目的：调查神经眼科用药现状，并对其超说明书用药进行评价，探索科学有效的管理策略，为神经眼科的临床合理用药提供参考。方法：随机抽取2023年7—12月郑州市第二人民医院神经眼科处方，依据国家药品监督管理局核准的药品说明书、美国食品药品监督管理局(Food and Drug Administration, FDA)说明书及PubMed、Micromedex等循证医学数据库证据判断超说明书用药类型，并对其进行分类(包括超适应证、超剂量、超疗程、超给药途径等)。同时基于Thomson分级理论建立三级评价标准，分别为A级(高证据等级，仅需口头告知)，B级(中证据等级，需在病历或病程中详细记录)，C级(低证据等级，须患者签署书面知情同意书)。通过处方前置审核系统干预，对未纳入评价标准的超说明书用药进行实时拦截与分级警示，比较2023年下半年(干预前)与2024年下半年(干预后)超说明书用药处方的不合理率变化。 结果：干预前共抽取超说明书处方370张，超说明书类型主要为超适应证、超给药途径和超剂量及次。干预后，共抽取超说明书用药560张，超说明书用药不合理率从9.2%(34/370)降至2.9%(16/560)。结论：神经眼科超说明书用药普遍，主要源于视神经炎、缺血性视神经病变等复杂疾病的治疗需求与药品审批滞后之间的矛盾。基于循证医学证据构建分级管理体系，结合信息化实时干预，可有效规范超说明书用药行为，降低医疗风险。

Objective: To investigate the current status of medication use in neuro-ophthalmology, evaluate off-label drug use, and explore scientific and effective management strategies, providing references for rational drug use in neuro-ophthalmology clinical practice. Methods: We Randomly selected neuro-ophthalmology prescriptions from July to December 2023 in our hospital. By refering to the drug instructions approved by the National Medical Products Administration, FDA instructions, and evidence from evidence-based medical databases such as PubMed and Micromedex, we identified and classified the types of off-label drug use, including off-label indications, off-label doses, off-label courses, off-label administration routes, among others. Simultaneously, based on the Thomson classification theory, we established a threelevel evaluation standard: Level A (high evidence level, requiring only oral notification); Level B (medium evidence level, necessitating detailed records in the medical record or medical course); Level C (low evidence level, demanding patients sign a written informed consent form). Through the intervention of pre-prescription review system, we carried out real-time interception and classification warnings for off-label drug use not covered in the evaluation standards. We then compared the changes in the unreasonable rate of off-label drug use prescriptions from the second half of 2023 (before intervention) to the second half of 2024 (after intervention). Results: Before the intervention, a total of 1852 prescriptions were selected, with the proportion of off-label drug use at 19.98% (370 prescriptions), primarily involving off-label indications (90.27%). After the intervention, the unreasonable rate of off-label drug use decreased from 9.2% (34/370) to 2.9% (16/560). Conclusions: Off-label drug use is prevalent in neuro-ophthalmology, mainly due to the conflict between the treatment requirements for complex diseases such as optic neuritis and ischemic optic neuropathy and the delay in drug approval. By constructing a hierarchical management system based on evidence-based medical evidence and combing it with real-time information-based intervention, we can effectively regulate off-label drug use behavior and reduce medical risks.

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：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.