婴儿眼球震颤综合征的治疗进展

婴儿眼球震颤综合征(infantile nystagmus syndrome,INS)是一种在生后早期出现的病理性眼球震颤,以双眼非自主性共轭眼球运动为临床特征,会破坏双眼视力及视功能,同时是引起弱视、斜视以及斜颈的原因之一.目前尚无治愈INS的方法,但治疗方法有许多进展,如视光学治疗采用框架眼镜、角膜接触镜、三棱镜、低视力助视器及激光,药物治疗采用碳酸酐酶抑制剂、肉毒素眼外肌注射等,生物反馈治疗采用听觉反馈等,以及新的手术方法.     

先天性眼球震颤检查及手术治疗进展

先天性眼球震颤(congenital nystagmus.CN)是指出生或生后2～4月内出现的一种不自主性眼球运动.随着现代科技的迅速发展,各种检查手段引入CN的研究领域,如视觉电生理,眼震电图频谱分析,电子计算机,实现了对CN患者精确、完整地定量评估.手术治疗在传统的术式基础上也有了大的改进.本文扼要综述近年来对CN患者的检查方法和手术治疗的一些进展.     

婴儿眼球震颤综合征的研究进展

婴儿眼球震颤综合征(infantile nystagmus syndrome,INS)是一种先天性病理性眼球震颤,以双眼非自主性的共轭摆动和反向视动性眼球震颤为特征表现。INS患者可合并弱视、斜视及斜颈等,常有不同程度的视功能障碍。该病病因尚不明确,且无法完全治愈,应尽早对INS进行检查和适当干预。基于国内外对INS的研究成果,本文首先总结了INS目前所知的病因及发生机制; 其次,介绍了INS近年提出的检查方法与治疗方案,总结了相关临床实践中存在的问题,并在此基础上,对未来可能的研究方向给出了建议,旨在为临床应用及未来研究方向提供参考。     

隐性眼球震颤

隐性眼球震颤(Latent nystagmus又称潜在性眼球震颤.此症平时无眼球震颤表现,当遮盖一眼时,则出现显性眼球震颤.因检查不细心或对此症缺乏认识易造成漏诊.现结合一完整病历简要综述如下:     

眼球震颤一家系

１　临床资料　　先证者,男,４１ａ,已婚,农民,主诉自幼双眼球来回摆动。检查：全身无异常。视力右眼０－３,左眼０－４。收稿　１９９９０８０２本文编辑　张知达作者单位　２２６６００　江苏省海安县人民医院眼科眼位检查为钟摆式水平性眼球震颤,无明显斜视。震颤频率每分钟５０次,振幅２ｍｍ属中等频率中等振幅的眼球震颤,眼球转动不能随意识控制。屈光度透明,眼底未见明显异常。矫正插片不接受。其外祖父、舅父、哥哥、女儿、侄女和外甥共７人同样罹患此病。２　讨论眼球震颤是一种疾病,也可以是某些疾病的一种症状。有…     

眼球震颤的诊断与治疗研究进展

眼球震颤是常见的眼球运动障碍疾病,临床诊治困难。随着近年眼球运动记录方法的进步和药物、手术及基因治疗的研究进展,为临床该病的诊断和治疗提供了更多的选择,也为眼球震颤患者带来新的希望。本文就该病诊断与治疗的最新研究进展进行了综述。     

先天性眼球震颤脑血流速度的测定

目的：探讨先天性眼球震颤的发病机理，了解其脑血流速度的变化。 方法应用彩色经颅多普勒超声诊断技术，对５０例先天性眼球震颤患者进行脑血流速度的检测。 结果患者的大脑中动脉、大脑前动脉血流速度呈单侧性增高，双侧大脑后动脉血流速度较正常人低（Ｐ＜０．０５）。 结论先天性眼球震颤患者大脑两侧血流速度不均衡，可能是造成该区神经功能不平衡、导致眼球运动功能失调的主要原因之一。     

隐性眼球震颤1例

1　临床资料患者,男,33a,汽车司机。体检发现单眼视物时物体晃动,无法查视力5d来院。眼部检查:双眼视时,视力为1.5,眼位居中,眼球在各方向上均不受限。遮盖一眼,注视眼很快出现水平冲动性眼球震颤,且振幅与频率逐渐增加。快相指向注视眼,因此无法常规测单眼视力。将一眼遮盖物稍前移,见遮盖眼也有眼球震颤,快相也指向注视眼,仅振幅与频率较注视眼小。移去遮盖物,在一眼前加 8.0球镜后,无眼球震颤表现,此时测单眼视力,右眼为1.2 2,左眼为1.5。扩瞳后见晶状体、玻璃体透明,眼底无异常,视网膜镜检影,无屈光不正。诊断:双眼隐性眼球震颤。处理:…     

Infantile nystagmus: current concepts in diagnosis and management

This review examines current approaches to the diagnosis and management of congenital forms of nystagmus. Emphasis is placed on diagnostic features that are amenable to clinical identification but those issues that can be addressed only with more detailed investigations, such as eye movement recording, are indicated. Non‐surgical management, including prism spectacles, contact lenses and vision therapy, is discussed, as are surgical approaches. Because many aspects of congenital forms of nystagmus, particularly as experienced by patients with the condition in their normal lives, are poorly addressed in both the clinical and research literature, these limitations are also highlighted.     

伴有隐性眼球震颤弱视的遮盖治疗

目的：探讨遮盖疗法对伴有隐性眼球震颤弱视眼视力的治疗效果及眼球震颤的变化。方法：将隐性眼球震颤合并单眼弱视的52例患儿（52只眼）,按弱视程度分为轻、中、重三组,采用全部遮盖及部分遮盖方法,每月复查一次视力、眼位、眼底及眼球震颤情况。视力提高到1.0后继续治疗3-6月。结果：52例中视力提高≥2行者48例（国际标准视力表）,提高达1.0者22例。无论弱视眼视力提高≥2行,还是提高到1.0,眼球震颤无明显改变。结论：采用遮盖疗法可以提高伴有隐性眼球震颤弱视眼的视力,但视力提高后眼球震颤无明显改变。     

Long-term results of four horizontal rectus muscle recession in nystagmus treatment

Purpose: To report the long-term results of four horizontal rectus muscle recessions that were performed for infantile nystagmus syndrome treatment.

Methods: In this case series, patients with infantile nystagmus syndrome who had four horizontal muscle recessions previously were recruited and ophthalmological examination and electronystagmography recordings were performed. Objectively, amplitude and frequency of nystagmus were measured from the recordings and the intensity was calculated. Visual acuity, stereopsis, and alignment were evaluated and compared with the preoperative and postoperative values.

Results: The records of the 12 patients who had four horizontal rectus muscle recession surgery were evaluated and six patients (5 male, 1 female) who had regular follow-ups were included in this study. Mean follow-up was 14.17?±?0.41 years (minimum 14 years, maximum 15 years) and mean age of patients at the last visit was 22 years (20-28 years). On subjective evaluation, two-thirds (4/6) of the patients were satisfied with the surgical results and had the impression that after surgery, nystagmus decreased in intensity and head posture improved. On objective evaluation, visual acuity was found to be the same, however, stereopsis improved (preoperatively and postoperatively median stereopsis was 600?sec arc vs 200?sec arc final). The decrease in nystagmus amplitude and frequency was still maintained.

Conclusions: Nystagmus surgery on four horizontal rectus muscles has positive effects on binocular function and nystagmus parameters in the long-term follow-up. As we could not treat the primary pathology, the visual acuity was about the same but the decrease in nystagmus amplitude and frequency was still maintained with better stereopsis, and patient satisfaction.     

Topical lambda-cyhalothrin in reducing eye oscillations in a canine model of infantile nystagmus syndrome

Purpose:To determine the ocular and systemic safety of using topical Lambda-Cyhalothrin (LCL) in a canine model of infantile nystagmus syndrome (INS). The rationale for this proposal is based on a case study of a patient whose INS improved after inadvertent ocular exposure to a pyrethroid pesticide containing LCL.Methods:After in-vitro safety testing and IUCAC approval, we studied increasing concentrations of topical LCL drops (0.002% to 0.07%) in canines with a purposely bred defect in the RPE65 gene resulting in both retinal degeneration and INS. We collected data on ocular and systemic effects and performed eye-movement recordings (EMR).Results:At the 0.07% concentration dose of LCL, there was minimal, reversible, conjunctival hyperemia. There was no other ocular or systemic toxicity. At the 0.06% dose, there was a visible decrease in the INS and EMR showed a 153%–240% increase in the nystagmus acuity function and a 30%–70% decrease in amplitude across gaze. There was also a 40%–60% decrease in intraocular pressure while on the drop in both eyes.Conclusion:This animal study suggests this new pharmacological agent has potential for topical treatment of both INS and diseases with raised intraocular pressure. Further, this new treatment approach confirms the importance of extraocular muscle proprioception in ocular motor diseases and their treatment.     

先天性眼球震颤治疗方法研究进展

先天特发眼球震颤是以眼部表现为主的疾病,虽然不属于常见或多发病,但在临床上并非罕见,多见于婴幼儿,发病时间一般都在半岁以内。该病的特点为:发病早或发病时间不能确定,眼球不自主的持续跳动或摆动,极少患者有晃视感,大多数患者都有不同程度的视力损害,有的视力减低比较严重,而且不能矫正,较多患者有侧视现象和代偿头位表现,有的头部摇晃,还有的表现为频繁眨眼等代偿现象。该病的主要治疗手段是手术治疗,但影响手术效果的因素很多:发病年龄,眼震强度,代偿头位,休止眼位,视力,手术方式和手术量等。随着近年的发展,对该病有了更深入的认识,我们就一些问题做一简要回顾。     

A case of monocular nystagmus blockage syndrome and manifest-latent nystagmus

A 24-year-old man, left eye enucleated at the age of ten months, had jerk-right nystagmus with increasing amplitude in abduction and null position in near-extreme adduction. Under occlusion or in darkness, the nystagmus changed to jerk-left, the amplitude increased in adduction and null position was in abduction; as if his left eye had nystagmus blockage syndrome and he was seeing with this absent left eye! The difference between nystagmus blockage syndrome and latent nystagmus is discussed: nystagmus blockage syndrome is manifested by ‘motor’ maneuver of adduction and abduction, whereas latent nystagmus is induced with ‘sensory’ maneuver of occlusion. It suggests that nystagmus blockage syndrome and latent nystagmus may arise from different mechanisms despite their frequent concurrence.     

The sub-clinical see-saw nystagmus embedded in infantile nystagmus

A transient, decompensated vertical phoria in an individual with infantile nystagmus syndrome (INS) resulted in two images that oscillated vertically-a diplopic oscillopsia. Ocular motor studies during the vertical oscillopsia recreated by vertical prisms, led to the identification of a sub-clinical see-saw nystagmus (SSN), present under the prism-induced diplopic condition. Retrospective analysis of ocular motor recordings made prior to the above episode of vertical diplopia revealed the presence of that same sub-clinical SSN. The SSN had not been detected previously despite extensive observations and recordings of this subject's pendular IN over a period of forty years. Three- dimensional search-coil data from fourteen additional INS subjects (with pendular and jerk waveforms) confirmed the existence of sub-clinical SSN embedded within the clinically detectable horizontal-torsional IN in seven of the fifteen and a sub-clinical, conjugate, vertical component in the remaining eight. Unlike the clinically visible SSN found in achiasma, the cause of this sub-clinical SSN is hypothesized to be due to a failure of the forces of the oblique muscles (responsible for the torsional component of the IN) to balance out the associated forces of the vertical recti; the net result is a small, sub-clinical SSN. Thus, so-called "horizontal" IN is actually a horizontal-torsional oscillation with a secondary, sub-clinical SSN or conjugate vertical component. The suppression of oscillopsia by efference copy in INS appears to be accomplished for each eye individually, even in a binocular individual. However, failure to fuse the two images results in oscillopsia of one of them.