optic neuropathy

视神经病变
  • 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: 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
    嗜酸性粒细胞具有广泛的促凝血作用。在日常实践中,嗜酸性粒细胞相关的心血管毒性包括心内膜损伤,嗜酸性血管炎和动脉或静脉血栓形成。在这里,我们旨在报告无法解释的眼部血管表现和嗜酸性粒细胞增多患者的临床特征和治疗结果。
    我们进行了回顾,多中心,对嗜酸性粒细胞增多(≥0.5x109/L)并伴有眼部血管表现的患者的观察性研究和文献综述,这些患者与潜在的嗜酸性粒细胞疾病无关,但没有其他原因导致眼部表现.
    纳入57例患者(20例来自观察性研究,37例来自文献综述)。眼血管特征是34例(59%)患者嗜酸性粒细胞相关疾病的最初表现,包括29例视网膜中央动脉阻塞,视网膜六分支动脉阻塞,五个视网膜中央静脉阻塞,两个分支视网膜静脉阻塞,七种视网膜血管炎,两次视网膜血管痉挛,12Purtscher的视网膜病变,13个前部缺血性视神经病变和两个后部缺血性视神经病变。眼血管表现开始时的中位数[IQR]绝对嗜酸性粒细胞计数为3.5[1.7-7.8]x109/L。潜在的嗜酸性粒细胞相关疾病包括嗜酸性肉芽肿伴多血管炎(n=32),克隆性高嗜酸性粒细胞综合征(HES)(n=1),特发性HES(n=13),淋巴细胞性HES(n=2),药物不良反应(n=3),寄生虫病(n=2),结节性多动脉炎(n=1),IgG4相关疾病(n=1),嗜酸粒细胞性筋膜炎(n=1)和原发性硬化性胆管炎(n=1)。其他与嗜酸性粒细胞增多相关的眼外动脉或静脉血栓在4例(7%)和9例(16%)患者中报告,分别。视力预后较差:只有8例(10%)患者实现了眼科症状的完全恢复。在中位随访10.5[1-18]个月后,1例(3%)眼血管表现复发,3例患者(10%)有其他血管症状复发(2例患者有深静脉血栓形成,1例患者有肺栓塞).在复发的时候,所有病例的嗜酸性粒细胞绝对计数均高于0.5x109/L(n=4)。
    本研究通过增加眼血管表现,拓宽了与嗜酸性粒细胞增多相关的血管表现的范围。在有眼科血管表现和嗜酸性粒细胞增多的患者中,应积极治疗基础病理(和血液计数正常化)。
    UNASSIGNED: Eosinophils have widespread procoagulant effects. In daily practice, eosinophil-related cardiovascular toxicity consists of endomyocardial damage, eosinophilic vasculitis and arterial or venous thrombosis. Here we aim to report on the clinical features and treatment outcomes of patients with unexplained ophthalmic vascular manifestations and eosinophilia.
    UNASSIGNED: We conducted a retrospective, multicenter, observational study and a literature review of patients with eosinophilia (≥0.5 x109/L) and concomitant ophthalmic vascular manifestations independent of the underlying eosinophilic disease but with no alternative cause for ophthalmic manifestations.
    UNASSIGNED: Fifty-seven patients were included (20 from the observational study and 37 from the literature review). Ophthalmic vascular features were the initial manifestation of eosinophil-related disease in 34 (59%) patients and consisted of 29 central retinal artery occlusions, six branch retinal artery occlusions, five central retinal vein occlusions, two branch retinal vein occlusions, seven retinal vasculitides, two retinal vasospasms, 12 Purtscher\'s retinopathies, 13 anterior ischemic optic neuropathies and two posterior ischemic optic neuropathies. The median [IQR] absolute eosinophil count at onset of ophthalmic vascular manifestations was 3.5 [1.7-7.8] x109/L. Underlying eosinophil-related diseases included eosinophilic granulomatosis with polyangiitis (n=32), clonal hypereosinophilic syndrome (HES) (n=1), idiopathic HES (n=13), lymphocytic HES (n=2), adverse drug reactions (n=3), parasitosis (n=2), polyarteritis nodosa (n=1), IgG4-related disease (n=1), eosinophilic fasciitis (n=1) and primary sclerosing cholangitis (n=1). Other extra-ophthalmologic arterial or venous thromboses related to eosinophilia were reported in four (7%) and nine (16%) patients, respectively. Visual prognosis was poor: only eight (10%) patients achieved full recovery of ophthalmologic symptoms. After a median follow-up of 10.5 [1-18] months, one patient (3%) had a recurrence of an ophthalmic vascular manifestation, and three patients (10%) had a recurrence of other vascular symptoms (deep vein thrombosis in two and pulmonary embolism in one patient). At the time of recurrence, absolute eosinophil counts were above 0.5 x109/L in all cases (n=4).
    UNASSIGNED: This study broadens the spectrum of vascular manifestations associated with hypereosinophilia by adding ophthalmic vascular manifestations. In patients with ophthalmological vascular manifestations and hypereosinophilia, aggressive treatment of the underlying pathology (and normalization of blood count) should be implemented.
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  • 文章类型: Case Reports
    威尔逊病(WD)是一种罕见的遗传性疾病,临床表现广泛,包括肝,神经学,和精神症状。迄今为止,有5例(包括我们的病例)是由WD引起的视神经病变。
    一名15岁的女性因神经系统症状出现在急诊科。患者出现意识模糊,但生命体征保持稳定,体检都正常。腹部超声和轴向脑计算机断层扫描(CT)扫描均正常。患者的神经系统状况和实验室检查结果提示诊断为WD。2天后,患者经历了突然的双侧失明。病人的病情迅速恶化,随后被转诊至ICU。
    这种疾病的发病率因种族而异,在东亚人群中患病率较高。由于症状的不同表现,诊断可能具有挑战性,但对于出现急性肝炎和/或神经系统异常的患者,将WD作为鉴别诊断是很重要的.
    应教育医疗保健专业人员了解WD的各种临床表现,以帮助早期识别和诊断疾病。
    UNASSIGNED: Wilson disease (WD) is a rare genetic disorder with a wide range of clinical manifestations, including hepatic, neurologic, and psychiatric symptoms. To date, there have been five cases (including our case) representing optic neuropathy caused by WD.
    UNASSIGNED: A 15-year-old female presented to the emergency department with neurological symptoms. The patient exhibited confusion but maintained stable vital signs, and physical examinations were all normal. Abdominal ultrasound and axial brain computed tomography (CT) scan were both normal. The patient\'s neurological condition and laboratory test results suggested diagnoses of WD. After 2 days, the patient experienced sudden bilateral blindness. The patient\'s condition deteriorated rapidly, and was subsequently referred to the ICU.
    UNASSIGNED: The incidence of the disease varies by ethnicity, with a higher prevalence in Eastern Asian populations. Diagnosis can be challenging due to the diverse presentation of symptoms, but it is important to consider WD as a differential diagnosis in patients presenting with acute hepatitis and/or neurologic abnormalities.
    UNASSIGNED: Healthcare professionals should be educated about the diverse clinical manifestations of WD to help in early recognition and diagnosis of the disease.
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  • 文章类型: Review
    Wilson病(WD)是铜代谢的常染色体隐性遗传疾病。WD的临床表现复杂多变,Kayser-Fleischer环(K-F环)和向日葵白内障是最常见的眼部发现。视力障碍在WD患者中很少见。我们报告了一名17岁女性,患有与WD相关的双侧视神经萎缩,并总结了先前报道的WD视神经病变病例的临床特征。临床医生应该意识到WD是视神经病变的罕见原因,并且可能需要认识和筛查WD患者的视神经病变。
    Wilson disease (WD) is an autosomal recessive disorder of copper metabolism. The clinical manifestations of WD are complex and variable, with Kayser-Fleischer ring (K-F ring) and the sunflower cataract being the most common ocular findings. Visual impairment is rare in patients with WD. We report the case of a 17-year-old female with bilateral optic atrophy associated with WD and summarize the clinical features of previously reported cases of optic neuropathy in WD, Clinicians should be aware that WD is a rare cause of optic neuropathy and that optic neuropathy in patients with WD may need to be recognized and screened.
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  • 文章类型: Journal Article
    视神经病变,如青光眼,是全球不可逆失明的一些主要原因。已经有很多关于潜在疗法的研究,这些疗法可以减弱甚至减少导致视网膜神经节细胞(RGC)丢失的病理途径的影响。近年来,维生素B3(烟酰胺)由于其在增强RGC的线粒体代谢中的基本作用,已成为这些神经退行性疾病的可行选择。本文就维生素B3对视网膜细胞的影响进行综述,尤其是当处于视神经病变的功能失调状态时,尤其是青光眼.本文还总结了维生素B3在这些视神经病变中的临床作用的现有和未来研究。并确定有关其剂量的适当建议,功效,和最终的副作用。
    Optic neuropathies, such as glaucoma, are some of the leading causes of irreversible blindness worldwide. There has been a lot of research for potential therapies that could attenuate and even reduce the impact of the pathological pathways that lead to the loss of retinal ganglion cells (RGCs). In recent years, vitamin B3 (nicotinamide) has gained some interest as a viable option for these neurodegenerative diseases due to its fundamental role in enhancing the mitochondria metabolism of the RGCs. This review focuses on elucidating the impact of vitamin B3 on retinal cells, especially when in a dysfunctional state like what happens in optic neuropathies, especially glaucoma. This review also summarizes the existing and future research on the clinical effects of vitamin B3 in these optic neuropathies, and determines appropriate recommendations regarding its dosing, efficacy, and eventual side effects.
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  • 文章类型: Case Reports
    一名29岁女性,无家族史,表现为双侧进行性视力模糊。她的症状出现在12岁时,此后视力从双侧0.6下降到0.2,临界闪烁频率和双侧中央暗点降低。她没有相对传入的瞳孔缺损。胃镜检查显示没有明显的椎间盘充血,萎缩,或乳头周围毛细血管扩张血管。视网膜神经纤维层在每只眼睛的光学相干断层扫描上表现正常;然而,两只眼睛都观察到交叉区的丢失和中央凹的椭圆体区的破坏。多焦视网膜电图显示两个黄斑区域的振幅均降低。线粒体脱氧核糖核酸分析确定m.14502T>C突变,导致Leber遗传性视神经病变(LHON)的主要突变之一。尽管存在明显的LHON突变,然而,临床诊断为隐匿性黄斑营养不良。以前只有5例病例报告,所有这些都是零星的,其中详细介绍了m.14502T>C突变的临床特征。m.14502T>C表型与其他主要突变的表型有一定的一致性,包括年轻的发病,双侧进行性视力障碍,和典型的LHON眼底。然而,m.14502T>C单独具有极低的外显率,其表型可能是极小的或亚临床的,从我们的案例中可以看出。由于对m.14502T>C突变的临床过程知之甚少,因此LHON表型可能出现在生命的后期。此外,m.14502T>C可以作为修饰基因,这改变了其他共存的主要LHON突变的表型,包括外显率和疾病的严重程度,通过协同效应。
    A 29-year-old female with no family history presented with bilateral progressive blurred vision. Her symptoms appeared at 12-years-old and her visual acuity had since deteriorated from 0.6 to 0.2 bilaterally with decreased critical flicker frequency and bilateral central scotomas. She did not have a relative afferent pupillary defect. Fundoscopy revealed no distinct disc hyperaemia, atrophy, or peripapillary telangiectatic vessels. The retinal nerve fibre layer appeared normal on optical coherence tomography in each eye; however, loss of the interdigitation zone and the disruption of the ellipsoid zone at the fovea were observed in both eyes. Multifocal electroretinography revealed decreased amplitudes at both macula regions. Mitochondrial deoxyribonucleic acid analysis identified an m.14502T>C mutation, one of the primary mutations causing Leber\'s hereditary optic neuropathy (LHON). Despite the presence of a marked LHON mutation, however, she was clinically diagnosed as having an occult macular dystrophy. There have only been five previous case reports, all of which were sporadic, which detail the clinical characteristics of the m.14502T>C mutation. The m.14502T>C phenotype is somewhat consistent with that of the other major mutations, including young onset, bilateral progressive visual impairment, and a typical LHON fundus. Nevertheless, m.14502T>C alone has an extremely low penetrance and its phenotype may be minimal or subclinical, as seen in our case. Since little is known about the clinical course of the m.14502T>C mutation it may be possible that the LHON phenotype may appear in later stages of life. Moreover, m.14502T>C may function as a modifier gene, which alters the phenotype of other coexisting major LHON mutations, including penetrance and the severity of the disease, through synergistic effects.
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  • 文章类型: Journal Article
    在过去的二十年里,电刺激(ES)已在患有各种眼病的患者中进行了测试,并在色素性视网膜炎和视神经病变中显示出巨大的治疗潜力。然而,目前ES在眼科的临床应用有限。一方面,ES协议的优化和标准化仍然是一个未满足的需求。另一方面,对潜在机制的理解不足阻碍了临床开发。
    已经进行了许多实验研究,以确定ES在眼部疾病中的治疗潜力,并探索相关的细胞和分子机制。在这次审查中,我们总结了与眼疾病中ES的细胞和组织反应相关的体外和体内证据。我们强调了ES可能利用的几种途径对患病的视网膜施加影响。
    ES在视网膜退行性疾病中的治疗作用可能通过防止神经元凋亡,促进神经元再生,增加Müller细胞中神经营养因子的产生,抑制小胶质细胞激活,增强视网膜血流,调节大脑可塑性。建议未来的研究分析特定视网膜细胞的变化,以优化治疗参数并选择最适合目标疾病的ES递送方法。
    UNASSIGNED: In the last two decades, electrical stimulation (ES) has been tested in patients with various eye diseases and shows great treatment potential in retinitis pigmentosa and optic neuropathy. However, the clinical application of ES in ophthalmology is currently limited. On the one hand, optimization and standardization of ES protocols is still an unmet need. On the other hand, poor understanding of the underlying mechanisms has hindered clinical exploitation.
    UNASSIGNED: Numerous experimental studies have been conducted to identify the treatment potential of ES in eye diseases and to explore the related cellular and molecular mechanisms. In this review, we summarized the in vitro and in vivo evidence related to cellular and tissue response to ES in eye diseases. We highlighted several pathways that may be utilized by ES to impose its effects on the diseased retina.
    UNASSIGNED: Therapeutic effect of ES in retinal degenerative diseases might through preventing neuronal apoptosis, promoting neuronal regeneration, increasing neurotrophic factors production in Müller cells, inhibiting microglial activation, enhancing retinal blood flow, and modulating brain plasticity. Future studies are suggested to analyse changes in specific retinal cells for optimizing the treatment parameters and choosing the best fit ES delivery method in target diseases.
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  • 文章类型: Case Reports
    眼科和神经系统受累是CLL的罕见并发症,文献中报道的病例很少。我们报告了一例CLL伴视神经白血病浸润的病例,并回顾了有关治疗和结局的文献。一名接受严重预处理的CLL患者因进行性眼痛被送往我们医院,并被发现患有浸润性视神经炎。CSF分析证实与CLL有关。在用R-CHOP和大剂量甲氨蝶呤进行全身治疗后,鞘内注射阿糖胞苷和氢化可的松,她经历了显着改善,并出院回家。鉴于CLL中眼科的罕见性,我们回顾了之前报道的所有15例CLL伴视神经病变为中枢神经系统受累的首发表现,并讨论了所使用的治疗方案范围和各自的结局.
    Ophthalmic and neurologic involvement are rare complications of CLL, with few cases reported in the literature. We report a case of CLL with leukemic infiltration of the optic nerve and review of literature focusing on management and outcomes. A patient with heavily pretreated CLL presented to our hospital with progressive eye pain and was found to have infiltrative optic neuritis. CSF analysis confirmed involvement with CLL. After systemic treatment with R-CHOP and high-dose methotrexate, along with intrathecal cytarabine and hydrocortisone, she experienced significant improvement and was discharged home. Given the rarity of ophthalmic involvement in CLL, we reviewed all 15 previously reported cases of CLL with optic neuropathy as the first manifestation of CNS involvement and discussed the range of treatment options used and their respective outcomes.
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