目的：回顾2型神经纤维瘤病(neurofibromatosis type 2, NF2)患者的眼部表现，分析NF2眼部病变的临床和影像学特征，以助该病的早期诊断。方法：收集来自深圳市眼科医院的1例和来自中山眼科中心的3例NF2患者完整的临床资料并进行总结分析。结果：这4例患者均因眼部异常首诊于眼科且符合曼彻斯特诊断标准。4例患者中，3例为NF2早发型(<20岁)，1例为晚发型(>20岁)，男女比例1∶1。3例患者因视力下降、1例患者因复视首诊于眼科。3例行眼底光学相干断层成像(optical coherence tomography, OCT)检查显示，视网膜错构瘤2例，视网膜前膜2例，视盘隆起2例，视网膜神经纤维层和节细胞层变薄1例。其他表现包括麻痹性斜视2例，复视1例，白内障1例，球后段视神经增粗1例，眼眶肿瘤1例。结论：NF2的眼部表现多种多样，可出现在神经症状和听力损失之前。详细的眼科检查及影像学检查对年轻患者的早期诊断非常有价值，有助于选择更好的治疗计划。

Objective: To review the ocular manifestations of patients with neurofibromatosis type 2 (NF2), and analyze the clinical and imaging features of the ocular lesions in NF2, so as to facilitate the early diagnosis of this disease. Methods: The complete medical records of 1 case from Shenzhen Eye Hospital and 3 cases of NF2 from Zhongshan Ophthalmic Center were collected. Results: All four patients were first diagnosed in ophthalmology due to eye symptoms and met the Manchester diagnostic criteria. Of the four patients, three were NF2 early hairstyles (<20 years old), one was late hairstyle (>20 years old), and the male-to-female ratio was 1:1. Three patients were first diagnosed in the ophthalmology department due to decreased visual acuity, and one patient was first diagnosed due to diplopia. Optical coherence tomography (OCT) examinations were performed on three patients, which showed that there were two cases of retinal hamartoma, two cases of epiretinal membrane, two cases of optic disc elevation, and one case of thinning of the retinal nerve fiber layer and ganglion cell layer. Other manifestations included two cases of paralytic strabismus, one case of diplopia, one case of cataract, one case of thickening of the retrobulbar optic nerve, and one case of orbital tumor. Conclusions: The ocular manifestations of NF2 are diverse and can precede neurological symptoms and hearing loss. Detailed ophthalmic examinations and imaging studies are highly valuable for early diagnosis in young patients, aiding in the selection of a better treatment plan.

目的：设计一套瞳孔动、静态信息提取方案，尝试初步建立正常人群瞳孔动、静态信息数据库。方法：于2023年1月—7月，从中山大学眼科中心眼科门诊招募无眼部疾病、全身疾病的参与者。使用工业级红外相机搭配850 nm红外光源录制参与者瞳孔区域20 s视频，提取瞳孔横径数据并输出为txt文件。使用R语言分析数据，构建瞳孔舒缩过程中的波峰、波谷拟合线，并估算瞳孔舒缩频率。结果：共收集32位参与者的瞳孔动态数据，右眼数据纳入主要结果的分析。参与者年龄范围为7~61岁，男性占50%，等效球镜度范围为-5.630~+5.00 D。测量过程中的瞳孔横径平均值与年龄呈负相关，与等效球镜度、性别无关。在20 s的记录中，瞳孔舒缩次数为(15±7)次。基于波峰与波谷拟合线的趋势，瞳孔舒缩动态分为3种类型：舒缩扩张型、舒缩稳定型和舒缩缩小型。舒缩稳定型进一步分为稳定扩张型、稳定恒定型和稳定缩小型3个亚型，其中25人为舒缩缩小型。双眼的过滤后波峰、波谷拟合线的斜率和截距与过滤前比较差异无统计学意义(P>0.05)。结论：低成本、简易的设备配以算法可以便捷、快速地提取瞳孔动、静态信息。

Objective: To design a method for extracting dynamic and static information of the pupil and to establish a database of pupil dynamic and static information in a healthy population. Methods: From January to July 2023, subjects without any ocular or systemic diseases were recruited from the ophthalmology outpatient department. An industrial-grade infrared camera, paired with an 850nm infrared light source, was used to record 20-second videos of the pupil area of each subject. Horizontal pupil diameter data was extracted and saved as txt files. The data was analyzed using R software to construct fitted lines of peaks and troughs during the pupil constriction and dilation process, and the frequency of pupil contraction and dilation was estimated. Results: Pupil dynamic data was collected from 32 subjects with an age range of 7 to 61 years, of whom 50% were male. The spherical equivalent range was from +5.00 D to -5.625 D. The average number of pupil contractions and dilations within the 20-second recordings was (15 ± 7) cycles. Based on the trend of fitted lines for peaks and troughs, pupil dynamic types were categorized into three types: dilation type, stable type, and contraction type. The stable type was further divided into stable dilation, stable constant, and stable contraction subtypes. 25 subjects exhibiting the constriction type. A paired t-test showed no significant difference in the slope and intercept of the fitted lines for peaks and troughs between both eyes. Conclusion: Low-cost and simple equipment combined with algorithms can efficiently and quickly extract dynamic and static pupil information.

目的：评估EYESi手术模拟器在眼科医师专科培训玻璃体手术中的教学效果。方法：招募中山大学中山眼科中心进行专科医师规范化培训的15名学员，开展眼底手术培训课程，随机分为A组、B组、C组，每组各5名学员。以培训开始前进行初次视网膜脱离手术作为基础分数，除玻璃体后脱离项目、视网膜前膜及内界膜项目等基础练习内容外，A组仅接受双手剪刀训练，B组仅接受激光训练，C组同时接受双手剪刀训练及激光训练。最后所有学员再进行视网膜脱离手术项目作为最终考核成绩。记录下学员的每次得分和操作时间。结果：培训后学员视网膜脱离手术得分较培训前提高（P<0.001）。C组与A组学员最终视网膜脱离手术得分高于B组学员（P<0.05）。有内眼手术经验的学员初次视网膜脱离手术得分高于无内眼手术经验的学员，且手术时长低于无内眼手术经验的学员（P<0.05）。结论：EYESi手术模拟器能有效提高眼科医师的手术操作信心及玻璃体视网膜手术操作技能，双手剪刀训练模块是玻璃体视网膜手术培训中的重要模块。

Objective: To assess the instructional efficacy of the EYESi surgical simulator in training ophthalmology residents on vitreoretinal surgical procedures. Methods: Fifteen ophthalmology residents participating in standardized specialist training program at Zhongshan Ophthalmic Center, Sun Yat-sen University, were randomly assigned to three groups (A, B, C; n=5 per group). All participants underwent an initial baseline assessment through simulated retinal detachment surgery. In addition to standard training modules covering posterior vitreous detachment, anterior retinal membrane peeling, and inner limiting membrane manipulation, Group A received supplementary bimanual scissors training, Group B underwent laser training, and Group C received both types of advanced training. The final assessments were carried out using the same retinal detachment simulation, with performance scores and operation times carefully recorded. Results: Post-training surveys revealed significant increases in both trainees’ self-confidence and their self-assessed surgical proficiency. Objective performance scores for retinal detachment surgery showed marked improvement after training (P < 0.001). Groups C and A achieved significantly higher final scores compared to Group B (P < 0.05). Participants with prior intraocular surgical experience scored higher and completed procedures more quickly in the baseline assessment than those without such experience (P < 0.05). Conclusions: The EYESi simulator proves to be an effective tool for enhancing surgical skills and boosting operational confidence in vitreoretinal surgery training. In particular, bimanual instrumentation training offers significant educational value in the development of advanced microsurgical techniques.

目的：探讨蛋白A免疫吸附联合糖皮质激素治疗对MOG抗体相关视神经炎（MOG antibody-associated optic neuritis， MOG-ON）患者的临床疗效及安全性。方法：回顾性分析2022年6月—2024年12月在广东三九脑科医院神经内科确诊并接受蛋白A免疫吸附联合糖皮质激素治疗的7例MOG-ON患者。所有患者均接受蛋白A免疫吸附治疗（隔天1次，共5次为1个疗程）并同期联合糖皮质激素治疗。评估治疗前及治疗后3个月、6个月的视力变化、扩展伤残状态量表（expanded disability status scale，EDSS）评分变化及MOG抗体滴度变化，并记录不良反应。结果：治疗后6个月，6/7例患者视力较治疗前改善，其中4/7例视力改善显著。左眼logMAR视力值从治疗前的0.20(0.14，0.70)改善至0.10(0.10，0.42)，右眼logMAR视力值从0.30(0.19，0.47)改善至0.18(0.10，0.21)，EDSS视力评分从2.86±1.68降至1.43±1.51（P < 0.05）。治疗前血清MOG抗体滴度几何平均数为1:52.0（几何标准差GSD = 3.7），治疗后3个月降至1:8.8（GSD = 1.9）（P = 0.027），治疗后6个月降至1:13.0（GSD = 4.1）（P = 0.027）。7例患者共接受35次免疫吸附治疗，未观察到严重不良反应，仅有轻微可控的不良事件。结论：蛋白A免疫吸附联合糖皮质激素治疗能够有效降低血液中MOG抗体水平，改善MOG-ON患者的视力。

Objective: To investigate the clinical efficacy and safety of protein A immunoadsorption combined with glucocorticoid therapy in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis（MOG-ON）. Methods: A retrospective analysis was conducted on 7 patients with MOG-ON who were diagnosed and treated with protein A immunoadsorption combined with glucocorticoid therapy at the Department of Neurology，Guangdong Sanjiu Brain Hospital from June 2022 to December 2024. All patients underwent protein A immunoadsorption therapy （once every other day, with 5 sessions constituting one course） in conjunction with concurrent steroid therapy. Visual acuity changes, EDSS score changes, and MOG antibody titer changes were assessed before treatment, as well as at 3 and 6 months after treatment. Additionally, adverse events were meticulously recorded. Results: At the 6 months post-treatment mark, 6 patients （85.7%） demonstrated an improvement in visual acuity compared to their baseline levels, with 4 patients （57.1%） achieving a significant improvement. The median logMAR visual acuity values in the left eye  improved from 0.20(0.14,0.70) to 0.10(0.10,0.42), and in the right eye, they improved from 0.30(0.19,0.47) to 0.18(0.10,0.21). MeanWhile, the EDSS visual score decreased from 2.86±1.68 to 1.43±1.51（P < 0.05）. The geometric mean serum MOG antibody titer declined from 1:52.0（GSD = 3.7） before treatment to 1:8.8（GSD = 1.9） at 3 months after treatment（P = 0.027）, and further decreased to 1:13.0（GSD = 4.1） at 6 months after treatment（P = 0.027）. A total of 35 immunoadsorption sessions were administered to the 7 patients, and no serious adverse reactions were observed; only minor and manageable adverse events occurred. Conclusion: Protein A immunoadsorption combined with glucocorticoid therapy can effectively lower serum MOG antibody levels and enhance visual outcomes in patients with MOG-ON.

帕金森病（Parkinson’s disease, PD）作为仅次于阿尔茨海默病的第二大神经退行性疾病，其眼部表现近年来逐渐成为跨学科研究热点。以往医生多关注运动迟缓、静止性震颤和肌强直等PD典型症状，但大量临床研究表明，眼睑异常、眼球运动障碍、视觉功能异常等眼部表现不仅普遍存在于PD患者中，更可能在典型运动症状出现前就已显现。长期以来，这些眼部症状因其他症状的掩盖往往被忽视，进一步降低了患者的生活质量。本综述系统梳理PD患者眼部表现的三大方面：首先，眼睑异常方面，PD患者瞬目频率降低，61.1%患者出现干眼症状，导致PD患者的生活质量进一步下降。其次，眼球运动障碍表现为特征性的阶梯式方波急跳、集合功能减退以及反向扫视错误率增加，其中反向扫视潜伏期延长对步态冻结的发生具有预测价值。最后，视觉功能障碍方面，PD患者可出现视敏度下降、色觉异常、对比敏感度受损和视幻觉。影像学检查观察到视网膜神经节细胞层变薄，伴随视网膜微血管密度降低，这些结构性改变与PD患者的视觉功能障碍有关，作为生物标志物具有独特潜力。神经内科-眼科联合诊疗模式不仅有助于PD的早期诊断和预后评估，更有助于临床医生全面理解PD的疾病机制和表现，为未来诊疗策略的优化提供客观依据。

Parkinson’s disease (PD), the second most common neurodegenerative disorder after Alzheimer’s disease, has increasingly garnered interdisciplinary research attention due to its ocular manifestations. While the classical triad of motor symptoms—bradykinesia, resting tremor, and rigidity—remains the diagnostic hallmark, accumulating clinical evidence indicates that ocular abnormalities, including eyelid dysfunction, oculomotor disturbances, and visual impairments, are not only prevalent in PD patients but may also precede the onset of typical motor symptoms. Historically overlooked due to masking by other clinical features, these ocular manifestations contribute to the deterioration of patients' quality of life. This review systematically examines PD-related ocular abnormalities across three key domains: First, eyelid dysfunction manifests as reduced blink frequency, with 61.1% of PD patients reporting dry eye symptoms, further exacerbating their life quality impairment. Second, oculomotor disturbances are characterized by staircase-pattern square-wave jerks, convergence insufficiency, and increased error rates in antisaccade tasks, with prolonged antisaccade latency serving as a predictive marker for freezing of gait. Third, visual dysfunction encompasses diminished visual acuity, dyschromatopsia, impaired contrast sensitivity, and visual hallucinations. Imaging studies reveal structural alterations such as retinal ganglion cell layer thinning and reduced retinal microvascular density, which correlate with visual deficits and hold promise as potential biomarkers. The establishment of a neuro-ophthalmological collaborative framework not only facilitates early PD diagnosis and prognostic assessment but also enhances clinicians' comprehensive understanding of disease mechanisms. Such an approach provides an objective foundation for optimizing future therapeutic strategies.

        本文报告两例单眼无痛性视力下降病例。病例1为47岁男性，表现为右眼亚急性视力下降，伴视盘水肿及黄斑区星芒状渗出，血清汉塞巴尔通体（Bartonella henselae）IgG抗体阳性（滴度1:256），结合猫接触史及跳蚤叮咬史，诊断为猫抓病相关视神经视网膜炎。经口服多西环素联合球周注射曲安奈德治疗后，视力显著恢复至1.0。病例2为33岁男性，表现为右眼急性视野缺损伴轻度视力下降，视盘水肿伴出血及棉绒斑。血清学检查示汉塞巴尔通体IgG阳性（滴度1:256）及梅毒螺旋体抗体阳性（TPPA+, TRUST+）。初始经验性抗猫抓病（多西环素+利福平）及抗梅毒（苄星青霉素）治疗无效，视力持续下降。随访1个月时光学相干断层扫描（OCT）显示视盘周围视网膜色素上皮（RPE）局灶性钉状突起，符合梅毒眼部特征性改变，结合患者抗汉塞巴尔通体治疗反应不佳，最终修正诊断为梅毒性视神经病变。继续抗梅毒治疗后视力稳定于0.63，但遗留视神经萎缩。两例病例提示感染性视神经病变的诊断需综合病史、临床表现、辅助检查、治疗反应及特征性体征进行鉴别，尤其需审慎解读汉塞巴尔通体抗体血清学阳性结果。

This paper reports two cases of unilateral painless vision loss. Case 1 involved a 47-year-old male presenting with subacute vision loss in the right eye, accompanied by optic disc edema and macular stellate exudates. Serological testing revealed positive IgG antibodies against Bartonella henselae (titer 1:256). Combined with a history of cat contact and flea bites, a diagnosis of cat scratch disease-associated optic neuroretinitis was made. After treatment with oral doxycycline combined with periocular triamcinolone acetonide injection, the patient's vision significantly improved to 1.0. Case 2 involved a 33-year-old male presenting with acute visual field defect in the right eye accompanied by mild vision loss, optic disc edema with hemorrhage, and cotton-wool spots. Serological tests showed positive IgG antibodies against Bartonella henselae (titer 1:256) and positive antibodies against Treponema pallidum (TPPA+, TRUST+). Initial empirical treatment for cat scratch disease (doxycycline + rifampicin) and syphilis (benzathine penicillin) was ineffective, with continued vision decline. Optical coherence tomography (OCT) at the one-month follow-up revealed focal spiculated protrusions of the retinal pigment epithelium (RPE) around the optic disc, consistent with characteristic ocular changes of syphilis. Considering the patient's poor response to anti-Bartonella henselae treatment, the diagnosis was revised to syphlitic optic neuropathy. After continued anti-syphilis treatment, the patient's vision stabilized at 0.63, but optic atrophy persisted. These two cases suggest that the diagnosis of infectious optic neuropathy requires comprehensive differentiation based on medical history, clinical manifestations, auxiliary examinations, treatment responses, and characteristic signs, with particular caution in interpreting positive serological results for Bartonella henselae antibodies.

        文章报道了一例5岁女性Dandy-Walker综合征（Dandy-Walker syndrome，DWS）患儿的临床表现、眼科检查及影像学特征。该患儿在确诊DWS后行后颅窝蛛网膜囊肿部分切除术，然而术后视力仍进行性下降，眼科随访过程中发现视网膜神经纤维层（Retinal Nerve Fibers Layer，RNFL)进行性变薄，提示颅内压尚未有效控制，视神经损害在进一步发展。由于DWS病变位置特殊性，头颅核磁共振成像（Magnetic Resonance Imaging，MRI）及常规腰椎穿刺未能确定幕上颅内高压，后经侧脑室直接测压，证实仍然存在颅压异常增高。行右侧脑室腹腔分流术后，患儿双眼视力有所提高，双眼RNFL）厚度无继续变薄。目前DWS的治疗方式包括后颅窝囊肿切除术、脑脊液分流术及内镜下第三脑室造瘘术。 术后DWS患者的颅内压的测量有特殊性，幕上与幕下区域之间的脑实质内压力可能形成显著的压力梯度，腰椎穿刺测压可能无法准确反映颅内压，脑室内测压方法则创伤更大。此时需要眼科检查提供支持颅内高压的证据，如RNFL厚度进行性下降、视盘水肿及视网膜血管形态异常，这些检查无创、简便、可多次重复，是临床医生评估颅内压的有效手段，建议纳入DWS患者术后颅内压的常规管理中。

This article reports the clinical presentation, ophthalmologic examination, and imaging features of a 5-year-old girl with Dandy-Walker syndrome (DWS). She underwent partial resection of an arachnoid cyst in the posterior cranial fossa after the diagnosis of DWS. However, her visual acuity continued to deteriorate after the surgery, and the ophthalmologic follow-up revealed progressive thinning of the retinal nerve fibers layer (RNFL), suggesting that the intracranial pressure (ICP) had not yet been effectively controlled, and the damage to the optic nerve was further developing. However, due to the special pathological changes of DWS, MRI and routine lumbar puncture was not able to exactly identify the supratentorial ICP, subsequent ICP measurement through the lateral ventricle confirmed that increased ICP was still existed. After performing a right ventriculoperitoneal shunt, the child's binocular vision improved, and the thickness of the RNFL in both eyes remained stable. Current treatment modalities for DWS include cystectomy, cerebrospinal fluid shunt and endoscopic third ventriculostomy. The measurement of postoperative ICP in DWS patients is complicated, because there may be a significant pressure gradient between the supratentorial and infratentorial regions, which is not accurately reflected by lumbar puncture manometry, and intraventricular manometry is much more invasive. Ophthalmologic examinations including progressive decrease in RNFL thickness, optic disc edema, and abnormal retinal vascular morphology are required to provide evidences of increased ICP. These examinations are noninvasive, simple, and repeatable. They are an effective means of assessing ICP, and are recommended to be included in the routine management of ICP in patients with DWS after surgery.

目的：通过真实的临床病例，分析青光眼与非青光眼性视神经病变的不同表现，提高疾病的鉴别诊断能力。方法：采用病例研究与文献回顾方法，记录3例易误诊为正常眼压性青光眼（normal tension glaucoma, NTG）的神经眼科病例，通过视力、眼压、瞳孔对光反应、视盘形态、RNFL厚度、视野损害等特点，抽丝剥茧，得出诊断。结果：病例1视野呈弓形缺损，但视盘凹陷不深，且对侧眼“视盘拥挤”，随访期间发生急性前部缺血性视神经改变(anterior ischaemic optic neuropathy, AION)，最终确诊“AION遗留视神经萎缩”。病例2视力下降、视野缺损与视神经结构损害不相符，经完善MRI确诊为“左侧视神经鞘膜瘤”。病例3青年男性，中心视力下降，视盘凹陷不深，根据DNA结果确诊Leber遗传性视神经病变（Leber hereditary optic neuropathy, LHON）。结论：通过几个典型的神经眼科病例，掌握AION、视神经鞘膜瘤、LHON的结构和功能损害特征，如视盘凹陷不深、中心视力下降、视神经结构损害与视功能不相符，与NTG相鉴别。

Objective: To analyze the differentiating manifestations of glaucomatous and non-glaucomatous optic neuropathies using real clinical cases and to enhance the ability to make differential diagnosis. Methods: By using case study and literature review methods, we recorded three neuro-ophthalmic cases that were prone to being misdiagnosed as normal tension glaucoma (NTG). The diagnosis was established by extracting features related to visual acuity, intraocular pressure, pupillary light reflex, optic disc morphology, retinal nerve fiber layer (RNFL) thickness and visual field damage. Results: Case 1 presented with arcuate visual field defects, shallow optic disc cupping, and contralateral crowded discs. During follow-up, the patient subsequently developed acute anterior ischemic optic neuropathy (AION), and the final diagnosis confirmed was confirmed as optic atrophy secondary to AION. Case 2 exhibited a disproportionate visual acuity decline and visual field defects that were inconsistent with glaucomatous structural damage. MRI confirmed the diagnosis of left optic nerve sheath meningioma. Case 3 involved a young male who presented with central vision loss and shallow optic disc cupping. Genetic testing confirmed Leber's hereditary optic neuropathy (LHON). Conclusions: Through these representative neuro-ophthalmology cases, we illustrate the characteristic patterns of structural and functional damage associated with  AION, optic nerve sheath meningioma, and LHON, such as shallow optic disc cupping, central vision loss, and inconsistency between  optic nerve structural damage and visual function. These findings highlight the key differentiating features of these conditions from NTG.

视神经脊髓炎谱系疾病（neuromyelitis optica spectrum disorder, NMOSD）是一种罕见的中枢神经系统自身免疫性疾病，主要表现为视神经炎和纵向广泛性横贯性脊髓炎，复发率高，反复发作可导致不可逆的神经功能损害。文章报告了一例44岁女性的NMOSD患者，以反复发作的视神经炎为特征，经过长达11年的随访，共历经4次临床发作。患者首次发作时，因治疗过程中糖皮质（激素）减量过快和自行停药导致病情恶化，后续发作中采用了规范的急性期治疗和缓解期预防复发方案，包括大剂量激素冲击、免疫抑制剂（硫唑嘌呤、吗替麦考酚酯）和静脉注射免疫球蛋白（intravenous immunoglobulin, IVIG）等，显著延长了缓解期。最近一次复发中，患者首次使用了新型生物制剂伊奈丽珠单抗（CD19单克隆抗体）预防复发治疗。文章通过具体病例分析和文献综述，反映NMOSD急性期和缓解期预防复发治疗策略的时代变迁，并探索新型生物制剂使用过程中需要注意的问题。

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease that affects the central nervous system. It is predominantly characterized by optic neuritis and longitudinally extensive transverse myelitis. Given its high relapse rate, repeated attacks of NMOSD can result in irreversible nerve damage. The article details the case of a 44-year-old female patient with NMOSD, whose primary manifestion was recurrent optic neuritis.The patient was followed up over an 11-year span, during which she experienced a total of four clinical relapses. During the initial attack, the patient's condition deteriorated because of the rapid tapering of steroid therapy and her self-initiated discontinuation of medication. In subsequent attacks, standardized treatments for the acute phase and strategies for preventing relapses were implemented. These included high-dose steroid pulses, immunosuppressants (azathioprine, mycophenolate mofetil), and intravenous immunoglobulin (IVIG). These interventions significantly prolonged the remission period. During the most recent relapse, the patient received treatment with the novel biologic agent inebilizumab, which is  a CD19 monoclonal antibody. Through a comprehensive case analysis and a review of relevant literature , this report sheds light on the evolving treatment strategies for managing the acute phase and preventing relapses during remission in NMOSD. It also highlights key considerations in the practical application of the new biologic therapies.