Precipitous visual loss secondary to optic nerve toxoplasmosis as an unusual presentation of AIDS

Life-threatening conditions requiring urgent medical treatment rarely present to the ophthalmolgist. This case report describes a patient with precipitious visual loss as the primary complaint and which subsequently led to the diagnosis of Toxoplasmic papillitis and life-threatening cerebral involvement as an initial manifestation of AIDS.     

眶尖部及视神经肿瘤的早期诊断

目的探讨眶尖部及视神经肿瘤在眼球突出前的临床表现、影像学检查特征及临床诊断要点。方法回顾性分析22例无明显眼球突出单侧眼眶肿瘤患者的病历资料,包括主要症状、首发表现、临床诊治过程及影像学检查结果。结果22例肿瘤患者中,海绵状血管瘤6例,神经鞘瘤、视神经鞘脑膜瘤、鼻窦及鼻咽腔恶性肿瘤眶内蔓延各4例,后组筛窦黏液囊肿2例,神经纤维瘤和视神经胶质瘤各1例。以视力下降为首发症状者17例,视力下降合并复视者3例。全部患者均在CT或MRI检查后确诊。肿瘤位于眶尖部或起源于视神经。结论眶尖部及视神经肿瘤的早期症状是视功能障碍,无眼球突出表现。视力呈缓慢进行性下降,按视神经炎治疗效果差。CT或MRI检查对早期诊断起重要作用。     

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.     

颅眶损伤视神经减压术的探讨

目的：总结20例颅眶损伤急诊开颅手术时行视神经减压的疗效。方法：回顾性总结近4年来收治的20例严重颅眶损伤患者行急诊视神经减压术。结果：20例严重颅眶损伤共22眼有视神经损伤，减压术后3月随访，18眼视力有不同程度提高。结论：对严重颅眶损伤合并视神经损伤者，若能早期诊断，及时治疗，可有效地改善视力。     

视神经鞘切开减压术对大鼠视神经挤压伤后RGC凋亡的影响

目地观察早期视神经鞘切开减压术对大鼠视神经挤压伤后RGC凋亡的相关机制。方法大鼠91只分为对照组、损伤组、手术组各7、42、42只通过视网膜切片技术,HE染色,免疫组化SP法于伤后3、7、15各时问点视网膜神经节细胞计数及检测BCL-2和BAX阳性细胞数。结果手术组各时间点RGC计数均高于损伤组,差异有显著性（P〈0．05）。手术组各时间点BCL-2阳性细胞数均高于损伤组,BAX阳性细胞数均低于损伤组,差异有显著性（P〈0．05）。结论早期视神经鞘切开减压术可对大鼠视神经挤压伤后能上调BCL-2基因的表达和下调BAX基因的表达而抑制RGC的凋亡。     

Optic Neuropathy Following Endovascular Coiling of an Orbital Varix

Orbital varices are vascular malformations consisting of abnormal venous channels. Indications for intervention include loss of vision, elevated orbital pressure with motility deficit, and intractable pain. We present a case of a 65-year-old woman with an orbital varix, who underwent embolization via endovascular coiling. This treatment resulted in an intralesional thrombosis with subsequent enlargement of the varix, leading to a compressive optic neuropathy and severe vision loss. If an intervention is to be contemplated, the possibility of this rare but serious complication should be carefully considered and patients appropriately counseled.     

A case of multiple cranial nerve palsies as the initial ophthalmic presentation of antiphospholipid syndrome

PURPOSE: To report a case of third, fourth, and six cranial nerve palsies with antiphospholipid syndrome (APS). METHODS: Medical records of a 16-year-old female diagnosed with idiopathic intracranial hypertension (IIH) in primary APS were reviewed. RESULTS: A 16-year-old female presented with headache and diplopia. Ocular examinations revealed marked bilateral disc edema. She was unable to depress, adduct, and abduct in left eye and had limited abduction in the right eye. Cerebrospinal fluid had a normal composition and a pressure of 400 mmH20. Lupus anticoagulant and IgG anticardiolipin antibody were positive. There was no clinical evidence of other autoimmune disease. Brain magnetic resonance (MR) imaging, MR angiography, and conventional angiogram with venous phase were normal. She was diagnosed with bilateral sixth, and left third and fourth cranial nerve palsies secondary to idiopathic intracranial hypertension in primary APS. CONCLUSIONS: To our knowledge this is the first reported case of concurrent third, fourth, and sixth cranial nerve palsies in a patient with primary APS.     

Optic nerve hypoplasia in association with brain anomalies and an abnormal electroretinogram

Abnormal electroretinograms (decreased amplitude and prolonged implicit time > 2 standard deviations) in several patients with optic nerve hypoplasia (ONH) and developmental brain anomalies led us to study the electroretinogram (ERG) in 34 consecutive cases of ONH presenting to our practice. Ages of the subjects were between 7 months and 13 years (mean, 4 years). ERGs were recorded from each eye by means of a contact lens electrode and ganzfeld stimuli. Rod-dominated dark-adapted responses were recorded as well as cone-dominated light-adapted responses. When clinically indicated, brain imaging by either computed tomography (CT) or magnetic resonance imaging (MRI) was performed. The ERG was abnormal in 12 (35%) of the children, including five (42%) with unilateral ONH. Imaging studies of the brain in 12 children with ONH and an abnormal ERG disclosed brain malformations in nine (75%) of them compared to five (23%) in the group with ONH and a normal ERG. An abnormal ERG associated with ONH and brain malformations may represent retinal or transsynaptic degeneration beyond the ganglion cell layer and implies a shared causative mechanism.Abbreviations CNS central nervous system - ONH optic nerve hypoplasia     

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.     

Visual evoked potentials during the early phase of optic nerve compression in the orbital cavity

We obtained case histories and electrophysiologic recordings from four patients with transient vision impairment due to acute orbital compression. The visual evoked potentials (VEPs) displayed alterations that depended on the size and consistency of the compressing pathology and also on the duration of the compression. This study provides evidence of the utility of the VEP in the assessment of the severity and reversibility of optic nerve lesions. The case histories also emphasized the necessity to elucidate the pathologic process of compressive lesions of the optic nerve.     

12例视神经撕脱的临床分析

目的探讨视神经撕脱的发生机制、临床表现特点,评价分析其疗效和预后。方法通过本组12例视神经撕脱患者的临床表现和治疗经验,结合文献进行回顾性分析讨论。结果视神经撕脱是一种严重的眼外伤,预后差。结论提高本病早期诊断率,减少早期的误诊和漏诊,寻找有效的治疗方案,减少不必要的治疗。     

视神经胶质瘤的诊治研究现状及进展

视神经胶质瘤（ONG）是一种好发于儿童和青少年且相对罕见的中枢神经系统肿瘤,主要病理类型为低级别的毛细胞型星形细胞瘤。其分为散发和1型神经纤维瘤病（NF-1）相关的ONG。由于ONG与视神经的紧密关系,在诊疗上存在其特殊性,其诊断主要依靠病史、症状和体征,以及磁共振成像和CT等影像检查。ONG应与神经鞘脑膜瘤、视神经炎...     

Bilateral orbital myeloid sarcoma as initial manifestation of acute myeloid leukemia

Background Granulocytic sarcoma is a rare orbital complication of acute leukemia. It concerns primarily children under 10 years of age suffering from primitive acute myeloid leukemia. The diagnosis is made by clinical examination, computed tomography and confirmed by haematological investigations. The treatment approach is based on chemotherapy associated with intravenous steroid therapy. Case report We report the case of a 6-year-old girl who presented with bilateral proptosis revealing acute myeloid leukemia. The patient was treated by a combination of chemotherapeutic drugs in two phases, associated with intravenous steroids. After a follow-up period of 24 months, the patient was in complete remission. Conclusion The diagnosis of granulocytic sarcoma should be considered in any orbital mass of uncertain origin, particularly if it is bilateral. Special stains and immunohistochemistry play an important role in the diagnosis.     

Retrospective chart review of the use of imaging and biopsy in the diagnosis of optic nerve sheath meningiomas and intra-conal orbital lymphomas at a single institution

Optic nerve sheath meningiomas (ONSM) and intra-conal orbital lymphomas are common entities on the differential of a retrobulbar optic nerve involving space-occupying lesion. In this study, we compare the pre-surgical diagnosis, based on clinical presentation and neuroimaging, to the surgical pathology results of intra-conal orbital lymphomas and ONSM. This is an IRB approved retrospective chart review of orbital lymphomas and optic nerve sheath meningiomas biopsied by a single surgeon over a 4-year period at a single institution. Pre-surgical diagnosis and surgical pathology were compared. Fifteen cases of orbital lymphoma were identified. Fourteen were excluded based on extra-conal location. The single histologically confirmed intra-conal orbital lymphoma had a pre-surgical diagnosis of ONSM. Four cases of optic nerve sheath meningioma were identified. Three of the 4 cases of histologically confirmed ONSM had a pre-surgical diagnosis of ONSM. One of the 4 had a pre-surgical diagnosis of lymphoma. Diagnosis based on surgical pathology differed from the pre-surgical diagnosis in 2 out of 5 cases showing that clinical diagnosis does not always correlate with histologic diagnosis. Although both diseases are typically managed with radiation therapy, the treatment dosage and systemic disease implications are very different. These findings emphasis the importance of biopsy in the diagnosis of orbital lesions surrounding the optic nerve.     

应用液压冲击颅脑损伤仪建立大鼠外伤性视神经损伤动物模型

目的 观察应用液压冲击颅脑损伤仪(FPI)能否成功建立大鼠外伤性视神经损伤动物模型.方法 成年雌性Wister大鼠71只,随机选取5只为正常对照组,其余66只为模型组.模型组再随机分为3组.第1组8只大鼠,分别于损伤前、损伤后1、3 d、1、2、4、6、8周行双眼闪光视觉诱发电位(F-VEP)及视神经核磁共振成像(MRI)检查;第2组56只大鼠,随机分成7个亚组,每个亚组8只大鼠,分别于损伤后1、3 d、1、2、4、6、8周行视网膜组织病理学及末端脱氧核苷酸移换酶介导的dUTP缺口末端标记测定法(TUNEL)检测;第3组2只大鼠,分别于损伤后4、8周行视神经透射电子显微镜检测.根据打击力量不同.将损伤眼分为轻、重2组.重伤组打击锤以25.的预定角度打击视神经,平均打击力量(699.14±60.79)kPa,轻伤组以15°的预定角度打击,平均打击力量(243.18±20.26)kPa.每只大鼠右眼为重伤组,左眼为轻伤组.结果 重伤组损伤后1d主波潜伏期延长,与正常对照组比较,差异有统计学意义(t=2.06,P<0.05);F-VEP振幅在损伤后2周内逐渐降低,2周后趋于稳定(F=1.98,P>0.05).轻伤组损伤后1 d主波潜伏期延长,与正常对照组比较,差异有统计学意义(t=2.19,P<0.05);振幅在损伤后4周内逐渐降低,4周后趋于稳定(F=1.62,P>0.05).MRI检查显示,损伤后1 d视神经高信号,损伤后8周仍较明显.组织病理学检查发现,损伤后1 d视网膜神经节细胞层(GCL)可见毛细血管破裂出血;损伤后4周GCL内可见空化的视网膜神经节细胞.损伤后3 d视网膜各层均出现凋亡阳性细胞;TUNEL染色发现损伤后1、2周凋亡阳性细胞明显增多.结论 应用FPI能成功建立大鼠外伤性视神经损伤模型.     

Optic disc pit with peripapillary retinoschisis presenting as a localized retinal nerve fiber layer defect

A 59-year-old woman was referred to our clinic for a glaucoma evaluation. The visual acuity and intraocular pressure were normal in both eyes. However, red-free fundus photography in the left eye showed a superotemporal wedge-shaped retinal nerve fiber layer defect, and visual field testing showed a corresponding partial arcuate scotoma. In an optical coherence tomography examination, the macula was flat, but an arcuate-shaped peripapillary retinoschisis was found. Further, the retinoschisis seemed to be connected with a superotemporal optic pit shown in a disc photograph. After 3 months of a topical prostaglandin analogue medication, the intraocular pressure in the retinoschisis eye was lowered from 14 to 10 mmHg and the peripapillary retinoschisis was almost resolved. We report a rare case of an optic disc pit with peripapillary retinoschisis presenting as a localized retinal nerve fiber layer defect.     

源自视神经的畸胎性恶性髓上皮瘤一例

髓上皮瘤是源自神经系统的一种少见的恶性肿瘤,多发生在中枢神经系统和睫状体,而源自视神经的恶性髓上皮瘤则很少见,国内尚未有病例报道.此病早期类似胶质瘤,易造成误诊.本文报道了1例3岁10个月的男性患儿.经部分肿物切除活检发现肿瘤具有典型恶性髓上皮瘤的病理特点,部分瘤细胞向软骨细胞分化,并逐渐形成透明软骨岛.NSE及S-100表达阳性,病理诊断为源自视神经的畸胎性恶性髓上皮瘤.     

视觉诱发电位在视神经挫伤诊断治疗中的应用

目的 探讨视神经挫伤后的视觉诱发电位（ＶＥＰ）的异常率、挫伤程度及预后估计。方法记录单眼挫伤患者的伤眼、对侧眼及治疗后伤眼的视觉诱发电位（ＶＥＰ）。结果 （１） 挫伤眼各视力组ＶＥＰ异常率明显高于对照组,且随挫伤眼视力的下降而增高。（２）挫伤眼矫正视力〈０．１者ＶＥＰ异常更明显,且预后差。结论 ＶＥＰ是视神经 失伤早期诊断、判断预后的客观检查方法。