optic neuropathy

视神经病变
  • 文章类型: Case Reports
    神经梅毒,梅毒螺旋体传播到中枢神经系统的一种罕见表现,是关键的鉴别诊断,因为如果不治疗,其潜在的严重神经系统影响。我们介绍了一例37岁的妇女,她抱怨双侧视力丧失和头痛,最初是由于特发性颅内高压和不受控制的糖尿病。综合评估最终导致神经梅毒诊断。眼科检查显示糖尿病视网膜病变的多因素视觉症状以及神经梅毒。在诊断时迅速开始治疗并导致视觉症状的改善。这个案例强调了在适当年龄范围内进行梅毒筛查的重要性,即使在低流行地区。将神经梅毒视为潜在的诊断可能会影响患者的预后,并强调需要继续保持警惕以识别该疾病。
    Neurosyphilis, a rare manifestation of Treponema pallidum spreading into the central nervous system, is a critical differential diagnosis due to its potentially severe neurologic effects if left untreated. We present a case of a 37-year-old woman who complained of bilateral vision loss and headaches originally concerning for idiopathic intracranial hypertension and uncontrolled diabetes. Comprehensive evaluations eventually led to a neurosyphilis diagnosis. Ophthalmologic examination revealed multifactorial visual symptoms with diabetic retinopathy contributing alongside neurosyphilis. Treatment was started promptly at the time of diagnosis and resulted in improvement in visual symptoms. This case emphasizes the importance of syphilis screening in appropriate age ranges, even in low-prevalence areas. Recognition of neurosyphilis as a potential diagnosis can impact patient outcomes and highlights the need for continued vigilance to identify the disease.
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  • 文章类型: Journal Article
    背景:在营养不良的情况下,避免性/限制性食物摄入障碍(ARFID)患者通常会出现体重减轻或生长迟缓。然而,ARFID患者可以表现出微量营养素缺乏而不会出现体重减轻.在ARFID患者中,临床医生应警惕微量营养素缺乏及其表现.
    方法:我们报告了一个12岁女孩的ARFID独特病例,他们出现了微量营养素缺乏症,并出现急性视力丧失,先前有夜视障碍史。眼科检查显示干眼症和双侧视神经病变。调查显示严重的维生素A和叶酸缺乏,这解释了她的临床发现。此外,她还被发现含有低维生素B12,铜,和维生素D水平。她从小就有选择性饮食的历史,饮食主要由碳水化合物组成,没有经常摄入肉,乳制品,水果和蔬菜。这不是由体重或身体形象问题驱动的。通过适当的维生素替代和持续的多学科护理,患者的症状明显改善。
    结论:本报告描述了一名出现视觉不适的ARFID患者。在这种情况下,选择性进食行为导致眼干和视神经病变.微量营养素缺乏在发达国家并不常见。当怀疑这些缺陷时,饮食失调,比如ARFID,应该考虑。同样,照顾包括ARFID在内的限制性进食障碍患者的临床医师应熟悉各种微量营养素缺乏的临床表现,并考虑在临床需要时对微量营养素缺乏进行评估和治疗.
    BACKGROUND: Patients with avoidant/restrictive food intake disorder (ARFID) commonly present with loss of weight or faltering growth in the setting of poor nutrition. However, patients with ARFID can present with micronutrient deficiencies without weight loss. In patients with ARFID, clinicians should be vigilant for micronutrient deficiencies and their presentations.
    METHODS: We report a unique case of ARFID in a twelve-year-old girl, who developed micronutrient deficiencies and presented with acute visual loss with a preceding history of impaired night vision. Ophthalmic examination revealed xerophthalmia and bilateral optic neuropathy. Investigations showed severe Vitamin A and folate deficiencies which accounted for her clinical findings. In addition, she was also found to have low Vitamin B12, copper, and Vitamin D levels. She had a history of selective eating from a young age with a diet consisting largely of carbohydrates, with no regular intake of meat, dairy, fruit and vegetables. This was not driven by weight or body image concerns. The patient\'s symptoms improved significantly with appropriate vitamin replacement and continued multidisciplinary care.
    CONCLUSIONS: This report describes a patient with ARFID presenting with visual complaints. In this case, the selective eating behaviours resulted in xeropthalmia and optic neuropathy. Micronutrient deficiencies are uncommon in developed countries. When these deficiencies are suspected, eating disorders, such as ARFID, should be considered. Similarly, clinicians caring for patients with restrictive eating disorders including ARFID should be familiar with the clinical presentations of various micronutrient deficiencies and consider evaluation and treatment for micronutrient deficiencies when clinically indicated.
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  • 文章类型: Journal Article
    背景:最近一个国际共识小组提出了视神经炎的诊断标准和新的分类。我们旨在研究这些诊断标准和分类的临床相关性,在一组因怀疑诊断为视神经炎而住院的患者中。
    方法:我们纳入了2017年至2022年在我们的三级中心因(亚)急性视力丧失提示视神经炎而住院的所有患者。收集症状前3个月内获得的临床和副临床标准,以及最终诊断可能是视神经炎或非视神经炎。我们构建了一个列联表,将基于医生经验的诊断与基于最近提出的标准的诊断进行比较。将基于新分类的视神经炎亚型与基于临床医生经验的亚型进行比较。
    结果:本研究纳入了2557例患者。我们队列中视神经炎的患病率为88.3%。使用新标准进行正确诊断的敏感性和特异性,分别,99.5%和86.7%。拟议的诊断标准过度诊断了4例视神经炎患者,并在1例患者中漏诊。根据最近的分类,特发性视神经炎和临床孤立综合征被重新分类为单一孤立视神经炎。
    结论:在我们因高度怀疑视神经炎的急性和亚急性视神经病变住院的患者中,我们发现最近提出的视神经炎诊断标准和分类与我们的临床实践相关.我们对明确和可能的视神经炎诊断的临床要求的解释可能解释了我们出色的敏感性和明确的视神经炎的高百分比,相对于以前的出版物。中度特异性(86.7%)强调了将所有背景数据纳入诊断的重要性。子群的简化是有用的,但是我们的研究强调了寻找血清阴性NMOSD的适当亚组的复杂性。
    BACKGROUND: A recent international consensus panel proposed diagnostic criteria for optic neuritis and a new classification. We aimed to investigate the clinical relevance of these diagnostic criteria and classification, in a cohort of patients hospitalized for a suspected diagnosis of optic neuritis.
    METHODS: We included all patients hospitalized between 2017 and 2022 in our tertiary center for (sub)acute loss of visual acuity suggestive of optic neuritis. Clinical and paraclinical criteria obtained within the first 3 months of symptoms were collected, as well as the final diagnosis which could be optic neuritis or non-optic neuritis. We constructed a contingency table comparing diagnoses based on physician experience to those based on the recently proposed criteria. The subtypes of optic neuritis based on the new classification were compared to subtypes based on the clinician experience.
    RESULTS: Two hundred fifty-seven patients were included in this study. Prevalence of optic neuritis in our cohort was 88.3%. Sensitivity and specificity of a correct diagnosis using the new criteria were, respectively, 99.5% and 86.7%. The proposed diagnostic criteria overdiagnosed four patients with optic neuritis and missed the diagnosis in one patient. According to the recent classification, idiopathic optic neuritis and clinical isolated syndrome were reclassified mainly as single isolated optic neuritis.
    CONCLUSIONS: In our specific cohort of patients hospitalized for acute and subacute optic neuropathy highly suspect of optic neuritis, we found that recently proposed diagnostic criteria and classification of optic neuritis are relevant for our clinical practice. Our interpretation of clinical requirement for definite and possible optic neuritis diagnosis might explain our excellent sensitivity and our high percentage of definite optic neuritis, relative to previous publications. The moderate specificity (86.7%) underlines the importance to include all contextual data in consideration for the diagnosis. The simplification of subgroups is useful, but our study highlights the complexity to find the adequate subgroup for seronegative NMOSD.
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  • 文章类型: Journal Article
    暴发性特发性颅内高压(IIH)是IIH的快速视力退化表现,已发表的研究有限。本研究对暴发性IIH进行了叙述性回顾,目的是更好地表征暴发性IIH的表现和视觉结果。搜索了SCOPUS和PubMed引用IIH的论文,良性颅内高压,或者假性脑瘤.对文摘进行了视力快速退化的筛选。所有研究都需要同时满足改良的Dandy和暴发性IIH标准。36项研究符合纳入标准。人口统计,治疗,和视觉结果数据收集。案例研究占研究的69%,31%为案例系列。总的来说,报告72例暴发性IIH患者,其中23.6%为儿科,96%为女性。手术干预发生在85%的患者中。11%的患者存在贫血,85.7%的儿科患者患有第六次颅神经麻痹。总之,我们建议以下实践指南来帮助诊断和治疗暴发性IIH患者:1)存在视盘水肿的患者需要紧急视野测试以评估视力丧失;2)出现第六颅神经麻痹的儿科患者应进行全面的眼科检查;3)体重指数正常的患者可发生暴发性IIH;4)贫血应在暴发性IIH的情况下进行测试.由于对本次演讲的最佳治疗机制知之甚少,多机构和国际合作将是未来研究的关键一步。
    Fulminant idiopathic intracranial hypertension (IIH) is a rapid vision-degrading presentation of IIH with limited published studies. This study composed a narrative review of fulminant IIH with the aim of better characterising fulminant IIH presentation and visual outcomes. SCOPUS and PubMed were searched for papers referencing IIH, benign intracranial hypertension, or pseudotumour cerebri. Abstracts were screened for rapid degradation in vision. All studies were required to meet both the modified Dandy and fulminant IIH criteria. Thirty-six studies met the inclusion criteria. Demographics, treatments, and visual outcome data were collected. Case studies made up 69% of the studies and 31% were case series. In total, 72 patients with fulminant IIH were reported, of which 23.6% were paediatric and 96% were female. Surgical intervention occurred in 85% of patients. Anaemia was present in 11% of patients and 85.7% of paediatric patients had a sixth cranial nerve palsy. In conclusion, we propose the following practice guidelines to assist in diagnosing and treating fulminant IIH patients: 1) patients who present with optic disc oedema require urgent visual field testing to evaluate for vision loss; 2) a paediatric patient presenting with a sixth cranial nerve palsy should have a comprehensive eye examination; 3) fulminant IIH can occur in patients with a normal body mass index; and 4) anaemia should be tested for in the setting of fulminant IIH. As little is known about the optimal treatment mechanisms for this presentation, multi-institutional and international collaborations will be a critical step for future research.
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  • 文章类型: Case Reports
    背景:棘球蚴病,通常被称为包虫病,是由细粒棘球蚴引起的人畜共患感染。眼眶区包虫囊肿的发生并不常见,占所有报告的包虫病例的不到1%。本报告详细介绍了眼眶区域肌肉内包虫囊肿导致压迫性视神经病变的独特病例。
    方法:一名来自喀布尔的22岁男性,阿富汗的左眼有五个月的进行性眼球突出史,与过去三周视力逐渐下降有关。左眼表现出向上的反乌托邦,眼运动受限,轻度结膜注射,和化学.通过影像学和组织病理学检查获得诊断。治疗包括手术切除囊肿和延长阿苯达唑治疗。术后病程显示患者病情明显改善,视力恢复。
    结论:尽管它很少,这个案例强调了医生对包虫病的认识和知识的重要性,尤其是那些在流行地区工作的人。它强调了在眼眶肿块的鉴别诊断中包括包虫病的重要性,特别是在流行地区。
    BACKGROUND: Echinococcosis, commonly known as hydatid disease, is a zoonotic infection resulting from the tapeworm Echinococcus granulosus. The occurrence of hydatid cysts in the orbital region is uncommon, representing less than 1% of all reported hydatid cases. This report details a unique case of an intramuscular hydatid cyst in the orbital region that led to compressive optic neuropathy.
    METHODS: A 22-year-old male from Kabul, Afghanistan presented with a five-month history of progressive proptosis in his left eye, associated with a gradual decrease in vision over the past three weeks. The left eye exhibited upward globe dystopia, ocular motility limitation, mild conjunctival injection, and chemosis. Diagnosis was achieved through imaging and histopathological examination. Treatment involves surgical removal of the cyst and prolonged albendazole therapy. The postoperative course showed significant improvement in the patient\'s condition and restoration of his vision.
    CONCLUSIONS: Despite its rarity, this case underscores the importance of awareness and knowledge of hydatid disease among physicians, especially those working in endemic areas. It emphasizes the importance of including hydatid disease in the differential diagnosis of orbital masses, particularly in endemic regions.
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  • 文章类型: Journal Article
    甲醇诱发的视神经病变(MION)是一个关键的公共卫生问题,在社会经济水平较低的人群和酒精摄入受限的地区尤为普遍。MION,以不可逆的视力障碍为特征,产生于甲醇的毒性代谢为甲醛和甲酸,导致线粒体氧化磷酸化抑制,氧化应激,以及随后的神经毒性。发病机制涉及视神经内的轴突和神经胶质细胞变性以及潜在的视网膜损伤。尽管治疗干预措施取得了进展,相当比例的受影响个体忍受持续的视觉后遗症。本研究全面调查了MION的病理生理学,包括甲醇的吸收和代谢,随后的系统性影响,和眼部撞击。组织病理学改变,包括视网膜层和蛋白质的改变,穆勒细胞功能障碍,和视觉症状,经过精心检查,以提供对疾病机制的见解。此外,讨论了预防措施和公共卫生观点,以强调意识和干预策略的重要性。治疗方法,如净化程序,乙醇和fomepizole给药,血液透析,静脉输液,电解质平衡管理,营养治疗,皮质类固醇治疗,促红细胞生成素(EPO)治疗,评估它们在管理MION方面的功效。这一全面审查强调需要提高认识,改进的诊断策略,以及更有效的治疗方法,以减轻MION对全球健康的影响。
    Methanol-induced optic neuropathy (MION) represents a critical public health issue, particularly prevalent in lower socioeconomic populations and regions with restricted alcohol access. MION, characterized by irreversible visual impairment, arises from the toxic metabolization of methanol into formaldehyde and formic acid, leading to mitochondrial oxidative phosphorylation inhibition, oxidative stress, and subsequent neurotoxicity. The pathogenesis involves axonal and glial cell degeneration within the optic nerve and potential retinal damage. Despite advancements in therapeutic interventions, a significant proportion of affected individuals endure persistent visual sequelae. The study comprehensively investigates the pathophysiology of MION, encompassing the absorption and metabolism of methanol, subsequent systemic effects, and ocular impacts. Histopathological changes, including alterations in retinal layers and proteins, Müller cell dysfunction, and visual symptoms, are meticulously examined to provide insights into the disease mechanism. Furthermore, preventive measures and public health perspectives are discussed to highlight the importance of awareness and intervention strategies. Therapeutic approaches, such as decontamination procedures, ethanol and fomepizole administration, hemodialysis, intravenous fluids, electrolyte balance management, nutritional therapy, corticosteroid therapy, and erythropoietin (EPO) treatment, are evaluated for their efficacy in managing MION. This comprehensive review underscores the need for increased awareness, improved diagnostic strategies, and more effective treatments to mitigate the impact of MION on global health.
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  • 文章类型: Case Reports
    神经皮肤黑素细胞增多症(NCM)是一种罕见的,散发性神经外胚层发育不良,其特征是中枢神经系统中存在大量或多个先天性皮肤痣和黑素细胞沉积物。到目前为止,未报道,我们描述了来自印度的NCM伴视神经病变和脊髓黑色素瘤的病例。一位20岁的女士头痛和呕吐3个月,随后连续出现严重的无痛性视力障碍。视力是指在正常眼底的两只眼睛中在1m距离处计数手指。没有脊髓受累的症状。临床检查显示面部和身体上有多个小到大的黑素细胞痣。肌肉力量正常。肌腱反射被夸大了。视觉诱发电位显示双侧P100潜伏期延长(右眼-144毫秒;左眼-151毫秒)。脑部MRI显示脑干软脑膜增强,小脑,无视神经受累的动眼神经和面外神经复合体。MRI脊柱显示广泛的背侧胸髓硬膜外病变沿整个胸髓段延伸,背侧脊髓受压。正电子发射断层扫描(PET)成像显示沿脊髓D1-D12水平的氟脱氧葡萄糖F18(FDG)亲和力。脊髓病变活检提示脑膜黑色素瘤。在这里,我们记录了一例罕见的晚发性NCM,伴有颅内脑膜浸润和无症状的脊髓大硬膜外病变,扩大其表型谱。NCM中的视神经病变尚未早期报道。在NCM中,建议对大脑和脊柱进行定期筛查以进行早期预测和病变识别。
    Neurocutaneous melanocytosis (NCM) is a rare, sporadic neuroectodermal dysplasia characterized by the presence of large or multiple congenital cutaneous nevi and melanocytic deposits in the central nervous system. Hitherto, unreported we describe a case of NCM with optic neuropathy and spinal cord melanoma from India. A 20 year-old-lady had headache and vomiting for 3 months followed by consecutive profound painless visual impairment. Visual acuity was counting of fingers at 1 m distance in both eyes with normal fundus. There were no symptoms of spinal cord involvement. Clinical examination showed multiple small to large melanocytic nevi over the face and body. Muscle power was normal. Tendon reflexes were exaggerated. Visual evoked potential showed bilateral prolonged P100 latency (Right eye - 144 msec; Left eye - 151 msec). Brain MRI revealed leptomeningeal enhancement of brainstem, cerebellum, oculomotor and facial-abducent nerve complex without optic nerve involvement. MRI spine showed extensive dorsal thoracic cord epidural lesion extending along the entire thoracic cord segment with dorsal cord compression. Positron Emission Tomography (PET) imaging showed Fludeoxyglucose F18 (FDG) avidity along D1-D12 levels of spinal cord. Biopsy from the cord lesion was suggestive of meningeal melanoma. Here we document a rare case of late onset NCM with intracranial meningeal infiltration and asymptomatic large epidural lesion of spinal cord, expanding its phenotypic spectrum. Optic neuropathy in NCM has not been reported earlier. Periodic screening of brain and spine is recommended for early prognostication and lesion identification in NCM.
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  • 文章类型: Case Reports
    进行性核上性麻痹(PSP),也被称为斯蒂尔-理查森-奥尔谢夫斯基综合征,是一种罕见的神经退行性疾病,其特征是各种运动和神经眼科症状。我们介绍了一个73岁的男性患者,有2型糖尿病和高血压病史,为步态障碍咨询的人,四肢颤抖,难以控制共轭凝视。体检时,发现与PSP一致,包括低omimia,肌肉僵硬,异常动作。帕金森病的最初误诊和随后的左旋多巴给药突出了在复杂神经系统疾病中准确诊断的重要性。该临床病例强调需要对神经眼科症状和体征进行彻底评估,以确保采用适当的治疗方法并提高患者的生活质量。
    Progressive Supranuclear Palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a rare neurodegenerative disease characterized by a variety of motor and neuro-ophthalmological symptoms. We present the case of a 73-year-old male patient with a history of type 2 diabetes and high blood pressure, who consulted for gait disorders, tremors in the extremities, and difficulty controlling conjugate gaze. During physical examination, findings consistent with PSP were noted, including hypomimia, muscle rigidity, and abnormal movements. The initial misdiagnosis of Parkinson\'s disease and subsequent administration of levodopa highlight the importance of accurate diagnosis in complex neurological conditions. This clinical case highlights the need for a thorough evaluation of neuro-ophthalmological symptoms and signs to ensure an appropriate therapeutic approach and improve the quality of life of patients.
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  • 文章类型: Case Reports
    大麻是美国最常用的联邦非法药物。急性使用大麻与一些心血管和神经心理学的不良影响有关。滥用大麻的眼部并发症非常罕见。在这里,我们报道了吸食大麻后双侧视神经病变的首例报道.一名28岁的男子在吸食大麻8小时后突然出现双侧下视野模糊,来到急诊室。他的最佳矫正视力在右眼为20/30,在左眼为20/20。眼底检查显示,双眼视盘边缘模糊,右眼有裂片出血。检测到双侧下视野缺损,右侧严重程度更高。光学相干断层扫描证实了双侧视神经病变的诊断。尿液药物筛选试验对四氢大麻酚呈阳性,这是大麻素的主要活性成分。其余神经系统检查及影像学检查均正常。患者接受静脉注射皮质类固醇和抗血小板药物治疗。他的双眼视力恢复到20/20,在6个月的随访中完全解决了现场缺陷。滥用大麻后的视神经病变是不寻常的。我们报告的结果强调需要意识到大麻相关的视神经病变是大麻中毒的眼部不良反应的一部分。
    Marijuana is the most commonly used federally illegal drug in the United States. Acute marijuana use is associated with several cardiovascular and neuropsychological adverse effects. Ocular complications of marijuana abuse are very rare. Herein, we present the first report of bilateral optic neuropathy following smoking marijuana. A 28-year-old man presented to the emergency room with sudden onset of bilateral blurring of the inferior visual field 8 h after smoking marijuana. His best-corrected visual acuity was 20/30 in the right eye and 20/20 in the left eye. Fundus examination revealed blurring of the optic disc margins in both eyes and a splinter haemorrhage in the right eye. Bilateral inferior visual field defects were detected with greater severity on the right side. Optical coherence tomography confirmed the diagnosis of bilateral optic neuropathy. A urine drug screen test was positive for tetrahydrocannabinol, which is the primary active ingredient in cannabinoids. The rest of the neurological examination and imaging were normal. The patient was treated with intravenous corticosteroids and an anti-platelet drug. His vision recovered to 20/20 in both eyes, with complete resolution of the field defect over a follow-up of 6 months. Optic neuropathy following marijuana abuse is unusual. The results of our report emphasise the need for awareness of marijuana-associated optic neuropathy as part of ocular adverse effects of marijuana intoxication.
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  • 文章类型: Journal Article
    目的:非动脉炎性前部缺血性视神经病变(NAION)的评估和治疗缺乏标准化指南。本研究旨在调查神经眼科医生在典型NAION病例的评估和管理中的现实实践。
    方法:全国调查,在2019年至2021年之间进行,涉及所有执业神经眼科医生。一份结构化问卷评估了他们对NAION风险因素评估和治疗的方法,有19个关于危险因素的问题和6个关于治疗和预防同眼受累的问题。
    结果:37名神经眼科医生中有36名参加。大多数医生推荐患者评估以下危险因素:阻塞性睡眠呼吸暂停(83.3%),糖尿病(83.3%),高血压(77.7%),血脂异常(72.2%),和视盘玻璃疣(38.8%)。然而,所推荐的诊断性检查的选择存在相当大的差异.此外,近47%的人建议进行栓塞检查.关于治疗,大多数(91%)不推荐NAION的常规治疗,尽管在16.7%中,偶尔使用大剂量皮质类固醇.阿司匹林二级预防(80.6%),戒烟建议(86.1%),和建议男性使用勃起功能障碍药物(80.6%)是常见的建议。
    结论:虽然与NAION相关的危险因素报告良好,评估这些危险因素的测试缺乏统一性。大多数神经眼科医生都认为,没有必要对NAION进行常规治疗,但不是一致的。未来为NAION后的工作和管理建议制定共识指南的研究可能有助于发现和管理可预防的风险因素。
    OBJECTIVE: The evaluation and management of Nonarteritic Anterior Ischemic Optic Neuropathy (NAION) lacks standardized guidelines. This study aimed to investigate the real-world practices of neuro-ophthalmologists in the evaluation and management of typical NAION cases.
    METHODS: A national survey, conducted between 2019 and 2021, involved all practicing neuro-ophthalmologists. A structured questionnaire assessed their approach to risk factor evaluation and treatment of NAION, with 19 questions about risk factors and six questions concerning treatment and prevention of fellow-eye involvement.
    RESULTS: Thirty-six out of 37 neuro-ophthalmologists participated. Most physicians referred patients for evaluation of the following risk factors: obstructive sleep apnea (83.3%), diabetes mellitus (83.3%), hypertension (77.7%), dyslipidemia (72.2%), and optic disc drusen (38.8%). However, there was considerable variation in the choice of diagnostic tests recommended. Furthermore, nearly 47% recommended an embolism workup. Regarding treatment, the majority (91%) did not recommend routine treatment for NAION, although in 16.7%, high-dose corticosteroids were occasionally prescribed. Secondary prevention with aspirin (80.6%), smoking cessation advice (86.1%), and advising against erectile dysfunction medications for men (80.6%) were common recommendations.
    CONCLUSIONS: While the risk factors associated with NAION are well-reported, there is a lack of uniformity on which tests should be ordered to evaluate these risk factors. Most neuro-ophthalmologists concur that routine treatment for NAION is not warranted, but not unanimously. Future studies to develop a consensus guideline for post-NAION work-up and management recommendations may assist in the detection and management of preventable risk factors.
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