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.
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.
目的：系统分析脑卒中后视野缺损患者干预方案的相关研究，识别、归纳及总结干预的具体内容、结局指标和干预效果，为临床实践及未来该领域研究提供参考。方法：采用范围综述研究框架，系统检索中国知网、维普数据库、万方数据库、中国生物医学文献数据库、PubMed、Web of Science、Embase、CINAHL、Cochrane Library共9个数据库。检索时限为建库至2024年4月10日。对纳入文献进行筛选、汇总和分析。结果：最终纳入12篇文献，总结脑卒中后视野缺损患者干预方法及结局指标，干预方法包括替代性干预、补偿性干预、恢复性干预等，结局指标包括日常生活活动能力、日常生活扩展活动能力、阅读表现、视野检查等。结论：目前针对脑卒中后视野缺损患者的干预方案内容多样化、证据质量较低、结局指标不统一，有待进一步开展高质量研究探索最佳训练计划和规范结局指标。未来应不断改进、优化康复策略，建立最佳的多学科结构，制定科学、系统、个性化方案。
Objective: To systematically analyze the related studies on intervention schemes for patients with visual field defects after stroke, and to identify, summarize, and summarize the specific content, outcome indicators, and intervention effects, thereby providing a reference for clinical practice and future research in this field. Methods: Utilizing the scoping review method, a systematic search was conducted in 9 databases: CNKI, CQVIP, Wanfang Database, China Biomedical Literature Database, PubMed, Web of Science, Embase, CINAHL and the Cochrane Library. The search encompassed the period from the inception of each database to April 10, 2024. The selected literature was subsequrently screened, summarized, and analyzed. Results: A total of 12 articles were finally included, summarizing the intervention methods and outcome indicators for patients with visual field defects after stroke. The intervention methods comprised alternative intervention, compensatory intervention, and rehabilitative interventions. Outcome indicators include daily living activities, daily living extended activities, reading performance and visual field examination. Conclusion: Current intervention schemes for patients with visual field defects after stroke exhibit diverse content, charaterized by low evidence quality and inconsistent outcome indicators. Further high-quality research is needed to explore optimal training plans and standardize the outcome indicators. In the future, continuous improvement and optimization of rehabilitation strategies should be carried out to establish the best multidisciplinary framework and formulate scientific, systematic and individualized plans.
