视神经属于中枢神经的一部分，损伤后难以再生。视神经损伤通常伴随视网膜神经节细胞(retinal ganglion cells，RGCs)的持续性凋亡及视神经变性坏死，引起视力损害甚至完全失明。目前针对视神经再生的基础研究主要集中于保护和维持视神经损伤后RGCs的存活、促进RGCs轴突再生及重建视神经功能。本文以RGCs保护、轴突再生及视神经功能重建等为关键词，查询国内外最新视神经再生研究类文献，并分析整理，从抗氧化应激、提供外源性细胞因子、炎症刺激、抗胶质瘢痕、基因调控等方面阐述近年的视神经再生研究进展，以期对后续的基础研究开展及临床转化有所帮助。

Optic nerves are a part of the central nervous system, which is difficult to regenerate after injury. Optic nerve injury is usually accompanied by continuous apoptosis of retinal ganglion cells (RGCs) and degeneration or necrosis of optic nerves, resulting in visual impairment or even complete blindness. At present, the basic research on optic nerve regeneration mainly focuses on protecting and maintaining the survival of RGCs after optic nerve injury, promoting RGCs axon regeneration, and reconstructing optic nerve function. In this paper, RGCs protection,axon regeneration, and optic nerve function reconstruction are used as key words to collect the latest domestic and foreign literatures on optic nerve regeneration. The research progress of optic nerve regeneration in recent years was reviewed from the aspects of antioxidant stress, provision of exogenous cytokines, inflammatory stimulation, anti-glial scar, gene regulation and so on, in order to help the follow-up basic research and clinical translation.

作为一种新型无创且操作简单的主观检查手段，临界闪烁融合频率(critical flicker fusionfrequency,CFF)可动态反映人眼视功能变化情况。作为早期识别脱髓鞘病变和评估视功能恢复情况的敏感指标，上个世纪已被国外学者用于视网膜和视神经疾病研究中，包括氯喹中毒性视网膜病变、糖尿病视网膜病变、中心性浆液性视网膜病变、年龄相关的黄斑病变、乙胺丁醇中毒性视神经病变、视神经炎和非动脉炎性前部缺血性视神经病变。在视网膜和视神经疾病中，CFF均有不同程度下降，依据CFF改善程度以及主要损害的色光可能有助于视网膜和视神经疾病的鉴别，且CFF与其他视功能，视力、视野、视觉诱发电位的潜时具有较好的相关性。目前国内相关研究尚处于起步阶段，本文就CFF在视网膜和视神经疾病的应用情况做一总结。

As a new non-invasive and simple subjective examination method, critical flicker fusion frequency (CFF) can dynamically reflect the changes of visual function of human eyes. As a sensitive indicator for early identification of demyelinating diseases and assessment of visual function recovery, it has been used by foreign scholars in the last century in the field of retinal and optic nerve diseases, including chloroquine toxic retinopathy, diabetic retinopathy, central serous retinopathy, age-related macular degeneration, ethambutol-induced optic neuropathy, optic neuritis and non-arteritic anterior ischemic optic neuropathy. Though there was a different decrease of CFF in retina and optic nerve diseases, it may be helpful for the differentiation of retinal and optic nerve diseases according to the degree of CFF improvement and the main damaged color light. Moreover, CFF has a good correlation with other visual functions, visual acuity, visual field, and peak time of visual evoked potential. At present, and relevant domestic studies is still in its infancy. This article summarizes the application of CFF in retinal and optic nerve diseases.

视神经脊髓炎谱系疾病相关视神经炎是一种累及视神经的脱髓鞘性炎症疾病，视力损伤严重，预后差，复发率高。及时控制急性发作和有效预防复发是治疗的关键。目前治疗主要包括糖皮质激素、血浆置换、免疫吸附、免疫抑制剂、靶向单抗类药物。特别是近年来依库丽单抗、萨特利珠单抗、及依那利珠单抗取得重大进展。该文综述视神经脊髓炎谱系疾病相关视神经炎近年治疗研究进展，期望为临床决策提供有益参考。

Neuromyelitis optica spectrum disorders (NMOSD) is a central nervous system inflammatory demyelinating disease with involvement of the optic nerve and spinal cord, with poor prognosis and high recurrence rate. Timely control of acute attacks and effective prevention of recurrence are the keys to treatment. This article reviews the recent research progress in the treatment of optic neuritis associated with NMOSD , hoping to provide useful references for clinical decision-making.

目的：总结并分析视野为中心暗点的视神经病变的病因和临床特点，为临床诊治提供参考。方法：回顾性病例研究。分析2018年8月至2020年3月期间，在中山大学中山眼科中心神经眼科专科门诊就诊，视野表现为中心暗点且随访1年以上的视神经病变患者的资料。患者双眼均行最佳矫正视力、眼压、裂隙灯显微镜及前置镜、频域光学相干断层扫描、视野、颅脑和眼眶核磁共振检查，静脉采血行血常规、血生化、肝肾功能、感染指标(乙肝、丙肝、梅毒、HIV及结核T-spot)检查及Leber遗传性视神经病变的线粒体DNA和OPA1基因检测。结果：共纳入20例患者，病因诊断构成为：Leber遗传性视神经病变9例(45%)，显性视神经萎缩2例(10%)，乙胺丁醇中毒性视神经病变6例(30%)，营养性视神经病变2例(10%)和特发性脱髓鞘性视神经病变1例(5%)。遗传性视神经病变的视力预后差，特别是Leber遗传性视神经病变，78%的随访视力(≥1年)不高于0.1。伴有mtDNA或OPA1基因突变的乙胺丁醇中毒性视神经病变患者，视力预后差。结论：视野为中心暗点表现的视神经病变，主要为遗传、中毒和营养性视神经病变。遗传性视神经病变具有不完全外显率的特点，视野为中心暗点的视神经病变需行基因检测排除遗传性视神经病变。

Objective: To summarize and analyze the etiology and clinical features of optic neuropathy with visual field defect of central scotoma as a reference for clinical diagnosis and treatment. Methods: In the retrospective case study, the data of patients admitted in Neuro-ophthalmic Department of Zhongshan Ophthalmic Center of Sun Yat-sen University from August 2018 to March 2020, who presented with visual field defect of central scotoma and were followed up for more than 1 year, were analyzed. Both eyes of all the patients underwent best corrected visual acuity, intraocular pressure, slit lamp microscope and front mirror, spectral domain optical coherence tomography, humphry visual field tests and MRI of brain and orbit. We examined the blood routine, biochemical test, renal and liver function, infection indicators (hepatitis B, hepatitis C, syphilis, HIV and tuberculosis T-spot), mitochondrial DNA and OPA1 gene detection of Leber hereditary optic neuropathy. The follow-up time of the patients in neuro-ophthalmic department was more than 1 year. Results: A total of 20 patients were recruited. Among them, the etiological diagnosis consisted of 9 patients of Leber hereditary optic neuropathy (45%), 2 of dominant optic atrophy (10%), 6 of ethambutol-induced optic neuropathy (30%), 2 of nutritional optic neuropathy (10%) and 1 of idiopathic demyelinating optic neuropathy (5%). The patients with hereditary optic neuropathy showed a poorer visual prognosis, especially Leber hereditary optic neuropathy, with 78% of follow-up visual acuity (≥1 year) not higher than 0.1. The visual prognosis of ethambutol-induced optic neuropathy patients with mtDNA or OPA1 gene was poor. Conclusions: The optic neuropathy of visual field defects with central scotoma includes mainly hereditary, toxic and nutritional optic neuropathy. Hereditary optic neuropathy is characterized by incomplete penetrance, and genetic testing is required to exclude hereditary optic neuropathy if the visual field is the central scotoma.

目的：探讨小剂量利妥昔单抗(rituximab, RTX)预防视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder, NMOSD)复发的有效性和安全性。方法：采用前瞻性自身对照试验，选取2020年7月至2021年4月临床确诊为NMOSD的38例患者进行研究，给予小剂量RTX治疗。所有患者均进行病史采集、眼科检查和血清学指标检测，记录NMOSD年复发率(ARR)、最佳矫正视力(BCVA)、合并自身抗体情况和追加治疗的情况。视力检查采用Snellen视力表进行，并将结果转换为最小分辨角对数(logMAR)视力记录。随访至少12个月（17.29±2.2）个月，以末次随访为疗效判定时间点，比较治疗前后ARR、BCVA；分析复发与发病年龄、是否合并自身免疫抗体阳性和患自身免疫性疾病的关系。记录不良反应的发生率和追加治疗的时间。结果：共38例患者61眼纳入研究。其中男性4例，女性34例。发病年龄12~60岁，中位发病年龄23 (18~29.3)岁。病程10.0~265个月，中位病程65 (48.3~101.0)个月。治疗前logMAR矫正视力(1.15±0.13)，治疗后logMAR矫正视力(1.54±0.39)，比较差异无统计学意义(t=1.120，P=0.267)。治疗前ARR(1.50±0.86)次/年，治疗后ARR降低为(0.12±0.07)次/年，比较差异有统计学意义(t=8.304，P<0.001)。追加治疗时间为(6.4±2.3)月。随访期间3例患者复发，复发次数为 5次。 复发者与未复发者的发病年龄、合并免疫抗体阳性比例、合并自身免疫性疾病比例比较，差异均无统计学意义(均P>0.05)。38例患者中，出现输注不良反应7例，给予减慢RTX滴速及加用地塞米松5 mg治疗后均缓解，随访期间未见其他明显不良反应。结论：小剂量RTX可以有效清除B淋巴细胞，预防NMOSD复发，且安全性较好。

Objective: To evaluate the efficacy and safety of long-term treatment with low-dose rituximab for neuromyelitis optica spectrum disorders (NMOSD). Methods: A prospective self-control study. A total of 38 patients who were diagnosed with NMOSD from July 2020 to April 2021 were recruited for rituximab treatment. All patients collected medical history, ophthalmic examination and serological test. Recorded the annual recurrence rate (ARR), best corrected visual acuity (BCVA), combined autoantibodies and therapy times after the first treatment. The BCVA was examined using Snellen chart, and converted to logMAR. The patients were followed up at least 12(17.29±2.2) months, and the last follow-up was taken as the time point of efficacy evaluation. ARR and BCVA before and after treatment were compared. To analyze the relationship between relapse and age of onset, combination of autoimmune antibodies and autoimmune diseases. The incidence of side effects and duration of additional therapy were recorded. Results: A total of 38 NMOSD patients (4 male/34 female, 61 involved eyes) were included in this study. The ages of onset age were 12-60 years, the median onset age was 23 (18~29.3) years. Duration of disease was 10.0~265 months, the median duration was 65 (48.3~101.0) months. Before treatment, the mean BCVA was 1.15 ± 0.13 , the mean BCVA at last follow-up was 1.54 ± 0.39, which was no significant difference (t=1.120, P=0.267). The mean ARR before and after treatment were 1.50±0.86 and 0.12 ± 0.07, respectively, with significant difference (t=8.304, P<0.001). The mean reinfusion period was 6. 4±2.3 months. Five relapses in 3 patients were observed. There were no significant difference between relapsed patients and non-relapsed patients on onset age, with/without auto-immune antibody ratio, with/without auto-immune diseases ratio (all P>0.05). Of 38 patients, 7 patients had side effects, all patients who had side effects, slowing down the infusion speed of RTX or infusing 5 mg of dexamethasone could relieve the discomfort. Conclusions: Low-dose RTX can effectively clear B lymphocytes, prevent NMOSD recurrence and with good safety.

外伤性视神经病变是因外力损伤视神经，进而严重损害视力的致盲性眼病。自噬是一种细胞内降解途径，有助于维持细胞正常组分合成与受损细胞器及有毒细胞成分的分解之间的平衡。视神经受创后，视神经和视网膜中自噬标志物增加。自噬在外伤性视神经病变的不同阶段对视网膜神经节细胞可能起不同作用。多数研究表明，上调自噬可以减轻外伤性视神经病变中视网膜神经节细胞的死亡；也有研究提示在视神经损伤后极早的时期抑制自噬可以抑制视网膜神经节细胞轴突变性。该文对自噬的定义及功能、自噬的发生机制、视神经创伤后自噬水平改变、自噬在视神经创伤后对视网膜神经节细胞的作用的研究结果进行综述。

Traumatic optic neuropathy is a blinding eye disease that causes severe damage to vision due to external force damage to the optic nerve.. Autophagy is an intracellular degradation pathway that helps maintain the balance between the synthesis

of normal cell components and the breakdown of damaged organelles and toxic cellular components. Autophagy markers are increased in optic nerve and retina after optic nerve trauma. Autophagy may play different roles on retinal ganglion cells (RGCs) at different stages of traumatic optic neuropathy. Most studies have shown that upregulating autophagy can attenuate RGCs death in traumatic optic neuropathy; however, it has also been suggested that inhibition of autophagy at ultra-early stage after injury can inhibit RGCs axonal degeneration. In this review, we reviewed the definition and function of autophagy, the mechanism of autophagy, and summarized the change of autophagy level after optic nerve trauma, as well as the effects of autophagy in RGCs after optic nerve trauma.

渐进性视功能障碍多见于屈光不正、原发性开角型青光眼、白内障、视神经视网膜遗传代谢性疾病等，少见于眶内和颅内占位性疾病。颅内蛛网膜囊肿通常是无症状的先天性良性病变，少数出现视功能障碍。 视神经管骨壁缺如见于后组筛窦和蝶窦气化良好的正常人。该文报告 1例59岁男性患者，因左眼视野缺损伴视物模糊1年余就诊，确诊左侧颞极蛛网膜囊肿合并视神经管骨壁缺如。笔者通过收集该患者的病史、影像学资料和视功能检查结果，分析其出现视功能障碍的机制。

Progressive visual impairment is more common in ametropia, primary open-angle glaucoma, cataract, hereditary and metabolic diseases of optic nerve and retina, and less common in orbital and intracranial masses. Intracranial arachnoid cysts are usually asymptomatic benign congenital lesions with a small number of visual impairments. The absence of the bone wall of the optic canal was seen in normal subjects with good gasification of the posterior ethmoid sinus and sphenoid sinus. In this case report we describe a 59-year-old man with a left temporal arachnoid cyst and a defect of the bone wall of the optic canal complained of left visual field defect and blurred vision for more than one year. The mechanism of visual dysfunction was analyzed by collecting the patient’s medical history, imaging data and the results of visual function examination.

高度近视(high myopia，HM)作为一种特殊类型的屈光型眼病，不仅会导致进行性、退行性眼底改变，其视神经损伤的患病率也很高。青光眼是全球范围内最常见的一种不可逆致盲性眼病，原发性开角型青光眼(primary open-angle glaucoma，POAG)是最常见的青光眼类型。近年来的研究发现HM与POAG的病理改变存在相似之处。由于HM眼底改变与早期POAG眼底改变容易混淆，HM患者早期发现POAG对延缓或阻止疾病进展很重要。HM患者长期随访不仅要观察黄斑病变，视神经形态与结构改变的观察也不容忽视。

As a special type of refractive eye disease, high myopia (HM) not only causes progressive and degenerative fundus changes, but also has a high prevalence of optic nerve damage. Glaucoma is the most common form of irreversible blinding eye diseases worldwide, among which, primary open-angle glaucoma (POAG) is the most common type. In recent studies, HM is found to have similarities on pathological changes as that of POAG. And HM fundus changes are easily confused with early stage POAG fundus changes; thus, the early detection of POAG on HM patients is highly important on disease deferment or prevention of disease progression. Macular degeneration as well as optic nerve morphology and structural changes are to be observed in the long-term follow-up for HM patients.

      青光眼(glaucoma)是一组具有特征性视神经损害和视野缺损的眼病,在临床上常常表现为视力减退、眼部胀痛伴头痛等症状，是一种致盲率居第二位的不可逆性眼病。青光眼视神经损伤有一定 的遗传倾向，造成视神经凹陷性萎缩及视野不同程度缺损这两种结局的危险因素有多种,其中致盲的主要原因包括进行性视神经节损害、病理性眼压升高等。所以在青光眼的治疗中一方面要考虑降低眼压，另一方面还要保护好视神经。本文将从视神经损害的机制、影响因素、分级方法、视神经相关检测指标及其在临床上相关仪器应用、治疗等方面进行综述。

Glaucoma is an irreversible eye disease with the characteristic of optic nerve damage and visual ?eld defect, the clinical manifestation is behaved for vision loss, eye pain with symptoms such as headache, which rate of blindness ranks second place. The optic nerve injury of glaucoma has a certain genetic predisposition. There are many risk factors result in optic nerve atrophy and visual ?eld defect, one of the major causes of blindness including optic ganglion damage, pathological elevation of intraocular pressure. So on the one hand in the treatment of glaucoma should be considered to reduce intraocular pressure, on the other hand, to protect the optic nerve. This article will reviews the mechanism, influencing factors, classification methods, optic nerve related detection indexes and clinical application and treatment of optic nerve injury. 

       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。

       Medulloepithelioma is a clinically uncommon tumor originated from nervous system, often occurred in central nerve system and ciliary body, and malignant medulloepithelioma of the optic nerve is far rarer. So far, there has been no case report in China. It may be clinically misdiagnosed because it resembles glioma at the early stage of the disease. We reported a boy with a tumor in his right eye at age of 3.8 years, which was shown by biopsy of the partial tumor that there were some obviously heteromorphous neoplastic cells, karyokinesis, and moreover, some neoplastic cells differentiatied into cartilage cells, gradually formed into hyaline cartilage islands and the expressions of NSE and S-100 were positive. Teratoid malignant medulloepithelioma of optic nerve was made pathologically.