视神经萎缩的鉴别诊断

视神经萎缩的鉴别诊断中国医科大学附属第一临床学院眼科王海林，夏德昭前言视神经萎缩不是一个单一的疾病，而是在各种不同原因的影响下视神经纤维的变性性病理改变及由变性所引起的视神经传导障碍［１］。由炎症和缺血引起视神经萎缩，视神经纤维变性消失，取而代之的间...     

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.     

外伤性视神经萎缩临床分析

眼眶部的挫伤 ,常引起视神经损伤 ,导致视力严重障碍 ,甚至失明。随着社会的发展和医学诊断方法的改进 ,外伤性视神经萎缩的诊断、治疗方法和预后逐渐被较多的认识 ,作者 1985～1998年诊治外伤性视神经萎缩 3 5例 ,分析报告如下 :　　一般资料 :3 5例中男 2 6例 ,女 9例 ,单眼 3 3例 ,双眼 2例 ,年龄最大 72岁 ,最小 6岁 ,损伤原因 :车撞伤 7例 ,摔伤 3例 ,自行车把捣伤 2例 ,砖、石块等硬物击伤 6例 ,拳击伤 7例 ,雨伞尖刺入眶内 1例 ,筷子刺入眶内 1例 ,枪弹伤 4例 ,眼眶部及眼球其他间接外伤 4例。受伤后多表现眼痛、头痛及视力减退 ,头…     

图型刺激条件对视神经萎缩和视神经炎时视觉诱发电位潜时的影响

目的 :探讨不同图型刺激条件对视神经萎缩和视神经炎时视觉诱发电位 (VEP)潜时异常的影响。方法 :3 1例 5 3眼视神经萎缩和 14例 2 1眼视神经炎患者接受了 3 6%和 97%两种黑白方格对比度的给撤图型刺激视觉诱发电位(P -VEP)检查。刺激方格 3°12′。结果 :视神经萎缩和视神经炎时两种P -VEP的P10 0波潜时均值均显著延长 (P <0 0 5 ) ;潜时异常阳性率分别为 75 %和 88%左右。但两种疾病时 ,两种P -VEP之间的P10 0波潜时均值差异和潜时异常阳性率区别均无显著差异 (P >0 0 5 )。结论 :结果说明 :在大刺激方格 (3°12′)条件下 ,在对比度高于 3 6%以后 ,对比度因素对两种疾病的P -VEP潜时的影响均没有区别。     

视神经萎缩和视神经炎时VEP潜时异常的比较

目的 :比较视觉诱发电位 (VEP)检查在视神经萎缩和视神经炎两种疾病时的区别。方法 :3 7例 66眼视神经萎缩和 14厘 2 6眼视神经炎患者接受了给撤图型VEP (P -VEP)和闪光VEP (F -VEP)检查 ,其中最佳矫正视力低于 0 0 3者仅接受F -VEP检查。结果 :视神经萎缩时的P -VEP潜时均值延迟程度低于视神经炎 (P <0 0 5 ) ,但F -VEP恰好相反。视神经萎缩和视神经炎时的P -VEP和F -VEP潜时异常率分别是 74%和 65 % ,90 %和 42 % (P <0 0 5 )。视神经萎缩和视神经炎时仅在F -VEP检查时表现出潜时异常的患眼百分率 ,在全部患眼和视力低于 0 0 3患眼分别是 2 3 %和 9% ,12 %和 5 %。结论 :视神经萎缩时的P -VEP潜时延迟程度低于视神经炎 ,但F -VEP相反 ;因此 ,视神经萎缩时F -VEP联合应用的检查价值比视神经炎时高。另外 ,两种疾病时F -VEP联合应用的检查价值不仅仅在具有不能接受P -VEP检查的视力患眼中存在 ,而且在具有能接受P -VEP检查的视力患眼中也仍然存在。     

梅毒性视神经视网膜炎一例

获得性梅毒是由梅毒螺旋体感染引起的一种性传播疾病，眼部表现首发者少见。我们最近诊治了一例以眼部症状首发、表现为视神经视网膜炎的梅毒患者，现报告如下。[第一段]     

腰椎肿瘤致视神经萎缩一例

崔×女38岁因双眼视神经萎缩于2003年2月19日到我院治疗。既往史:患者9月前双眼视力下降伴头痛,头晕,有时恶心、呕吐,双腿痛,不能行走。MRI检查诊断     

视神经萎缩260例病因及疗效分析

视神经萎缩不是一个单独的疾病，而是指各种病因引起的视神经纤维退行性改变，导致视功能障碍的疑难眼疾病。本文回顾总结了我院近6年来视神经萎缩患者260例，着重对其进行了病因分析及疗效评价，旨在提高临床上对本病的认识。     

Childhood optic atrophy

Purpose : To determine the causes, and relative incidence of the common causes, of optic nerve atrophy in children under 10 years old and to compare prevalent aetiologies with those given in previous studies. Methods : The Wilmer Information System database was searched to identify all children, diagnosed between 1987 and 1997 with optic atrophy, who were under 10 years old at diagnosis. The medical records of these children were reviewed retrospectively. Results : A total of 272 children were identified. Complications from premature birth were the most frequent aetiology of optic atrophy (n = 44, 16%); 68% of these premature infants having a history of intraventricular haemorrhage. Tumour was the second most common aetiology (n = 40, 15%). The most frequent tumour was pilocytic astrocytoma (50%), followed by craniopharyngioma (17%). Hydrocephalus, unrelated to tumour, was the third most common aetiology (n = 26, 10%). In 114 cases (42%), the cause of optic atrophy became manifest in the perinatal period and/or could be attributed to adverse events in utero. A cause was not determined in 4% of cases. Conclusions : In the last decade, prematurity and hydrocephalus appear to have become important causes of optic atrophy in childhood. This trend is probably the result of improved survival of infants with extremely low birth weight.     

电按摩联合球后注射血管扩张剂治疗视神经萎缩

目的:探讨电按摩联合球后注射血管扩张剂治疗视神经萎缩的疗效及其并发症的原因、结果及防治原则.方法:对行电按摩治疗的132例(243眼)视神经萎缩患者的疗效及并发症的病例资料进行回顾性总结分析.结果:132例243眼总有效率为84.8%(206眼),其中视神经炎性视神经萎缩的有效率为93.7%.8种并发症除1例眼痛3mo完全恢复外,其它均在4 h～10 d内恢复.结论:电按摩治疗视神经萎缩疗效显著,并发症可防可治.     

视神经萎缩98例患者CT资料分析

目的:探讨CT对视神经萎缩的诊断价值。方法:用CT扫描98例已确诊的视神经萎缩患者视神经脑外段,观察其影像学变化。结果:视神经萎缩CT表现为密度减低影,钙化,硬化带,密度增高影,继发性视神经萎缩较原发性视神经萎缩表现明显,视力衰减程度和视神经萎缩程度无明显关系。结论:CT扫描可用于视神经萎缩的诊断,并可鉴别原发性和继发性视神经萎缩。     

视神经萎缩的中医药治疗概况

视神经萎缩是一种由于各种病因所致视神经纤维传导功能产生障碍的视神经退行性改变,临床以视力减退和视乳头呈灰白或苍白为特征。本病属于中医学“青盲”的范畴。西医目前对该病治疗尚无理想药物及技术;中医采用中药与针刺等方法可使大多数患者视力有所改善。本文就中医药治疗本病进行综述。     

Difficulties in diagnosing leber's optic atrophy

The lowered vision of a patient was attributed to an acute quinidine intoxication, although his condition differed from that of another patient with an acute quinine intoxication. Later, the uncle of the first patient showed lowered vision too, possibly due to a tobacco-alcohol neuropathy. Both uncle and nephew belonged to a family, in which Leber's hereditary optic atrophy occurred.     

Dominant optic atrophy: Culprit mitochondria in the optic nerve

Dominant optic atrophy (DOA) is an inherited mitochondrial disease leading to specific degeneration of retinal ganglion cells (RGCs), thus compromising transmission of visual information from the retina to the brain. Usually, DOA starts during childhood and evolves to poor vision or legal blindness, affecting the central vision, whilst sparing the peripheral visual field. In 20% of cases, DOA presents as syndromic disorder, with secondary symptoms affecting neuronal and muscular functions. Twenty years ago, we demonstrated that heterozygous mutations in OPA1 are the most frequent molecular cause of DOA. Since then, variants in additional genes, whose functions in many instances converge with those of OPA1, have been identified by next generation sequencing. OPA1 encodes a dynamin-related GTPase imported into mitochondria and located to the inner membrane and intermembrane space. The many OPA1 isoforms, resulting from alternative splicing of three exons, form complex homopolymers that structure mitochondrial cristae, and contribute to fusion of the outer membrane, thus shaping the whole mitochondrial network. Moreover, OPA1 is required for oxidative phosphorylation, maintenance of mitochondrial genome, calcium homeostasis and regulation of apoptosis, thus making OPA1 the Swiss army-knife of mitochondria. Understanding DOA pathophysiology requires the understanding of RGC peculiarities with respect to OPA1 functions. Besides the tremendous energy requirements of RGCs to relay visual information from the eye to the brain, these neurons present unique features related to their differential environments in the retina, and to the anatomical transition occurring at the lamina cribrosa, which parallel major adaptations of mitochondrial physiology and shape, in the pre- and post-laminar segments of the optic nerve. Three DOA mouse models, with different Opa1 mutations, have been generated to study intrinsic mechanisms responsible for RGC degeneration, and these have further revealed secondary symptoms related to mitochondrial dysfunctions, mirroring the more severe syndromic phenotypes seen in a subgroup of patients. Metabolomics analyses of cells, mouse organs and patient plasma mutated for OPA1 revealed new unexpected pathophysiological mechanisms related to mitochondrial dysfunction, and biomarkers correlated quantitatively to the severity of the disease. Here, we review and synthesize these data, and propose different approaches for embracing possible therapies to fulfil the unmet clinical needs of this disease, and provide hope to affected DOA patients.     

马来西亚视神经萎缩的临床特点及病因

目的：研究在马来西亚非青光眼视神经萎缩的病因及临床特点。

方法：一系列回顾性的研究分析马来西亚理科大学校医院眼诊所在2007/2011年间被诊断为非青光眼视神经萎缩的患者。至少随访1a。评估这些患者的医疗记录及汇编调查结果。

结果：100例患者符合选择标准,56%的患者双眼都参与研究。主要症状为视力模糊(61%),除了视力模糊外还出现神经方面病症(18%),视野狭窄(9%)。大多数患者(63%)患眼视力下降到3/60以下。主要的病因是颅内占位性病变(26%),先天性疾病(13%),脑积水(12%),创伤(12%)及血管因素(12%)。对大多数患者(67%)采用保守治疗。不管其病因,视神经萎缩都伴有不同程度的视功能障碍。随访1a后,50%的患者出现不同程度的视觉障碍。

结论：视神经萎缩主要的病因是颅内占位性病变,其次分别是先天性疾病,创伤和血管疾病。在诊断之前常常就出现视觉和神经上的症状,而疾病显著地影响着视力的变化。为了早期诊断视神经萎缩,当视力模糊的主诉为非特异性时,应该高度怀疑本病。     

实验性外伤性视神经萎缩动物模型的建立

目的　探索并确立实验性外伤性视神经萎缩模型的建立方法。方法　采用改良的Kr lein术式 ,在定量致伤的条件下建立实验性外伤性兔视神经萎缩模型 ,于 2 0d和 40d后分批作闪光视觉诱发电位 (F VEP)和病理形态学检查。结果　改良的Kr lein术式造模 40d后 ,造模组兔眼视觉电生理的F VEPP1波和N1波波幅极显著降低 (P <0 0 1) ,峰时极显著延长 (P <0 0 1) ;神经纤维数量极显著减少 (P <0 0 1)、髓鞘积分光密度极显著降低 (P <0 0 1)、神经胶质细胞显著增生 (P <0 0 5 )。结论　采用改良的Kr lein术式建立的实验性外伤性兔视神经萎缩模型是成功的。     

中药疏肝明目丸治疗视神经萎缩129例

目的:观察疏肝明目丸对视神经萎缩治疗效果。方法:视神经萎缩患者129例(230眼),分为治疗组115例,口服疏肝明目丸;对照组115例,口服VitC、VitB1、复方路丁、肌注VitB12,静滴复方丹参注射液随访。结果:治疗组115例,显效61.0%,好转33.0%,无效6.0%;对照组显效35.9%,好转44.9%,无效19.2%,P <0.05。结论:疏肝明目丸治疗视神经萎缩疗效优于对照组,对视神经有明显保护作用。