神经系统疾病中神经眼科体征的临床分析

目的 旨在了解目前我国与神经眼科体征相关的疾病现状,初步探索和分析神经系统疾病中与神经眼科相关的就诊疾病谱状况,为今后开展神经眼科的相关研究和防治策略提供科学依据.方法采用回顾性调查方法,对浙江大学医学院附属第一医院2010年6月至2011年5月神经内(外) 科住院病历进行调查与分析.结果 共调查神经内(外)科住院患者3660例.535例(14.6％)有神经眼科相关体征,其中男性329例(61.5％),女性206例(38.5％).神经眼科体征以瞳孔改变(38.5％)和视力下降(27.7％)为最常见,其次为眼球运动障碍(16.1％)、上睑下垂(14.4％).与神经眼科发病相关的首要病因主要是脑梗(27.5％),肿瘤(15.3％)、外伤(13.8％),颅内出血(10.5％)、颅内缺血性病变(5.6％),其中视神经病变(4.1％)也占有一定比例.结论 神经眼科体征对神经科疾病的定位诊断具有重要意义;神经眼科涉及范围广泛,应针对性探讨制定神经眼科相关疾病的诊疗规范和可能干预策略.     

前交通动脉瘤引起视神经病变的临床特征及其诊断

颅内前交通动脉瘤压迫视神经和视交叉所致的视力下降、视野缺损、视盘水肿、视神经萎缩等表现极易与缺血性视神经病变、球后视神经炎、正常眼压性青光眼等眼病混淆。对此，我们对其所引起的视神经病变的临床特征及诊断要点进行分析，强调临床眼科医师应加强学习神经眼科疾病的基础知识，掌握相关疾病之间的关键鉴别点，选用合理的影像学检查手段，及时查明确切病因和病变部位。     

2007年神经眼科学习班将在北京举办

吴阶平医学基金会、解放军总医院眼科联合举办的“神经眼科学习班”，将于2007年5月11日至5月13日在北京由中国人民解放军总医院举办。学习班将邀请国内知名专家及中青年医师授课。授课主要内容：（1）神经眼科学的基础知识；（2）视野检测方法；（3）神经眼科学的基本检查法；（4）常见的神经眼科学疾病：颅内静脉窦血栓形成与视乳头水肿、颈内动脉狭窄与相关眼病、蝶鞍区病变与眼科表现、糖尿病视神经病变、视神经炎的诊治、旁中心视力与视神经炎的关系、缺血性视神经病变、外伤性视神经病变诊治的新观点、Leber视神经病、视网膜动脉阻塞、视交叉疾患、有神经眼科意义的眼眶疾病；     

加强神经眼科基础知识和基本技能的教育

掌握神经眼科基础知识和基本技能是眼科医师在临床实践中正确诊治神经眼科相关疾病的基础.鉴于神经眼科疾病的学科交叉性,其基础知识和基本技能包括眼相关神经解剖、神经生理以及对于神经影像和神经电生理等重要辅助检查手段的正确理解和应用.特别应该强调的是建立正确的诊断思路对于神经眼科疾病诊治的重要性.采用多种途径,加强神经眼科基础...     

以视神经萎缩为首发表现的神经梅毒8例

神经梅毒是皮肤科、神经内科的常见疾病.既往报道多集中在病变累及中枢神经系统而表现出的精神症状、抽搐发作、记忆力减退、肢体无力、双下肢疼痛等方面[1].对于以眼部视力障碍、视神经萎缩在眼科确诊并得到及时治疗的报道较少.为此,我们观察了8例以视力下降、视神经萎缩在眼科确诊为神经梅毒患者的临床特征,以提醒眼科同仁在临床遇到视神经萎缩患者,在考虑颅内占位、遗传性疾病等因素外,还需警惕感染性因素.现将结果报道如下.     

《视神经疾病案例图解》出版

视神经疾病是神经眼科学领域尤为重要的疾病,同时也是导致视觉传入障碍性疾病的主要病因。本书12章,由总论及10余种视神经疾病单病种约120个病例组成。总论中叙述了视神经的解剖特征、视神经常用检查手段、辅助检查及视神经疾病的诊断流程、鉴别诊断。作为分章节病例学习阅读的基础,总论概述了所有视神经疾病的诊疗通则,其临床表现及检查手段适用于任何视神经疾病,也包括病史描述中的常用英文缩略词。分章节中按照先天发育异常、视神经炎、缺血、压迫、遗传、代谢中毒、外伤、肿瘤导致的视神经疾病顺序对各种常见视神经病变进行了病例图解。每个分章节同样按照概述、临床特征、诊断、治疗及预后的顺序,随后用数个典型病例强调说明上述临床特征。     

重视神经外科疾病的眼部表现

眼与神经系统的解剖生理关系非常密切,十二对颅神经中7对与视觉传入或眼球运动直接相关。神经外科的许多疾病均与眼科相关,包括颅脑外伤、颅内肿瘤、脑血管病、特发性颅内压增高症、面肌痉挛等,手术治疗是改善症状、挽救视力或生命的关键措施。正确认识神经外科疾病的眼部表现,是做出早期正确诊断、及时合理治疗的基础,需要眼科、神经外科以及相关学科专业人员密切合作和共同努力。（眼科, 2015, 24： 4-7）     

基因治疗在神经眼科中的应用

目的应用基因技术治疗眼病的可行性备受国内外学者关注，并且通过利用动物模型进行了一系列实验取得很大进展，在治疗神经眼科疾病方面有临床应用前景。本文就应用该技术治疗视神经疾病的可行性及其研究现状进行综述。     

内皮素-1与眼科主要致盲性疾病的关系

内皮素-1(Endothelin-1,ET-1)是一种含有21个氨基酸的血管活性肽,它是目前被认为的最强的缩血管物质。它不仅具有强大的收缩血管的作用,还可以促进炎症的产生。它除了对心血管、泌尿、呼吸、消化、神经、生殖、内分泌等系统中起作用外,对眼科的各种致盲性疾病起着十分重要的作用,如内皮素-1在眼部中主要分布在脉络膜中,它对白内障的发展、青光眼眼压的升高及视神经的损害有一定联系。内皮素-1对糖尿病性视网膜病变中微血管稳态的维持及病情的诊断有一定作用;在非动脉炎性前部缺血性视神经病变中,它的浓度的高低与病变程度相吻合,对病情的判断和预后评估均有一定的临床意义。随着内皮素-1在这些疾病中的作用机制研究的不断深入,未来眼科相关疾病的预防和治疗将大有裨益。     

中国脱髓鞘性视神经炎诊断和治疗循证指南(2021年)

脱髓鞘性视神经炎(DON)是主要累及视神经的中枢神经系统炎性脱髓鞘疾病。准确诊断、降低复发率、改善预后一直是临床诊疗过程中关注的重点。随着有关DON临床诊断标志物、疾病分型及不同亚型治疗研究的深入,越来越多的临床证据不断涌现,迫切需要及时制定DON的诊断和治疗循证指南,指导临床进一步规范开展工作。为此中华医学会眼科学分会神经眼科学组联合兰州大学循证医学中心/世界卫生组织指南实施与知识转化合作中心,按照循证指南制定的方法和步骤,成立指南指导委员会、指南共识专家组和指南外审专家组以及指南制定工作组,通过调研临床一线神经眼科医师、眼科医师及神经内科医师,收集并整理临床关注问题,全面检索和系统评价国内外相关研究证据,制定新的系统评价,组织召开专家共识会议,深入访谈和剖析患者偏好和价值观,最终形成16条针对DON诊断和治疗的推荐意见,主要涉及分类和诊断、急性期和慢性期治疗、随访管理等方面,并对每条推荐意见进行详细的解释说明,以期为临床有效治疗DON和降低复发率发挥指导作用。(中华眼科杂志,2021,57:171-186)     

Anterior ischemic optic neuropathy in association with optic nervehead drusen

Optic nerve head drusen (ONHD) are incidental ophthalmologic finding in the optic nerve. Patients with ONHD are often asymptomatic, but sometimes present with transient visual obscuration''s (TVO), the reported incidence of which is 8.6%. Optic nerve head drusen are of two types: Superficial; visible and deep. The deep-buried drusen mimic papilledema. Because of the varied presentation deep-buried drusen pose a diagnostic challenge to the ophthalmologists. In young patients, they are mistaken for papilledema as it is clinically difficult to detect a buried drusen in the optic nerve head, but are seen on the surface with aging as the retinal nerve fiber layer thins out. They are observed as pale yellow lesions more often located towards the poles. Clinical examination aided with diagnostic tests like computed tomography (CT) orbits and ultrasound B scan can help establish the diagnosis. Herein, we report a rare case of optic nerve head drusen in a young lady, who presented with loss of vision and clinical evaluation and investigations suggested ONHD with anterior ischemic optic neuropathy.     

Non-arteritic anterior ischemic optic neuropathy: role of systemic corticosteroid therapy

OBJECTIVE: To investigate systematically the role of systemic corticosteroid therapy in non-arteritic anterior ischemic optic neuropathy (NA-AION). METHODS: The study consists of a cohort of 613 consecutive patients (696 eyes), first seen in our clinic from 1973 to 2000. Of this cohort, 312 patients (364 eyes) voluntarily opted for systemic steroid therapy, and 301 (332 eyes) for no treatment. At first visit, all patients in both groups had a detailed ophthalmic and medical history, and comprehensive ophthalmic evaluation. Visual evaluation was done by recording Snellen visual acuity, and visual fields with a Goldmann perimeter. The same ophthalmic evaluation was performed at each follow-up visit. Patients in the steroid-treated group were initially given 80 mg Prednisone daily for 2 weeks, and then tapered down to 70 mg for 5 days, 60 mg for 5 days, and then cutting down by 5 mg every 5 days. Visual outcome in the two groups was compared RESULTS: Median follow-up was 3.8 years. At 6 months from onset of NA-AION, of the eyes with initial visual acuity 20/70 or worse and seen within 2 weeks of onset, there was visual acuity improvement in 69.8% (95% confidence interval (CI): 57.3%, 79.9%) in the treated group, compared to 40.5% (95% CI: 29.2%, 52.9%) in the untreated group (odds ratio of improvement: 3.39; 95% CI:1.62, 7.11; p = 0.001). Comparison of visual field defect at 6 months from onset of NA-AION, among those seen within 2 weeks of NA-AION onset with moderate to severe initial visual field defect, there was improvement in 40.1% (95% CI: 33.1%, 47.5%) of the treated group, and 24.5% (95% CI: 17.7%, 32.9%) of the untreated group (odds ratio: 2.06, 95% CI: 1.24, 3.40; p = 0.005). In both treated and untreated groups, the visual acuity and visual fields kept improving up to about 6 months from onset of NA-AION, and very little thereafter. CONCLUSION: This study suggested that NA-AION eyes treated during the acute phase with systemic corticosteroids resulted in a significantly higher probability of improvement in visual acuity (p = 0.001) and visual field (p = 0.005) than in the untreated group. Both visual acuity and visual fields improved up to 6 months after onset of NA-AION.     

前部缺血性视神经病变血液流变学分析

目的观察前部缺血性视神经病变的血液流变学改变,探讨其发生因素,为治疗和预防提供客观依据。方法前部缺血性视神经病变视乳头水肿期患者３９例（男１９例,女２０例）与相同例数的可以与之相匹配的正常对照组男、女各２０例进行血液流变学检查及统计学处理。结果患者组明显高于对照组（Ｐ＜０．０１,Ｐ＜０．０５）的项目有：红细胞压积、纤维蛋白原、低切粘度、聚集指数、红细胞电泳时间。女性患者高切粘度和血浆粘度亦显著高于对照组（Ｐ＜０．０１）。结论高血粘度与非颞动脉炎性前部缺血性视神经病变的发生密切相关     

Ischemic optic neuropathy

Ischemic optic neuropathy (ION), based on vascular anatomy of the optic nerve, pathogenesis and clinical picture, consists of two distinct entities: anterior (AION) and posterior (PION) ischemic optic neuropathies. AION is due to interference with posterior ciliary artery supply to the optic nerve head and retrolaminar part of the optic nerve; it initially presents with visual loss and optic disc edema which progresses to optic atrophy in a month or two. PION is due to occlusion of nutrient arteries to the posterior part of the optic nerve; in this condition during the initial stages the optic disc is normal in spite of marked visual loss, but the atrophy develops later on. Their pathogeneses, causes, clinical pictures, diagnosis and management are discussed briefly.Some of the figures have been reproduced by courtesy of the British Journal of Ophthalmology (Figs. 2, 5, 7), American Academy of Ophthalmology and Otolarngology (Fig. 1) and Springer-Verslag (Fig. 8). This investigation was supported by Public Health Service Grant EY-01151.     

Validación de la escala de graduación del color en la fotografía del nervio óptico,una alternativa para la clasificación cualitativa

BackgroundTo validate objectively the proposed Teherán-Morales's color grading scale, comparing to the subjective readings of specialists in optic nerve photography.MethodsConcordance study and diagnostic tests, in which 150 photographs of the optic nerve were evaluated, from three groups, glaucomatous neuropathy, neuropathy of other origin and control group with the Teherán-Morales's color scale and the analysis of three experts in optic nerve. Spearman's Rho correlation was performed between both analysis methods.ResultsIn the analysis of all the photographs using Spearman's Rho, we found moderate correlations that were statistically significant P < .0001, the highest was in the temporal quadrant by observer 1 (r = 0.650 95% CI 0.546 to 0.733). In photographs of optic neuropathy, the correlations become moderately high, and statistically significant P < .0001, the highest correlation was for the temporal quadrant by observer 1 (r = 0.772 95% CI 0.626 to 0.865). In the glaucoma and normal eyes groups, there were moderate to low correlations with statistical significance P < .05.ConclusionsThe Teherán-Morales's scale, for color grading, is useful in detecting color, correlates moderately with the subjective assessment of experts in the optic nerve having its best performance in optic neuropathy with very pale discs. However, in normal or glaucomatous optic discs, it has a low correlation, compared to the subjective clinical assessment.     

Familial non-arteritic anterior ischemic optic neuropathy

PURPOSE: Primary objective was to investigate clinical characteristics of nonarteritic anterior ischemic optic neuropathy (NA-AION) in three families; secondarily, to test these families for a previously detected mitochondrial mutation in a pedigree with familial NA-AION. METHODS: Study comprised three families where more than one member developed NA-AION. All patients with NA-AION had a detailed ophthalmic, medical and family history, and comprehensive ophthalmic evaluation at initial visit and on follow-up. One patient from family 1, one from family 2, 41 non-familial NA-AION patients, 97 control subjects and 1,488 patients with suspected Leber hereditary optic neuropathy (LHON) were tested for the presence of mitochondrial mutation (G4132A) in a previously reported genetic study of family 3. RESULTS: Familial NA-AION was found in seven individuals of family 1, four of family 2 and six of family 3. Symptoms, signs and clinical findings of familial NA-AION were similar to classical NA-AION, with two exceptions: familial NA-AION had an earlier onset (47.3 + 8.6 years versus 60.1 + 13.6 years) and a higher frequency of bilateral disease. The G4132A mitochondrial variant was not detected outside family 3. None of the three major mutations associated with LHON (G3460A, G11778A, T14484C) was found among Familial NA-AION patients. CONCLUSIONS: The only difference in clinical features between familial NA-AION and classical NA-AION is that the former has an earlier onset and a higher frequency of bilateral disease. The G4132A mutation is not commonly associated with familial NA-AION, and was not detected in patients with non-familial NA-AION. The role of hereditary factors in familial NA-AION remains largely unknown.     

Ultrasonic Elastography Evaluation in Optic Neuritis

Purpose: In the present study, we attempted to determine whether ultrasonic elastography (USE) evaluation can be used in a diagnosis of optic neuritis (ON). Materials and Methods: Thirteen patients who each had one normal eye and one eye with a diagnosis of ON were included in the study. Ultrasonography (US) and USE examinations were performed on the affected and non-affected eyes of all participants. Optic nerve and adjacent fat tissue regions at the same depth were selected, and USE measurements were obtained. The optic nerve diameter was measured in both normal and affected eyes. Results: The mean USE values for the optic nerve were 2.58 ± 0.50 m/s in ON eyes and 1.91 ± 0.39 m/s in normal eyes (p = 0.001). The mean USE values for the optic-nerve adjacent tissue were 2.26 ± 0.45 m/s in ON eyes and 1.77 ± 0.22 m/s in normal eyes (p = 0.001). The mean optic-nerve diameter was 3.80 ± 1.09 mm in ON eyes and 3.28 ± 0.98 mm in normal eyes (p = 0.005). Conclusions: USE may be considered an accessible, safe technique for the detection of significant optic-nerve tissue stiffness in ON and may be used an adjunctive tool for confirming this diagnosis.     

Analysis of the causes of optic disc swelling

Purpose

To investigate the clinical manifestations and diagnoses of optic disc swelling.

Methods

The medical records of 49 patients who experienced optic disc swelling between March 2008 and June 2009 were retrospectively reviewed. The characteristics of non-arteritic anterior ischemic optic neuropathy (NA-AION) and optic neuritis (ON), which showed optic disc swelling most commonly, were compared.

Results

NA-AION was the most common disorder (34.7%) that presented with optic disc swelling. ON was identified in 15 patients (30.6%). Seven out of 49 patients (14.3%) had intracranially associated diseases, such as papilledema and compressive optic neuropathy. Pseudopapilledema was noted in four patients (8.2%). Other diseases (e.g., papillophlebitis, neuroretinitis, and diabetic papillopathy) were seen in six patients (12.2%). Ocular pain was observed more commonly in patients with ON (p = 0.001). Patients with ON expected a better visual prognosis than patients with NA-AION (0.12 ± 0.32 vs. 0.49 ± 0.35, p = 0.001).

Conclusions

NA-AION and ON should be considered in the differential diagnosis when patients with optic disc swelling present to the neuro-ophthalmology clinic. Detailed history taking and supportive examinations, such as visual field, color-vision and imaging tests, should also be performed as indicated. Regular follow-up of such exams is necessary for the differential diagnosis of these diseases.     

外伤性视神经病变26例临床分析

目的探讨外伤性视神经病变的诊断方法及治疗效果。方法对5年间收治的26例（28只眼）视神经损伤患者的临床特点与治疗效果进行总结分析。结果无光感21只眼有5只眼视力提高，光感以上7只眼均有不同程度提高。6只眼行视神经管减压术，其中2只眼仍无光感，4只眼视力提高。结论外伤性视神经病变多见于车祸头部外伤后，应尽早用大剂量糖皮质激素和脱水剂及血管扩张剂等治疗，早期视神经管减压术效果好。伤后立即失明者效果差。     

Diagnosis of optic nerve sheath meningioma during optic nerve sheath decompression

Optic nerve sheath meningioma (ONSM) is typically diagnosed based on clinical suspicion and imaging characteristics and is most often treated with radiation. Historically, biopsy, optic nerve sheath decompression, and debulking surgeries have been avoided for fear of optic nerve vascular disruption and tumor spread into the orbit. This is a case of a 48-year-old man who presented with unilateral optic disc edema, declining visual acuity, and a visual field defect. Despite an initial improvement with acetazolamide, his vision subsequently worsened. With an elevated lumbar puncture opening pressure and imaging showing right optic nerve sheath enhancement, the differential diagnosis included ONSM, perineuritis and idiopathic intracranial hypertension (IIH). Optic nerve sheath decompression (ONSD) with biopsy was performed, simultaneously decompressing the nerve and yielding a sample for pathologic analysis. A pathologic diagnosis of ONSM was made and treatment with radiation was subsequently initiated, but vision began to improve after the surgical decompression alone.