innovative approaches and fostering a strong collaborative community.

Cavernous angiomas (CA) affecting cranial nerves (CN) are exceptionally rare. Most reported cases involve the optic nerves, with even fewer occurrences in other CN. In this report, the authors describe a case of an extra-axial CA affecting the oculomotor nerve, which was managed conservatively. The authors also review the outcomes of other therapeutic options based on cases documented in the existing literature. Case report: 36-year-old woman presented to the Emergency Department with a headache in the left temporal region, predominantly at night, along with mild left palpebral ptosis and binocular diplopia of 2 months duration. Brain MRI revealed a lesion exhibiting typical characteristics of CA in the left lateral region of the interpeduncular cistern, in close contact with the left oculomotor nerve. After considering treatment options and in consultation with the patient, a conservative management plan with periodic MRI follow-up was chosen. After 2 years of follow-up, the patient showed favorable progress. Although exceptional, CA should be considered in the differential diagnosis of other more common extra-axial lesions involving CN. The therapeutic management of a CA of the oculomotor nerve remains controversial due to the limited number of cases described in the literature. The authors suggest that for individuals with asymptomatic or mildly symptomatic CA affecting the oculomotor nerve, a conservative treatment approach is the most suitable choice to preserve neurological function. In cases characterized by progressive symptoms, a history of recurrent bleeding, or evidence of lesion enlargement on sequential imaging assessments, total microsurgical resection should be considered.

Erythropoietin (EPO) has demonstrated neuroprotective properties and has been used in small case series to treat methanol optic neuropathy. This study aims to evaluate the effectiveness of EPO. This retrospective case series included data from patients diagnosed with methanol optic neuropathy between November 2022 and December 2023 from two centers in Jeddah, Saudi Arabia. Demographic information, time of consumption of methanol to EPO treatment, and other treatments administered were collected. Vision assessment was performed before and after EPO treatment. A total of 8 male patients were included, with an average age of 38.25 ± 7.15 years. The median duration of the follow-up was 66 days, ranging from 13 to 660 days. The means of vision in the logMAR of both eyes before EPO treatment was 1.98 ± 1.08, which changed to 1.87 ± 0.89 after EPO treatment. Patient's presenting vision before EPO treatment is a significant positive predictor for the vision after treatment with coefficient = 0.782 and 95% CI = 0.349, 0.936. Time to EPO treatment was not statistically significant in defining end vision. Treating methanol optic neuropathy is challenging and time sensitive. In this case series, EPO and adjuvant steroids showed variable effects on visual improvement. Although the vision improved after the treatment, these differences were not statistically significant. Repeat EPO did not give better outcomes. Long-term follow-up is needed to determine the overall impact of EPO treatment.

Leber's hereditary optic neuropathy (LHON) causes subacute visual loss, and, in the acute phase, the optic disc shows hyperemia, peripapillary telangiectasia, and swelling of the retinal nerve fiber layer (RNFL). Rarely, retinal hemorrhage may be present. In this study, we investigated LHON cases with retinal hemorrhage in the acute phase. Among 82 cases (164 eyes) of LHON who visited the Inoue Eye Hospital, retinal hemorrhage was observed in 5 cases (5 eyes). The age at onset was 36 (27-46) years, with 4 male cases. Mitochondrial DNA analysis revealed the presence of the m.11778G > A variant in four patients and the m.14484T > C variant in one patient. There was no medical history and no excessive smoking or alcohol consumption in any of the cases. In all cases, retinal hemorrhages were observed in the RNFL, accompanying the characteristic optic disc findings of LHON. Fluorescein angiography performed in three cases showed no leakage from the optic disc or blood vessels. While rare, the presence of retinal hemorrhage along the RNFL during the acute phase of LHON should be recognized, as it may warrant consideration of alternative diagnoses.

A 54-year-old man with a history of Waldenstrom's macroglobulinemia (WM) presented with sudden-onset blurred vision in the left eye. This case will demonstrate a unique presentation of unilateral optic disc edema that stems from a patient's medical history of WM leading to a rare diagnosis of Bing-Neel Syndrome.

We report a case of painful left abducens nerve palsy that progressed to a left cavernous sinus syndrome (CSS). The initial differential diagnosis was Tolosa-Hunt syndrome (THS), but the clinical course was atypical. Subsequently, perineural invasion (PNI) was demonstrated, after biopsy of a previously occult undiagnosed new cutaneous squamous cell carcinoma (SCC) of the forehead. This case emphasizes the challenges of diagnosing painful ophthalmoplegia when there is an absence of positive neuroimaging or typical findings. The take home message is that a detailed medical history and high index of suspicion are necessary in suspected THS.

A 79-year-old woman presented with a one-week history of left ptosis and diplopia. These symptoms were preceded by an evolving headache, jaw claudication and one episode of transient loss of vision, all of which had resolved by the time of presentation. Examination revealed unilateral complete ophthalmoplegia, ptosis and a minimally reactive pupil. The right eye was unaffected and visual acuity was normal bilaterally. Raised inflammatory markers and positive ultrasound doppler of temporal arteries confirmed the diagnosis of giant cell arteritis (GCA). The patient responded well to oral corticosteroid therapy, showing near resolution of symptoms during 3-week follow-up. Complete ophthalmoplegia and ptosis secondary to multiple cranial nerve (CN) palsies, with sparing of vision, is a rare presentation of GCA as per the literature. In reported cases of CN palsies in GCA, there is often some degree of accompanying visual impairment and rarely are multiple CNs affected. Such a presentation holds a better prognosis as visual loss in this setting is often permanent whereas ocular CN palsies respond well to corticosteroid therapy.

Nonarteritic ischemic optic neuropathy (NAION) is believed to be an ischemic insult to the optic nerve head and is one of the most common acute optic neuropathies of adulthood. Prevention of NAION in the fellow eye has not yet been accomplished. Optical coherence tomography angiography (OCTA) is a new and emerging non-invasive technology that provides microvascular information that complements the structural data. This prospective study is aimed to fill the lacunae in data that is available in the Indian population. We included 36 patients with NAION, their 36 fellow eyes and 37 healthy controls. The peripapillary perfusion index, peripapillary flux, peripapillary retinal nerve fibre layer (RNFL) thickness values and disc volumes of eyes were evaluated. The NAION eyes had lower peripapillary perfusion index, flux and RNFL thickness values in all sectors compared with both the fellow and the healthy control eyes (p = < .05). A statistically significant difference was found in disc volume between control eyes and fellow eyes, which included eyes with disc at risk configuration as well as normal disc configuration. Eyes with disc at risk configuration had a numerically lower disc volume than eyes with normal disc configuration. Fellow eyes overall had numerically lower perfusion index, higher RNFL thickness, and similar flux which was statistically non-significant compared with the healthy eyes. Correlation between the localization of visual field defects and the quadrants showing impairments of perfusion index and peripapillary RNFL were also assessed. These findings may indicate the potential vascular risk factors for the development of NAION in fellow eyes.