Optic perineuritis as a rare initial presentation of sarcoidosis

Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology and establishing the correct diagnosis can be challenging. Although dysfunction of the anterior visual pathways is uncommon, it is the most common neuro-ophthalmological manifestation of this condition and given the potential for irreversible, severe visual loss, prompt diagnosis and treatment are essential. We describe a patient with optic perineuritis as a rare initial presentation of sarcoidosis and discuss the underlying pathophysiology and management.     

The Retinal Nerve Fiber Layer Defects in Patients with Anterior Ischemic Optic Neuropathy

Purpose: To demonstrate the effects of optic nerve ischemia on retinal nerve fiber layer (RNFL) and the associated visual dysfunction.Methods: 23 patients (25 eyes) with anterior ischemic optic neuropathy (AION) underwent fundus fluorescein angiography (FFA), and then red-free light pictures were taken via SE-40 exceiter filter. All pictures were printed for RNFL analysis. Humphrey central field analysis was conducted. All data obtained from FFA and visual field defects were analysed statistically.Results: The RNFL defects and the corresponding visual field defects were presented in 23 of 25 eyes (92%). The optic disc filling defects, RNFL defects and visual field defects were found to be highly correspondent to each other. The RNFL defects were mainly the local losses of RNFL which were correspondent to the ischemic regions.Conclusion: The poor optic disc filling or ischemia can result in the RNFL defects which cause the associated visual dysfunction. Because RNFL defects are irrever-siable changes, th     

Optic nerve cysticercosis

Cysticercosis of the optic nerve is an extremely rare entity and only seven cases have been reported in the world literature. A case of optic nerve cysticercosis in a 25-year-old woman is reported, along with a review of literature. The patient presented with two episodes of pain, diminution of vision and proptosis. Computed tomography and ultrasonography revealed an intraneural cyst with scolex in the retrobulbar portion of the optic nerve. A positive enzyme-linked immunosorbent assay test for cysticercosis further confirmed the diagnosis. Medical therapy in the form of oral albendazole and steroids resulted in complete resolution of the cyst, with few visual sequelae.     

Primary Bilateral Optic Nerve Sarcoidosis

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. The nervous system is involved in 5%–15% of patients. In 1%–5% of patients, sarcoidosis affects the optic nerve in some manner. Sarcoid manifesting as an isolated tumor of the optic nerve is uncommon. We report here a 50-year-old woman with primary bilateral optic nerve sarcoidosis. She had a history of progressive, painless visual loss over a 2-month period. Her cerebral magnetic resonance imaging (MRI) scan revealed diffuse thickening and enhancement of the optic chiasm affecting the pre-chiasmatic optic nerves. Surgery was performed for histopathological diagnosis and decompression of the optic nerve. Analysis of a biopsy specimen confirmed a diagnosis of sarcoidosis, and the patient was treated successfully with corticosteroids.     

Optic nerve enlargement and chronic visual loss

We present four patients with sarcoidosis of the anterior visual pathways. The first patient presented with unilateral visual loss, a mass lesion at the optic nerve head, and an enlarged orbital optic nerve. The second patient presented with bilateral progressive painless visual loss, associated with optic nerve pallor and visual field loss. In these two patients, optic nerve biopsy was diagnostic of sarcoidosis. The third patient developed optic nerve and chiasmal involvement after sarcoidosis was established by lacrimal gland biopsy. The fourth pateint had optic nerve, pulmonary, and lymph node involvement with sarcoidosis. A conjunctival and lung biopsy were diagnostic. Computed tomography and magnetic resonance imaging have greatly facilitated diagnosis of sarcoidosis of the anterior visual pathways. Sarcoidosis of the anterior visual pathways may occur alone or in association with other ocular or systemic manifestations. A conjunctival or lacrimal gland biopsy may be preferable as the initial diagnostic approach. Treatment of patients with this condition may require systemic immunosuppression, in addition to corticosteroids, to prevent permanent visual loss.     

Optic nerve axoplasm and papilledema

A detailed review of optic nerve axoplasm is presented. A number of hypotheses have been postulated for the pathogenesis of papilledema associated with increased intracranial pressure. These hypotheses, mechanical and nonmechanical, are critically evaluated in relation to five essential features of papilledema. Theories, as well as clinical and experimental studies, of axonal transport are reviewed, and a new hypothesis is proposed: Papilledema is primarily a mechanical, nonvascular phenomenon in which an excess amount of extracellular fluid is present in the prelaminar region ofthe optic disc and the accumulation of that fluid results from the leakage of axoplasm from optic nerve fibers which are compressed posterior to the lamina cribrosa of the optic disc. The authors believe that this is the only existing hypothesis consistent with all the known facts about papilledema. Discussions by Drs. J. Terry Ernest, Thomas R. Hedges, and S. S. Hayreh follow the review.     

Optic nerve breast metastasis mimicking meningioma

Purpose: We report on an optic nerve breast metastasis masquerading initially as a central retinal vein occlusion and later as an optic nerve meningioma. Methods: A 60-year-old female presented with a left central retinal vein occlusion (CRVO). She represented 7 months later with left upper ptosis, proptosis and painful rubeotic glaucoma. Computed tomography (CT) and magnetic resonance imaging suggested an optic nerve meningioma. On referral to the regional orbital unit, a mild left external ophthalmoplegia was noted and, in view of previous right mastectomy and chemotherapy 3 years earlier, the left optic nerve was biopsied simultaneously with left enucleation of her painful eye. Results: Histopathology showed infiltration of the optic nerve and meningeal sheath spreading into the subretinal space and vitreous by malignant epithelial cells, consistent with breast origin. Further CT imaging and bone scans revealed no other metastases. Single field left orbit radiotherapy of 20 Gy was given in five fractions and Arimidex (Zeneca Pharmaceuticals, Cheshire, England) was commenced with the cessation of tamoxifen. The patient was also given an ocular prosthesis. Sadly, she lost vision in her other eye due to retrograde malignant invasion of her optic chiasm and died 6 weeks later. Conclusions: Orbital and choroidal metastases are relatively common but isolated optic nerve metastases are extremely rare. Progressive infiltration of the nerve is likely to enhance CRVO ischaemia and resultant rubeotic glaucoma. In the diagnosis of CRVO, proptosis or external ophthalmoplegia, the presence of pre-existing malignant disease should raise concerns, as delay in diagnosis may affect outcome, particularly if the metastases are sensitive to pharmacological therapy.     

眼底荧光血管造影对钝挫伤视神经病变预后判断的意义

报告１７例（１９眼）钝挫伤视神经病变的眼底荧光血管造影观察，伤后２０天内的患者，ＦＦＡ表现主要为视乳头弱荧光，视乳头周围脉络膜充盈缺损，视网膜中央动脉，静脉充盈延迟。所显示的组织受损害的层次，范围，程度与临床视力预后基本上呈直接性关系，初步认为ＦＦＡ能较准确揭示了早期视神经受损害的情况，为早期诊断及判断预后提供有用的客观依据。     

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。     

Optic nerve sheath meningiomas. Clinical manifestations

A retrospective clinical study of optic nerve sheath meningiomas based on 22 patients showed that symptoms most commonly develop in women between the ages of 35 and 60 years. The most common presenting symptoms were decreased vision and transient visual obscurations. In the earliest stages, many patients presented with normal to mildly impaired acuity (despite subjectively decreased vision), optic disc edema and enlargement of the blind spot. Optic disc edema was frequently associated with refractile bodies indicative of chronic swelling. Optic disc edema preceded the development of optic atrophy. Another group of patients presented with a history of longstanding vision loss, visual acuity of 20/200 or worse and optic atrophy. Optociliary shunt vessels were late findings only seen in five patients. The most consistent visual field abnormality was peripheral constriction. Cecocentral scotomas were uncommon. Intracranial involvement was present in five patients. There were two patients with bilateral optic nerve sheath meningiomas without CT evidence of intracranial involvement. Computerized tomography was found to be indispensable in the diagnosis of optic nerve sheath meningiomas and the detection of intracranial involvement.     

Optic nerve involvement as the initial manifestation of sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown cause. It may have several diverse manifestations that may be progressive yet develop slowly. Ocular disease may occur with inactive systemic disease. We describe two patients in whom optic nerve involvement was the first manifestation of sarcoidosis and clinically mimicked an optic nerve tumour. Sarcoidosis involving the optic nerve should be considered when an optic nerve tumour is suspected.     

Optic nerve blood flow in glaucoma

Background : Glaucomatous optic neuropathy often occurs in the absence of elevated intraocular pressure and, conversely, elevated intraocular pressure may occur without associated damage of the optic nerve. These findings challenge the simple explanation of intraocular pressure being the sole cause of neural loss and have led to theories of ischaemic causes of the morbidity. This paper reviews the vascular anatomy of the optic disc, the factors that control its blood flow and the existing techniques for measurement of the blood flow. It also briefly discusses the possible role of apoptosis in glaucomatous visual loss. Method : Literature review. Conclusions : The posterior ciliary artery circulation is the main source of the blood supply to the optic nerve head with additional lesser supply via the central retinal artery and the choroidal circulation. There is considerable individual variation in the distribution of this circulation and complex regulatory systems govern its function. It is likely that microcirculatory changes in the vascular supply of the optic disc play a role in glaucoma, either as the primary abnormality or as a co‐factor that increases susceptibility to damage from increased intraocular pressure through impaired auto‐regulation. Clinical trials are currently in progress for the treatment of glaucoma with systemically administered agents that are antagonists of the receptors that mediate glutamine toxicity, a factor in the process of apoptosis.     

三种视神经髓鞘染色法的比较

目的 探讨视神经髓鞘染色方法，寻求最佳视神经髓鞘染色法．用以指导视神经疾病的临床诊治和科研工作中疾病机制的研究。方法 采用三种视神经髓鞘染色法：经典的Weil苏木素髓鞘染色法、固绿髓鞘染色法和改良的劳克坚牢蓝-核固红髓鞘染色法．比较三种髓鞘染色法的特异性、对比度等。结果 以Weil苏木素髓鞘染色将髓鞘染成黑色．以固绿髓鞘染色法将髓鞘染成绿色．以劳克坚牢蓝-核固红髓鞘染色法将髓鞘染成蓝色。结论 Weil苏木素法染色步骤较繁琐．固绿染色法清晰度较劳克坚牢蓝法差．改良的劳克坚牢蓝核固红髓鞘染色法为髓鞘染色的首选．     

Comparison of Optic Nerve Head Microvasculature Between Normal-Tension Glaucoma and Nonarteritic Anterior Ischemic Optic Neuropathy

PurposeTo compare the microvasculature of the optic nerve head (ONH) and peripapillary tissues in eyes with normal-tension glaucoma (NTG) and nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography angiography (OCTA).MethodsThirty-eight eyes with treatment-naïve NTG, 38 eyes with NAION matched for retinal nerve fiber layer (RNFL) thickness in each superior and inferior quadrant, and 38 healthy eyes matched by age were included. ONH and peripapillary retinal microvasculature was evaluated in en face images obtained using OCTA. Vessel density (VD) was calculated as the percent area occupied by vessels in the measured region in each layer segmented into the prelaminar tissue (PLT), lamina cribrosa (LC), and peripapillary retina (PR).ResultsVDs in the PLT and LC were lower in NTG eyes than in both NAION and healthy eyes (P ≤ 0.008), and did not differ between the NAION and healthy eyes. VDs in the PR did not differ between the NTG and NAION eyes. In intersectoral comparisons, VDs in the PLT (P = 0.030) and LC (P = 0.028) were lower in the affected than in the unaffected sector of eyes with NTG, but the differences did not occur in eyes with NAION. VD in the PR was lower in the affected than in the unaffected sector in both NTG and NAION eyes (both P < 0.001).ConclusionsDespite similar degrees of RNFL loss and VD decreases in the PR, VDs in the ONH differed between eyes with NTG and NAION, indicating different mechanisms of vascular impairment and ONH damage in each condition.     

视神经挫伤后的眼底血管造影

目的：用荧光素眼底血管造影（Fundus fluorescein angiography,FFA)和吲哚青绿血管造影（Indocyanine green angiography,IGGA)探讨视神经挫伤后，视神经及周围视网膜，脉络膜的循环改变。方法：对30例（30只眼）不同程度的眼球挫伤致视神经损伤的患者进行FFA与ICGA同步检查，并对它们的图像进行分析（本组除外脉络膜破裂）。结果：除1例视盘及周围视网膜，脉络膜荧光大致正常外，其余29例均出现了异常的荧光表现。FFA主要表现为：在造影早期视盘呈象限性或全视盘性的荧光充盈不良，后期荧光素渗漏或始终不能充盈，ICGA主要表现为：在FFA显示的视盘象限性弱荧光区的相邻区域脉络膜充盈时间明显延迟；FFA显示的全视盘性的弱荧光，盘周的脉络膜充盈时间明显延长，在局限性脉络膜灌注不良的对应区均出现了视网膜色素上皮（Retinal pigment epithelium,RPE)的损害，而盘周脉络膜灌注不良的区域，有9例相应区视网膜并未出现RPE的损害；有2例合并视网膜分支动脉阻塞；有19例视盘缺血的部分正是“分水区”的位置，占63.3%，本组病例中有80%视力在0.1以下。结论：眼球挫伤不仅可使视神经损伤，其周围的视网膜，脉络膜均可受到损害，应尽早施行FFA与ICGA检查，它可以详细观察，正确判断视神经挫伤后的视盘缺血情况及周围视网膜，脉络膜损害的范围和程度，及时正确地指导治疗。     

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.     

Optic nerve functions and visual evoked potential after acute optic neuritis

目的:比较视神经炎患者和正常人的视神经功能与视觉诱发电位.方法:本研究为2011年9月至2013年2月在马来西亚大学眼科医院进行的横断面研究.研究包含在检查前3mo至2y间发生特发性神经炎一次的视神经炎患者20例和10例正常人.眼科检查包括视力、色觉、视觉灵敏度、视野和视觉诱发电位.独立t检验用于比较视神经炎组与对照组视神经功能和VEP参数的差异.在参数非正态分布时,Mann-Whitney试验用于比较两组间的中位数.结果:视神经炎组的平均年龄为30.8岁.在视神经炎发作至评估期间的平均持续时间为6.6个月.视神经炎组视力较差,平均LogMAR值(0.52)明显高于对照组(P=0.001).色觉下降,视神经炎组的平均值为63%(P=0.001).视神经炎患者的对比敏感度在四个空间频率上均有所下降 [3CPD(P=0.029),6CPD(P=0.026),12CPD(P=0.002)、18CPD(P=0.006)].视神经炎组的视野下降有统计学意义(P<0.001).与对照组相比,视神经炎组的VEP P100潜伏期有轻微延长.但使用棋盘格模式1或2时,VEP潜伏期的差异不显著.视神经炎患者的VEP振幅较高,但两组差异无统计学意义.结论:视神经炎急性发作,平均6mo后视神经功能(即视力、色觉、对比敏感度和视野)显著下降.视神经炎组和对照组的VEP振幅和潜伏期无显著差异.VEP可能不是理想的诊断视神经炎既往发作史的试验,尽管VEP参数在浮动后趋于正常.     

Optical Coherence Tomography Imaging of Presumed Sarcoid Retinal and Optic Nerve Nodules

Purpose: To characterize nodular lesions of the retina and optic nerve with spectral-domain optical coherence tomography (SD-OCT) in patients with sarcoidosis.

Methods: This is a retrospective series of 6 eyes from 5 patients with an established diagnosis of sarcoidosis, with clinically detected nodules of the optic nerve or retina. All lesions were imaged with fundus photography and SD-OCT on presentation, and followed with serial imaging after treatment with corticosteroids and/or immunomodulatory therapy.

Results: Spectral OCT through the lesions revealed nodular hyperreflective processes obscuring the retinal layers or optic cup, with local structural changes, including subretinal and intraretinal fluid. After treatment with corticosteroids and/or immunosuppression in 4 followed patients, all lesions regressed with improvement in associated structural changes, but did not entirely disappear.

Conclusions: Spectral OCT can be useful in identifying lesion morphology and location, and in tracking the response to treatment in eyes with posterior-segment nodules, presumably secondary to sarcoidosis.