视神经炎(optic neuritis,ON)是指视神经的炎性脱髓鞘病变，是引起中青年人视力下降的主要原因。近年来，髓鞘少突胶质细胞糖蛋白抗体阳性视神经炎(myelin oligodendrocyte glycoprotein antibody-positive ON,MOG-ON)成为神经眼科领域的研究热点，国内外报道不断增加。2021年3月，中华医学会眼科学分会神经眼科学组制定了《中国脱髓鞘性视神经炎诊断和治疗循证指南(2021年)》，将MOG-ON作为新的视神经炎亚型纳入脱髓鞘性视神经炎的诊疗体系，给广大眼科医生提供了新的参考依据。因此，临床医生需要充分认识MOG抗体相关疾病和MOG-ON的临床特征和治疗进展，努力提高其诊断和治疗水平，使此类患者能够得到更多的获益，造福于更多的视神经疾病患者。
Optic neuritis(ON)is an inflammatory demyelinating disease of the optic nerve, which is the main cause of vision loss in young and middle-aged people. In recent years, myelin oligodendrocyte glycoprotein antibody-positive ON(MOG-ON)has become a research hotspot in the field of neuro-ophthalmology, and reports at home and abroad are increasing.In March 2021, the Neuro-ophthalmology Group of Ophthalmology Branch of Chinese Medical Association formulated the Evidence-Based Guidelines for the Diagnosis and Treatment of Demyelinating Optic Neuritis in China(2021) , and included MOG-ON as a new optic neuritis subtype in the diagnosis and treatment system of myelinating optic neuritis providing a new reference for the majority of ophthalmologists. Therefore, clinicians need to fully understand the clinical features and treatment progress of MOG antibody-related diseases and MOG-ON, and strive to improve the level of diagnosis and treatment, so that such patients can get more benefits and benefit more patients with optic nerve diseases.
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：分析单中心神经眼科疾病谱及流行病学特点，为指导神经眼科疾病诊断和治疗提供基础。方法：纳入2010年1月1日—2021年12月31日中国人民解放军总医院神经眼科病区收治的神经眼科疾病患者，从电子病例系统检索和记录所有纳入病例的年龄、性别、地区分布及病种亚型分析。结果：共计7245例神经眼科患者纳入统计，其中男性3331例(46.0%)、女性3914例(54.0%)，男女比例1∶1.2；年龄(38.2±17.5)岁。83.25%(6031/7245)为传入神经系统疾病，9.92%(719/7245)为传出神经系统疾病和眼眶疾病，6.83%(495/7245)未归类。病种分析显示，占比最高的是脱髓鞘性视神经炎(demyelinating optic neuritis,DON)，为40.17%(2910/7245)；占比第二的是非动脉炎性前部缺血性视神经病变(nonarteritic anterior ischemic optic neuropathy,NAION)，为11.37%(824/7245)；占比第三的是外伤性视神经病变5.15%(373/7245)，其中7.85%(569/7245)表现为不明原因视神经萎缩。从年龄分布来看，DON和外伤性视神经病变患者中18~40岁者占比最高(分别为48.63%和44.24%)，NAION患者中41~60岁者占比最高(66.14%)，小于18岁的未成年患者在遗传性视神经病变中占比最高，比例为48.58%。在2226例DON患者中，视神经脊髓炎(neuromyelitis optica,NMO)/视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)比例最高，为60.02%；髓鞘少突胶质细胞糖蛋白抗体(myelinoligodendrocyte glycoprotein antibody,MOG-IgG)阳性视神经炎比例为11.68%；多发性硬化(multiple sclerosis,MS)和MS相关性视神经炎和慢性复发性炎性视神经病变(chronic recurrent inflammatory optic neuropathy,CRION)占比较低，分别是1.8%和2.25%。DON整体患者中，男女比例为1∶3.08；在NMO/NMOSD患者中男女比例为1∶8；MOG阳性视神经炎患者中，男女比例为4∶5；在非典型视神经炎患者中，男性比例高于女性，为1.28∶1；DON患者中，81.79%患者为中青年，MOG阳性视神经炎未成年患者可达41.15%。结论：DON和NAION是神经眼科传入系统疾病最常见两大病种。
Objective: To analyze the spectrum and epidemiological characteristics of neuro-ophthalmic diseases from single center, and to provide basis for guiding the diagnosis and treatment of neuro-ophthalmic diseases. Methods: Patients with neuro-ophthalmic diseases admitted to the neuro-ophthalmology ward of Chinese PLA General Hospital from January 1, 2010 to December 31, 2021 were enrolled. The age, gender, regional distribution and disease subtypes of all included patients were retrieved and recorded from the electronic case system. Results: A total of 7245 patients with neuro-ophthalmic diseases were enrolled, including 3331 males(46.0%)and 3914 females(54.0%), with a male to female ratio of 1:1.2. The average age was 38.2±17.5 years. 83.25%(6031/7245)were afferent nervous system diseases, 9.92% (719/7245)were efferent nervous system diseases and orbital diseases, and 6.83%(495/7245)were not classified. The ratio of demyelinating optic neuritis(DON)was the highest(40.17%,2910/7245), followed by nonarteritic anterior ischemic optic neuropathy(NAION)(11.37%,824/7245)and traumatic optic neuropathy(TON) (5.15%,373/7245). The ratio of optic nerve atrophy with unknown causes was 7.85%(569/7245). Characteristics of age distribution, the DON and TON were more common in 18-40 age group(the proportion were 48.63% and 44.24%,respectively), the NAION was common in 41-60 age group(66.14%), and the hereditary optic neuropathy was common in younger 18 age group (48.58%). In 2226 DON patients, the proportion of neuromyelitis optica(NMO)/neuromyelitis optica spectrum disorder(NMOSD)-optic neuritis(ON)was the highest(60.02%)and myelinoligodendrocyte glycoprotein antibody(MOG-IgG)ON was 11.68%, while multiple sclerosis(MS)-ON and chronic recurrent inflammatory optic neuropathy(CRION)were relatively low(1.8% and 2.25%,respectively). In DON patients, the male to female ratio was 1:3.08. In NMO/NMOSD-ON patients, the ratio of male to female was 1:8, and that of MOG-ON was 4:5. In atypical ON, the ratio of male to female was higher than that of female(1.28:1). In DON patients, 81.79% of patients were young and middle-aged, and the proportion of children with MOG-ON(less than 18 years old)was 41.15%.Conclusions: DON and NAION are the two most common diseases of neuro-ophthalmic afferent system.
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.
目的：回顾性分析以双眼复视为主要症状患者的病因及临床特点。方法：总结2021年1月至2022年3月就诊于潍坊医学院附属医院神经眼科的双眼复视患者的临床资料，分析其病因及临床特点。结果：共29例患者，男16例，女13例，年龄17岁～81岁，平均(59±14)岁；其中血管性因素8例，包括脑血管病5例，后交通动脉瘤2例，核间性眼肌麻痹1例；炎症、免疫性因素8例，包括重症肌无力4例，Tolosa-Hunt综合征2例，肥厚性硬脑膜炎1例，炎性假瘤1例；内分泌因素9例，包括糖尿病外周神经病变5例，甲状腺相关眼病4例；肿瘤2例，包括动眼神经鞘瘤1例，眼眶MALT淋巴瘤1例，外伤2例。结论：双眼复视的病因复杂，临床医生应重视筛查全身疾病，参照先定位，后定性原则，提高诊断正确率、减少误诊率。
Objective: The etiology and clinical characteristics of patients with binocular diplopia as main symptom were investigated using retrospective analysis method. Methods: The clinical data of patients with binocular diplopia treated in department of ophthalmolog y, affiliated hospital of Weifang Medical University from January 2021 to March 2022 was summarized and the etiology and clinical characteristics retrospectively. Results: There were totally 29 patients, 16 males and 13 females, aged from 17 to 81 years, with an average of (59 ± 14) years; among them, there were 8 cases derived from vascular factors, including 5 cases with cerebrovascular disease, 2 cases with posterior communicating artery aneurysm and 1 case with internuclear ophthalmoplegia. There were 8 cases derived from inflammatory and immune factors, including 4 cases with myasthenia gravis, 2 cases with Tolosa-Hunt syndrome, 1 case with hypertrophic meningitis and 1 case with inflammatory pseudotumor. There were 9 cases derived from endocrine factors, including 5 cases with peripheral neuropathy in diabetes and 4 cases with thyroid related ophthalmopathy. There were 2 cases derived from tumors, including 1 case with oculomotor schwannoma, 1 case with orbital MALT lymphoma and there were 2 other cases of trauma. Conclusions: The etiology of binocular diplopia is complicated and the clinicians should pay attention to the screening of systemic diseases of patients refer to the principle of localization diagnosis first and qualitative analysis next so as to improve the diagnostic accuracy and reduce the misdiagnosis rate.
报告一例视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)合并人体免疫缺陷病毒(human immunodeficiency virus,HIV)感染/获得性免疫缺陷综合征(acquired immune deficiency syndrome,AIDS)，并通过文献复习，总结其发病机制、临床特征、治疗及预后。检索文献包括7篇英文文献(8个病例)，1篇中文文献，共报道9例NMOSD合并HIV感染/AIDS病例，结合本文报道的1例共10例，其中5例为女性，5例为男性，3例HIV感染/AIDS为新发，其他病例的HIV感染/AIDS发病均早于NMOSD。临床表现上，7例均为视神经炎和脊髓炎同时或相继发生，2例表现为单相病程或复发性脊髓炎，1例仅表现为双眼相继发生的视神经炎，10例患者头或脊髓MRI均有典型的视神经或脊髓异常信号，伴或不伴强化。2例患者未进行水通道蛋白4(aquaporin protein-4,AQP4)抗体IgG检测，其余8例中5例AQP4抗体阳性、3例阴性。针对AIDS的治疗，10例患者中，8例接受了高效抗逆转录病毒治疗(highly active antiretroviral therapy,HAART)。针对NMOSD的治疗，10例患者中，急性期有8例患者接受糖皮质激素冲击治疗、3例患者接受血浆置换、2例接受丙种球蛋白治疗，序贯治疗期有6例患者接受免疫抑制剂治疗，其中1例因高胆红素血症停药。发生视神经炎的7例中，2例患者经治疗仍失明、5例视力部分恢复，发生脊髓炎的8例中，5例患者遗留截瘫或轻瘫、3例肌力部分恢复。1例因严重并发症去世。NMOSD合并HIV感染/AIDS临床较罕见，预后差，往往遗留严重的视力障碍及瘫痪等，临床治疗较为棘手，糖皮质激素和免疫抑制剂并非使用禁忌证，但制定治疗决策前需要充分考虑风险与获益的平衡。
A case of neuromyelitis optica spectrum disorders(NMOSD) complicated with human immunodeficiency virus(HIV) infection/acquired immunodeficiency syndrome(AIDS) was reported, and the pathogenesis, clinical characteristics, treatment and prognosis were summarized through the literature review. The retrieved literatures included seven English literatures (eight cases) and one Chinese literature, in which a total of nine cases of NMOSD co-infected with HIV infection/AIDS were reported. Combined with the case reported in this paper, the total number of cases was ten, among which five cases were female and five cases were male, three cases of HIV infection/AIDS were newly developed, and the other cases had earlier onset of HIV infection/AIDS than NMOSD. In terms of clinical manifestations, seven cases all had simultaneous or sequential optic nerve and myelitis, two patients presented with a uniphasic course or recurrent myelitis, and one case presented only with bilateral optic neuritis occurring sequentially in both eyes. All ten patients had typical abnormal signals of the optic nerve or spinal cord with or without enhancement on cranial or spinal MRI. Two patients did not undergo AQP4 antibody IgG testing , and of the remaining seven cases, five were positive for AQP4 antibodies and three were negative. For AIDS treatment, eight of the ten patients received highly active antiretroviral therapy(HAART). For NMOSD treatment, among the ten patients, eight patients received intravenous methylprednisolone,three patients received plasmapheresis, and two patients received intravenous immunoglobulin in the acute phase. Six patients received immunosuppressive therapy during the sequential treatment period, and one of them was discontinued due to hyperbilirubinemia. Of the seven cases with optic neuritis, two patients remained blind after treatment and five had partial recovery of vision. Of the eight cases with myelitis, five patients were left with paraplegia or mild paralysis, and three had partial recovery of muscle strength. One case died due to serious complications.NMOSD combined with HIV infection/AIDS is rare in clinic and has a poor prognosis. Patients are often left with severe visual impairment and paralysis. Clinical treatment is quite difficult, hormones and immunosuppressive agents are not considered as contraindications. Treatment decisions need to be made with fully considered about the balance of risks and benefits.
视网膜是中枢神经系统的一部分。在胚胎起源上，视网膜和大脑均由神经管发育而来。因此，许多发生在大脑的神经退行性疾病往往会同时累及视网膜。而神经退行性疾病过程中相关的特征性病理改变，如病理性蛋白聚集和神经血管单元破坏也常能在视网膜组织中被检测到。在一些神经退行性疾病中，眼部的病理改变甚至在临床症状出现之前就已发生；其次视网膜易于观察且局部治疗操作便捷，因此近年来视网膜在中枢神经退行性疾病发病机制研究、早期诊断和新型治疗方式探究等方面备受关注。该文对常见神经退行性疾病的眼部病理改变进行综述，旨在为大脑和视网膜神经退行疾病的发病机制、诊断以及治疗研究提供新的见解。
The retina is a part of the central nervous system. Developmentally, both retina and brain are derived from the neural tube. Therefore, many neurodegenerative diseases that occur in the brain tend to involve both the retina. In the process of neurodegenerative diseases, related characteristic pathological changes, such as pathological protein aggregation, neurovascular unit impairment can often be detected in retinal tissue. In some neurodegenerative diseases, pathological changes in the eye occur even before clinical symptoms appear. In addition, the retina are easy to observe and local treatments are convenient. In recent years, the manifestations of the retina have attracted much attention in the study of pathogenesis, early diagnosis, and new treatments of systemic central neurodegenerative diseases. In this way, this article reviews the ocular pathological changes of common neurodegenerative diseases, aiming to provide new insights into the pathogenesis, diagnosis, and treatment of brain and retinal neurodegenerative diseases.
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.
视网膜神经纤维层是视网膜的最内层，主要由来自视网膜神经节细胞的无髓鞘轴突组成，此外还有神经胶质细胞与视网膜血管，其厚度与年龄、眼球增长、眼底结构改变等因素相关。光学相干断层扫描可以清晰展示角膜、视网膜、脉络膜、视神经等高分辨率断层图像，可以在活体上显示生物学组织的细微结构，在临床与科研中已获得广泛应用。在青光眼视神经病变中，光学相干断层扫描可以发现视野异常前的视网膜神经纤维层损害，已成为青光眼早期诊断与视神经损伤程度检测的重要手段。除视神经病外，越来越多的研究表明许多视网膜血管疾病、神经元变性疾病等视网膜疾病也有视网膜神经纤维层的损伤。探讨视网膜疾病与神经纤维层的关系，将有利于进一步推进对视网膜疾病发病机制及病理改变的认识。本文就视网膜神经纤维层的定量评估与多种视网膜疾病的关系展开综述，为其在视网膜疾病中的应用提供参考。
The retinal nerve fiber layer, the innermost layer of the retina, consists mainly of unmyelinated axons from retinal ganglion cells, as well as glial cells and retinal blood vessels , the thickness of which is related to factors such as age, ocular growth and fundus structure changes. Optical coherence tomography (OCT) can clearly display the cornea, retina, choroid, optic nerve and other high-resolution tomography images. It can show the fine structure of biological tissues in vivo, which has been widely used in clinical and scientific research. In glaucomatous optic neuropathy, OCT can detect the damage of retinal nerve fiber layer before abnormal visual field, which has become an important means of early diagnosis of glaucoma and detection of the degree of optic ner ve damage. In addition to optic neuropathy, more studies have shown that many retinal diseases such as retinal vascular diseases and neurodegenerative diseases also have retinal nerve fiber layer injury. Exploring the relationship between retinal diseases and nerve fiber layer will be beneficial to further promote the understanding of the pathogenesis and pathological changes of retinal diseases. This paper reviews the relationship between the quantitative evaluation of retinal nerve fiber layer and various retinal diseases, and provides reference for its application in retinal diseases.
随着光学相干断层扫描(optical coherence tomography,OCT)的快速发展和广泛应用，视盘周围高反射卵圆形团块样结构(peripapillary hyper-reflective ovoid mass-like structure,PHOMS)已成为神经眼科临床实践中OCT检查的常见征象之一。该结构是生理性改变还是病理性改变目前尚无定论，推测可能与视乳头轴浆瘀滞有关。该文针对PHOMS的形态特征做一综述，总结各种疾病相关的PHOMS，并提出一些针对PHOMS的疑点及研究方向，旨在为临床医生及早辨别PHOMS、早期治疗眼底疾病提供依据。
With the rapid development and widespread application of optical coherence tomography(OCT), peripapillary hyper-reflective ovoid mass-like structure (PHOMS) has become one of the common signs of OCT in neuro-ophthalmic clinical practice. Whether this structure is physiological or pathological has not been determined, and it is speculated that it may be related to the stagnation of the axial plasma of the optic papilla. In this review, we describe the morphological characteristics of PHOMS, summarize various diseases related to PHOMS, and proposes some doubtful points and research directions for PHOMS, aiming to provide evidence for clinicians to identify PHOMS as early as possible and treat fundus diseases in the early stage.
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 synthesisof 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.
近视防控已经上升到我国国家战略层面，高度近视引起的视神经病变会损害视功能，但在临床上常常被忽视。OCT可以非侵入、高分辨率、快速以及可重复地定量视网膜各层厚度，是评估高度近视相关视神经病变的有力工具。由于高度近视常合并视盘和盘周的改变，视神经纤维层厚度的定量常出现误差。近年来，学者开始聚焦于黄斑区神经节细胞复合体(ganglion cell complex,GCC)厚度的研究，但其在高度近视眼中的变化规律尚不统一。该文针对近年来高度近视眼黄斑区GCC的测量规范、诊断价值、变化规律等进行综述，以期提高眼科医师对高度近视视神经病变的重视和研究水平。
Myopia prevention and control has risen to the national strategic level in China. Optic neuropathy caused by high myopia can damage visual function, but it is often ignored in clinical practice Optical coherence tomography (OCT) characterized by non-invasiveness, high resolution, rapid, and repeatable quantifying the thickness of each layer in the retina has emerged as a powerful tool for evaluating high myopia related optic neuropathy. Due to the changes in and near the optic disc in high myopia, errors often occur in the quantification of the thickness of the optic nerve fiber layer. In recent years, researchers have gradually focused on the study of the thickness of ganglion cell complex (GCC), but the regularity of its changes in high myopia is not yet unified. This article reviews the measurement specifications, diagnostic values, and change rules of GCC in the macular region of high myopia in recent years, in order to improve the attention and research level of ophthalmologists on high myopia optic neuropathy.
后视路病变是视交叉以后的视觉通路其本身或毗邻结构发生病变，引起视觉功能改变的一类疾病。神经眼科医生比较熟悉枕叶病变引起的对称性同侧偏盲，但枕极(纹状皮质的最后部分)的病变产生中心性对称性同向盲点，此类视野改变容易被忽略或误诊。该文报道一例老年男性患者，因双眼视觉清晰度下降、视物变形就诊。眼科检查：最佳矫正视力：右眼0.8，左眼1.0，FM-100检查提示重度色觉异常，颅脑磁共振成像(magnetic resonance imaging,MRI)提示双侧枕叶脑梗死(右侧枕极部，左侧纹状皮质前部)，24-2 Humphrey视野检查可见双眼同向暗点趋势(不典型)，10-2 Humphrey视野检查可见双眼中心视野同向偏盲(暗点)，故而确诊。后视路病变可引起多种特征性的视野改变，可伴有高级视功能异常及其他神经系统症状和体征，是神经眼科的重要组成部分。该例枕极脑梗死病变产生对称性同向性盲点伴色觉改变患者的诊治过程，提示需关注后视路病变视野改变的多样性及其他视觉功能异常，提高早期诊断率，改善患者预后。
The disease of the posterior visual pathway is a kind of lesion in which the visual pathway itselfor its adjacent structure changes after optic chiasma causes pathological changes, resulting in changes in visual function. Neuro-ophthalmologists are familiar with symmetrical ipsilateral hemianopia caused by occipital lobe lesions, but occipital tip (the last part of the striatal cortex) lesions produce central symmetrical homonymous scotomas, which can easily be overlooked or misdiagnosed. This article reported a case of an olderly male patient treated with decreased binocular visual clarity and distortion. Ophthalmology examination: best corrected visual acuity: 0.8 in the right eye, 1.0 in the left eye; FM-100 examination indicated severe dyschromatopsia; cranial magnetic resonance imaging: infarction of bilateral occipital lobe (right portion of the occipital tip and left anterior portion of striate cortex); 24-2 Humphrey field examination showed a tendency of homonymous scotoma in bilateral eyes (atypical); 10-2 Humphrey field examination showed homonymous hemianopia (scotoma) in the central visual field. These results confirm a diagnosis of the disease of the posterior visual pathway. As an important part of neuro-ophthalmology, the posterior visual pathway can cause various characteristic visual field defects, which can be accompanied by advanced visual dysfunction and other neurological symptoms and signs. The diagnosis and treatment process of this case of occipital tip cerebral infarction with symmetrical homonymous blind spot accompanied by color vision changes suggests that attention should be paid to the diversity of visual field changes and other visual functional abnormalities in the posterior visual pathway lesions, so as to improve the early diagnosis rate and prognosis of the patient s.
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.
非器质性视力下降也称为心因性或功能性视力下降，除视力下降外，还可伴有视野缺损，多由于精神心理疾患导致的转换障碍引起，部分患者为诈病以获取利益。本文报道1例6岁的女性患者，主诉双眼反复视力下降1年余，早期被误诊为儿童视神经炎，给予糖皮质激素冲击治疗，治疗后稍有好转。通过本例患者误诊的教训，提醒我们在遇到儿童出现不明原因的视力下降时，在没有明确器质性疾病证据时要想到非器质性视力下降的可能，掌握识别非器质性视力下降的检查方法，不能忽略相对性传入性瞳孔障碍等基础的神经眼科检查。
Non-organic vision loss is also known as psychogenic or functional vision loss. In addition to vision loss, it can also be accompanied by visual field defect. It is mostly caused by conversion obstacles caused by mental and psychological diseases. Some patients cheat to obtain benefits. This paper reports a 6-year-old female patient who complained of repeated visual acuity decline for more than one year. She was misdiagnosed as pediatric optic neuritis in the early stage and was treated with glucocorticoid shock therapy, which her condition improved slightly after treatment. The misdiagnosis of this patient teaches us that when children have unexplained visual acuity decline, we should think of the possibility of non-organic visual acuity decline when there is no clear evidence of organic diseases, master the examination methods to identify non-organic visual acuity decline, and cannot ignore the basic neuro-ophthalmic examination such as relative afferent pupillary defect (RAPD).
