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.
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.
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.
目的: 分析甲醇中毒性视神经病变的临床特征、影像学动态演变及治疗反应，探讨中毒程度、治疗时机与视功能预后的相关性，为优化临床诊疗路径提供循证依据。方法: 采用病例系列研究与文献回顾方法，回顾性纳入2017—2024年在中山大学中山眼科中心确诊的9例(18只眼)甲醇中毒性视神经病变患者，收集其临床资料包括瞳孔变化、眼底照相、光学相干断层成像(optical coherence tomography; OCT)、视野、视觉诱发电位(visual evoked potential; VEP)等，结合文献整合病例数据。纳入标准包括明确甲醇暴露史、急性视力下降伴视盘水肿或视网膜神经纤维层(retinal nerve fiber layer; RNFL)增厚、血/尿甲醇或甲酸浓度升高。排除既往视力不佳或视网膜病变者。通过多模态影像学评估视神经损伤特征，分析治疗反应及预后相关因素。结果: 9例(18只眼)患者年龄为23~51岁(男性7例，女性2例)，中毒后就诊时间为3 d~3个月。最佳矫正视力为无光感(11眼)至1.0，66.7%(12/18眼)出现瞳孔变化，眼底表现为视盘水肿，VEP提示振幅下降伴视野缺损，OCT显示急性期视盘区域RNFL增厚，慢性期进行性萎缩、杯盘比显著增大。经治疗后，12只眼视力提高(随访2个月，4只眼中毒后视力为1.0，未纳入其中)，持续随访(4个月~3年)的患者中，多数患者急性期干预后进入视力平台期，但少数患者仍存在迟发性神经退行风险。因此，部分患者通过早期干预联合全身激素冲击、血液透析、营养神经等治疗可能有助于改善视功能，而部分延迟治疗患者可出现不可逆视力丧失。瞳孔对光反射保留者预后更佳。结论: 甲醇中毒性视神经病变的预后与中毒程度、干预时机及瞳孔反射状态密切相关。早期给予激素冲击联合营养神经治疗可能改善部分患者的预后，但中毒时间长者易遗留不可逆视神经损伤，个性化分期治疗及长期视功能监测对保护残余视力至关重要。
Objective: To analyze the clinical characteristics, dynamic imaging changes, and treatment responses of methanol-induced toxic neuropathy. Additionally, it sought to explore the correlation between poisoning severity, intervention timing, and visual functional outcomes, so as to evidence-based insights for optimizing clinical diagnosis and treatment strategies. Methods: A case-series study combined with a literature review was conducted. Nine patients (18 eyes) diagnosed with methanol-induced toxic neuropathy at Zhongshan Ophthalmic Center, Sun Yat-sen University from 2017 to 2024, were retrospectively included. Clinical data, including pupillary changes, fundus photography, optical coherence tomography (OCT), visual field tests, and visual evoked potentials (VEP), were collected and integrated with findings from the literature. The inclusion criteria were a confirmed history of methanol exposure, acute vision loss accompanied by optic disc edema or retinal nerve fiber layer (RNFL) thickening, and elevated blood/urine methanol or formic acid levels. Patients with pre-existing poor vision or retinal diseases were excluded. Multimodal imaging was employed to evaluate the characteristics of optic nerve injury, and treatment responses and prognostic factors were analyzed. Results: The nine patients (18 eyes) ranged in age from 23–51 years (7 males, 2 females), with the time from poisoning to consultation spanning from 3 days to 3 months. The best-corrected visual acuity (BCVA) ranged from no light perception (NLP, 11 eyes) to 1.0. Pupillary abnormalities were observed in 66.7% (12/18 eyes) of the cases. Fundus examination showed optic disc edema, while VEP revealed reduced amplitude and visual field defects. OCT demonstrated RNFL thickening in the acute phase and progressive atrophy with an increased cup-to-disc ratio (C/D) in the chronic phase. After treatment, 12 eyes showed visual improvement (followed at 2 months; 4 eyes with post-poisoning BCVA of 1.0 were excluded from this analysis). Among patients with extended follow-up (4 months to 3 years), most reached a visual acuity plateau after acute-phase intervention, although a minority remained at risk of delayed neurodegenerative decline. Early intervention with systemic high-dose steroid therapy, hemodialysis, and neuroprotective treatment might improve visual function in some patients, whereas delayed treatment often led to irreversible vision loss. Patients with preserved pupillary light reflexes had more favorable prognoses. Conclusions: The prognosis of methanol-induced optic neuropathy is closely related to poisoning severity, intervention timing, and pupillary reflex status. Early high-dose steroid therapy combined with neuroprotective treatment may enhance outcomes in some patients; however, prolonged poisoning typically results in irreversible neurovisual damage. Personalized staged treatment and long-term visual function monitoring are essential for preserving residual vision.
目的：探讨外伤性视神经病变(traumatic optic neuropathy，TON)患者内镜下经蝶筛径路视神经管减压术(endoscopic trans-ethmosphenoid optic canal decompression，ETOCD)的整体护理。方法：选取中山大学中山眼科中心2020年1月至2021年3月收治的80例TON患者，回顾总结患者 ETOCD期间的护理措施及手术疗效。结果：所有患者经过综合护理后均顺利完成手术，未发生感染，出血、疼痛情况经治疗和护理后均改善，68.8%患者术后视力有提高。结论：针对TON患者ETOCD的特点，采取个体化的整体护理具有重要意义，有利于帮助患者顺利完成手术，降低并发症的发生率，促进患者康复。
Objective: To investigate the holistic nursing care of patients with traumatic optic neuropathy undergoing endoscopic trans-ethmosphenoid optic canal decompression (ETOCD). Methods: A total of 80 patients with traumatic optic neuropathy admitted to Zhongshan Ophthalmology Center of Sun Yat-sen University from Jan 2020 to Mar 2021 were selected as the subjects, and the nursing measures and surgical effect during ETOCD were reviewed and summarized. Results: All 80 surgical patients successfully completed the operation after comprehensive nursing without infection. The bleeding and pain were improved after treatment and nursing, and 68.8% patients presented with vision improvement. Conclusion: According to the characteristics of ETOCD in patients with traumatic optic neuropathy, it is of great significance to take individualized overall care, which is beneficial to help patients successfully complete the operation, reduce the incidence of complications, and promote the recovery of the patient’s healthy.
