Ultrasonic Elastography Evaluation in Optic Neuritis

Purpose: In the present study, we attempted to determine whether ultrasonic elastography (USE) evaluation can be used in a diagnosis of optic neuritis (ON). Materials and Methods: Thirteen patients who each had one normal eye and one eye with a diagnosis of ON were included in the study. Ultrasonography (US) and USE examinations were performed on the affected and non-affected eyes of all participants. Optic nerve and adjacent fat tissue regions at the same depth were selected, and USE measurements were obtained. The optic nerve diameter was measured in both normal and affected eyes. Results: The mean USE values for the optic nerve were 2.58 ± 0.50 m/s in ON eyes and 1.91 ± 0.39 m/s in normal eyes (p = 0.001). The mean USE values for the optic-nerve adjacent tissue were 2.26 ± 0.45 m/s in ON eyes and 1.77 ± 0.22 m/s in normal eyes (p = 0.001). The mean optic-nerve diameter was 3.80 ± 1.09 mm in ON eyes and 3.28 ± 0.98 mm in normal eyes (p = 0.005). Conclusions: USE may be considered an accessible, safe technique for the detection of significant optic-nerve tissue stiffness in ON and may be used an adjunctive tool for confirming this diagnosis.     

视神经管减压术治疗间接性视神经挫伤30例

目的评价视神经管减压术治疗视神经挫伤的疗效和影响疗效的因素。方法手术治疗限定于外伤后1月内,并且无严重颅脑损伤、无严重全身病患者。手术方式采用经眶—筛窦—蝶窦视神经管减压术,全麻或局麻下施行。术前、术后常规应用大剂量糖皮质激素和神经营养药物治疗。结果30例患者中19例术后视力有不同程度提高,有效率为63%(19/30),24例术前无光感者,13例术后1周以后恢复光感以上的视力,有效率为54%(13/24),6例术前有光感～0.04,术后视力提高显著,有效率100%。手术病程15d以内者优于15d以上者。结论对严重的视神经挫伤患者应尽早行视神经管减压术,手术的效果与患者手术前的视力状况及病程的长短有关,术前、术后大剂量糖皮质激素及神经营养药物的应用对提高有效率也尤为必要。     

骨管部视神经挫伤的诊断及治疗体会

目的探讨骨管部视神经挫伤早期诊断及治疗。方法16例患者均进行早期的CT或MRI检查，确诊后给予皮质类固醇及脱水剂等治疗，对其中4例进行视神经管减压术。结果3日内确诊者视力恢复至0．1以上的9例，3～7日确诊者视力恢复至0．1以上的3例，7例日后确诊者，视力恢复至0．1以上的0例，7日后确诊者，视力恢复至0．1以上的0例。结论骨管部视神经挫伤应避免漏诊，预后与早期发现并合适的治疗手段密切相关。     

Multicentric Glioblastoma Multiforme Mimicking Optic Neuritis

A 49-year-old previously healthy woman presented with acute painless visual loss in the right eye, a right relative afferent pupillary defect, and a normal fundus examination. She was diagnosed with retrobulbar “optic neuritis” and given a course of intravenous steroids. Despite treatment, however, she continued to lose vision and serial visual field testing confirmed a junctional scotoma in the fellow eye. Cranial magnetic resonance imaging (MRI) showed a mass at the junction between the right optic nerve and the anterior aspect of the chiasm and a right frontal lesion that proved to be multicentric glioblastoma multiforme. Clinicians should be aware of the possibility of aggressive neoplasm in the differential diagnosis of retrobulbar optic neuritis.     

Morphological changes in the visual pathway induced by experimental glaucoma in Japanese monkeys

Glaucoma, an optic neuropathy, is the leading cause of world blindness. In this condition, the damage extends from the retina to the visual center in the brain, although the primary region of damage is thought to be the optic nerve head (ONH), with the lateral geniculate nucleus (LGN) being secondarily affected. We investigated time-dependent alterations in the ONH, the optic nerve (ON), and the LGN after intraocular pressure (IOP) elevation in Japanese monkeys (a species more similar to humans than other macaque species). Nine Japanese monkeys, each with an experimental glaucomatous left eye, and two naive monkeys were studied. Ocular-testing sessions (including IOP measurement and fundus photography) were held weekly. Eyes and brains were enucleated at 2-48 weeks after IOP elevation, and alterations in ONs and LGN were evaluated. The IOP of the treated eyes was monitored periodically and found to be elevated continuously throughout the observation period in each monkey. The ONH of the glaucomatous eyes exhibited time-dependent deep cupping and thinning of the rim area from 2 weeks after the IOP elevation. Loss of axons and a decrease in the area of ON were first observed at 4 and 28 weeks, respectively. Neuronal loss was first observed at 2 weeks in layers 1 and 2 of LGN [magnocellular (M)-layer] and at 12 weeks in layers 3-6 of LGN [parvocellular (P)-layer]. Neuronal shrinkage was first observed at 2 weeks in all layers in LGN. These findings indicate that in Japanese monkeys, damage to neurons in LGN can be detected in the early phase (first few weeks) after an IOP elevation, as can damage to ONH.     

Optic Nerve Meningioma

An obese white woman presented with a 10-month history of progressive loss of vision in the right eye associated with a moderate proptosis. Examination of the fundus revealed marked congestion of the retinal vasculature with dilation of both the arteries and the veins. Numerous blot hemorrhages were scattered throughout the retina. The retina was elevated and appeared to be displaced anteriorly by solid tissue, but B-scan ultrasonography demonstrated only a serous detachment of a thickened, markedly convoluted retina. A diagnosis of optic nerve meningioma was considered, but definitive treatment was deferred because of medical problems. One month later the patient returned with a blind, painful right eye associated with neovascular glaucoma. High resolution CT coronal sections of the orbit showed generalized thickening of the optic nerve. The blind eye was enucleated facilitating exposure of the optic nerve which was found to be diffusely thickened by meningioma. The orbital portion of the optic nerve was subsequently removed and meningioma was demonstrated along its entire length to the bony optic canal. A transfrontal craniotomy was later performed to rid the patient of residual tumor.     

Retinitis pigmentosa,mental retardation,marked short stature,and brachydactyly in two sibs

We present two siblings with retinitis pigmentosa, mental retardation, markedly short stature, and brachydactyly. This association of clinical findings appears to be distinct from previously described syndromes and seems to represent the pleiotropic effects of a single autosomal recessive gene.     

视神经束内营养小动脉硬化引起视神经纤维形态学变化的组织病理学研究

目的研究颈内动脉粥样硬化和视神经干小动脉硬化与视神经萎缩的关系。方法收集猝死于心脑血管疾病的60～83岁老年人的双侧脑组织标本30例（60个）,取颈内动脉和相应的视神经切片行苏木精-伊红染色和Wegert染色,进行病理解剖学研究。结果颈内动脉有粥样硬化改变者55个,占91.7%;视神经内小动脉有硬化改变者45个,占75%。其中纤维增生型15个,占25.0%;纤维斑块型和粥样斑块型28个,占46.6%;钙化斑块型（复合斑块型）17个,占28.4%。视神经束内小动脉有轻度硬化改变者35个,占63.7%;中度改变者14个,占25.3%;重度硬化改变并阻塞小动脉而致视神经萎缩者6个,占11%。结论颈内动脉硬化可同时伴视神经小动脉硬化,小动脉硬化可致视神经缺血萎缩,是引起老年人视野缺损的原因之一。     

Management of sight-threatening optic nerve sheath meningioma with fractionated stereotactic radiotherapy

BACKGROUND: Previous management of optic nerve sheath meningioma included conservative observation, surgery, radiosurgery and conventional radiotherapy. All carried significant risk, either of visual loss or damage to adjacent structures. Fractionated stereotactic radiotherapy appears to have few side-effects and may preserve or improve vision. To date only three groups have published their experience with this modality. METHODS: A retrospective review of patients with optic nerve sheath meningioma treated with stereotactic radiotherapy in an academic complex was conducted. Patients with greater than 18 months follow up and no previous related surgery were eligible for inclusion. Patients received an average of 43.5 Gy to the tumour in 26 fractions. Case records from the treating institutions as well as those of the referring ophthalmologist were analysed. The limited literature on the subject was reviewed in order to draw conclusions relevant to contemporary patient management. RESULTS: Four patients had undergone follow up for over 18 months. Prior to treatment all had exhibited progressive loss of visual function. In all cases visual function remained stable or improved at the last assessment. Side-effects included radiologically detected cerebral changes in one patient and transient hair loss in one patient. CONCLUSIONS: This treatment modality represents a promising refinement of previous treatment options. It may be offered to patients who demonstrate progressive loss of visual function caused by optic nerve sheath meningioma. It offers significant advantages over other currently available therapeutic options but its use should be tempered by the knowledge that long-term side-effects are yet to be determined.     

Syndrome of myelinated retinal nerve fibers, myopia, and amblyopia: a review

Myelinated retinal nerve fibers are developmental anomalies that are present in approximately 1% of all eyes. They may be associated with ipsilateral high myopia and amblyopia, and also may occur in association with various ocular and systemic abnormalities. Lesions may be congenital or acquired, static or dynamic, and can regress in the setting of several described conditions. The pathogenesis is thought to be due to the presence of ectopic oligodendrocyte-like cells in the retina as a result of a development or acquired insult. Visual acuity is typically poor to begin with, and the improvement of visual function in patients undergoing occlusion therapy has been, in general, limited. We discuss a series of 11 patients seen at the Cole Eye Institute with the condition of myelinated retinal nerve fibers, myopia, and amblyopia and describe the clinical characteristics. Three patients also had optic nerve dysplasia and a discussion of the literature related to this association was also performed.     

Longterm follow‐up of children with traumatic optic nerve avulsion

Purpose: We report the longterm follow‐up of children with optic nerve avulsion (ONA) caused by traumatic events. The remarkable differences in courses and outcomes may elucidate the spectrum of ONA‐associated symptoms and injuries. Methods: During the last 15 years, three children with ONA were referred to our department. These cases are presented with special attention to their longterm follow‐up. Results: Two patients suffered from complete ONA after head injury. The third patient presented with partial ONA caused by a bicycle accident. Longterm follow‐up varied between 7 and 15 years. In the first patient, a pale swollen retina without any visible retinal vasculature was observed early in the course of follow‐up. The retina later completely detached. In the second patient, extended fibroglial scarring occurred and an extremely large epiretinal membrane formed and was finally released spontaneously into the vitreous. The third patient developed only mild fibroglial scarring and retinal pigment epithelium hyperplasia. The optic nerve head in this patient came to resemble a morning glory disc. Conclusions: Optic nerve avulsion can adopt different courses and outcomes in different patients. Final visual outcome seems to depend on the degree of visual acuity immediately after injury. Substantial intraocular architecture changes can occur as a result of ONA.     

Tilted Optic Disks

Tilted optic disks are a common finding in the general population. An expression of anomalous human development, the tilted disk appears rotated and tilted along its axes. Visual sequelae described with tilted optic disks include myopia, astigmatism, visual field loss, deficient color vision, and retinal abnormalities. Although the natural course of tilted optic disks is nonprogressive, the anomaly can be mistaken for tumors of the anterior visual pathway, edema of the optic nerve head, or glaucoma. A thorough examination of patients with tilted disk includes refraction, dilated fundus examination, and visual field testing. At times, neuroimaging may be necessary to arrive at the correct diagnosis. Until normative data are validated for tilted disks, the role of new imaging technologies for the optic nerve head is limited. Familiarity with the spectrum of ophthalmoscopic appearance and the clinical manifestations of tilted disks may be the most critical factors in avoiding misdiagnosis.     

晶状体损伤对视神经损伤后再生影响的研究

目的观察单纯视神经损伤及视神经损伤联合晶状体损伤后视神经中Nogo-A mRNA及Nogo-A的表达,探讨晶状体损伤促进视神经损伤后再生的机制。方法 66只雄性Wistar大鼠随机分为正常对照组(A组,6只)、单纯视神经损伤组(B组,30只)和视神经损伤联合晶状体损伤组(C组,30只)。分别于造模后7d、14d、21d处死大鼠2只(A组)、10只(B组)、10只(C组),光镜下观察视神经的病理变化,免疫组织化学染色检测Nogo-A表达情况,采用逆转录-聚合酶链反应(RT-PCR)、半定量分析不同组视神经Nogo-A mRNA的表达。结果大鼠正常视神经中表达Nogo-A mRNA及Nogo-A,但表达量较低;损伤视神经后7d Nogo-A mRNA的表达量显著升高,至伤后21d B组及C组均维持较高的水平。B组损伤后7d Nogo-A表达阳性的细胞数开始增多(136.80±3.94),与A组比较差异有统计学意义(P<0.05),并保持高表达;C组损伤后21d Nogo-A的表达较B组低,差异有统计学意义(P<0.05)。结论视神经损伤后髓鞘相关抑制因子Nogo-A表达增高,晶状体损伤促进视神经再生的机制可能与Nogo-A有关。     

Optic nerve sheath decompression: A clinical review and proposed pathophysiologic mechanism

Surgical decompression of the intraorbital meningeal sheath of the optic nerve has undergone a recent resurgence of interest. Previously, the procedure had been reserved only for the most desperate cases of severe visual loss associated with chronic papilloedema. However, recent investigations have dernonstrated the operation's efficacy not only for chronic papilloedema and pseudotumor cerebri, but also for the optic neuropathy associated with the acute retinal necrosis syndrome, traumatic subdural haematomas of the optic nerve and progressive nonarteritic ischaemic optic neuropathy. In this article, we review the previous literature concerning optic nerve sheath decompression, the indication for the procedure, its techniques and results. Finally, we propose that optic nerve sheath decompression may be effective for what appear to be very different optic neuropathies because the operation may reverse optic nerve ischaemia, a common pathophysiologic feature of all these disorders.     

Nogo在视神经损伤后再生中的研究进展

哺乳动物外周神经损伤后轴突能再生很长距离。在一定条件下,中枢神经系统受损后也能部分再生。Nogo是一种已经被证实的髓鞘相关抑制因子,中枢神经损伤后Nogo释放增加、表达增强,启动神经元凋亡过程,导致神经元死亡。我们从Nogo-A,Nogo-66,可溶性NgR片段、RhoA酶与Rho-A/Rho激酶信号通道、钙离子几个方面综述Nogo的作用机制,并探讨其在视神经损伤后神经再生中的应用前景。     

颅外伤合并间接性视神经损伤

目的 探讨颅外伤合并间接性视神经损伤的临床诊断和治疗。方法 回顾分析了颅外伤合并间接性视神经损伤36例（36眼）的临床资料。11例行视神经减压术，25例接受药物治疗。结果 全部患者伤侧眼的相对瞳孔传入障碍（RAPD）阳性；眼眶CT扫描显示有眶壁和／或鼻窦骨折。药物治疗组有8例（32．00％）视力提高1行，其余17例（68．00％）视力无改善，手术治疗组有1例（909％）由数指／1．5m提高到03，5例（45．46％）恢复了瞳孔直接对光反应，1例（9．09％）恢复了VEP波，其余4例（36．36％）视力无改善。结论 对颅外伤患者应进行RAPD检查及眼眶CT扫描，确定有无间接性视神经损伤。皮质类固醇和视神经减压联合应用可能更有利于视功能的恢复。     

The usefulness of multimodal imaging for differentiating pseudopapilloedema and true swelling of the optic nerve head: a review and case series

Ophthalmic practitioners have to make a critical differential diagnosis in cases of an elevated optic nerve head. They have to discriminate between pseudopapilloedema (benign elevation of the optic nerve head) and true swelling of the optic nerve head. This decision has significant implications for appropriate patient management. Assessment of the optic disc prior to the advanced imaging techniques that are available today (particularly spectral domain optical coherence tomography and fundus autofluorescence), has mainly used diagnostic tools, such as funduscopy and retinal photography. As these traditional methods rely on the subjective assessment by the clinician, evaluation of the elevated optic nerve head to differentiate pseudopapilloedema from true swelling of the optic nerve head can be a challenge in clinical practice with patients typically referred for further neuroimaging investigation when the diagnosis is uncertain. The use of multimodal ocular imaging tools such as spectral domain optical coherence tomography, short wavelength fundus autofluorescence and ultrasonography, can potentially aid in the differentiation of pseudopapilloedema from true swelling of the optic nerve head, in conjunction with other clinical findings. By doing so, unnecessary patient costs and anxiety in the case of pseudopapilloedema can be reduced, and appropriate urgent referral and management in the case of true swelling of the optic nerve head can be initiated.     

Five rules to evaluate the optic disc and retinal nerve fiber layer for glaucoma.

A systematic approach for the examination of the optic disc and retinal nerve fiber layer is described that will aid in the detection of glaucoma. This approach encompasses 5 rules: evaluation of optic disc size, neuroretinal rim size and shape, retinal nerve fiber layer, presence of parapapillary atrophy, and presence of retinal or optic disc hemorrhages. A systematic process enhances the ability to detect glaucomatous damage as well as the detection of progression, and facilitates appropriate management.     

视神经周围炎的研究进展

视神经周围炎(OPN)是指涉及视神经鞘膜的一系列病理性炎症.OPN的经典三联征包括单侧视神经病变伴随疼痛和/或视盘水肿,此病症与其它视神经病变相似,导致诊断延迟和治疗欠佳.2016年1月,我们对发表于Medline和Ovid数据库的关键词为“视神经周围炎”的各种语言的文献进行了检索,共查找到60篇文献,发表于1956-2015年.两位作者(Tai ELM和Tevaraj JMP)分别对论文摘要进行了独立筛选,并筛选出相关文章.本次综述,我们强调OPN的特点,特别是OPN和视神经炎之间的临床差异.虽然大多数OPN的病例是特发性的,但仍需进行调查以排除特异性感染和继发性OPN的炎症原因.MRI是非常重要的检查方法,由于OPN视神经周围炎症的影像学诊断.糖皮质激素治疗可使症状与体征迅速好转,长期口服糖皮质激素并慢速递减可以降低复发的风险.     

A review of optic perineuritis

Optic perineuritis (OPN) refers to a spectrum of conditions involving pathologic inflammation of the optic nerve sheath. The classic triad of OPN consists of unilateral optic neuropathy associated with pain and/or disc oedema, but the condition often mimics other optic neuropathies, resulting in delayed diagnosis and suboptimal treatment. We performed a database search of Medline and Ovid in January 2016 for articles published in any language with the keywords ‘optic perineuritis’. Sixty articles were found, published from 1956 to 2015. Two reviewers (Tai ELM and Tevaraj JMP) performed an independent screening of abstracts. Articles of interest were subsequently examined. In this review, we highlight the salient features of OPN, with particular emphasis on the clinical differences between OPN and optic neuritis. Although the majority of cases of OPN are idiopathic, investigations are required to rule out specific infectious and inflammatory causes of secondary OPN. MRI is an invaluable component of the workup, as radiographic demonstration of peri-neural inflammation is diagnostic of OPN. Corticosteroid therapy results in dramatic and rapid reversal of the signs and symptoms, but prolonged therapy with slow tapering of oral corticosteroids may be necessary to reduce the risk of relapses.