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:…     

OCT对先天性眼球震颤儿童黄斑结构的评价

目的 应用光学相干断层扫描技术(optical coherence tomography,OCT)来评价各种儿童眼球震颤中黄宽中心凹的结构及功能,指导治疗和判断预后.方法 对22例(44眼)先天性眼球震颤的儿童进行视力、眼前段检查、眼底检查、散瞳检影、屈光矫正、眼底照相及Zeiss Stratus 3000 OCT进行黄斑OCT的检查.结果 先天性婴幼儿眼球震颤19例(其中3例为隐性眼球震颤)、3例眼球震颤阻滞综合征.OCT检查有异常结果(阳性结果)19眼,其中黄班缺如11眼,黄斑发育不良8眼;阴性结果25眼.11眼黄斑缺如者中4眼为眼部白化病、1眼牵牛花综合征、6眼脉络膜缺损.结论 OCT用于动态即时评价黄斑中心凹的解剖学结构形态,对于眼球震颤的儿童可以早期找到病因,正确地评估预后和采取干预措施.     

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.     

先天性与后天性眼球震颤的眼震电流描记术表现

应用眼震电流描记术,对16例先天性和10例后天性眼球震颤患者进行了多项试验检查。结果表明,两者在凝视性眼球震颤、扫视和视跟踪反应,以及视动性眼球震颤等方面,都有很大的差异。认为,此项技术可对两类眼球震颤作出较为客观的鉴别诊断。     

儿童眼球震颤波形分析

目的 评价儿童眼球震颤的类型、检测方法、红外视频眼球震颤仪对儿童眼球震颤波形的分析能力及眼球震颤与视力的关系.方法 使用MG6B-5型手持式放大镜对148例(296眼)儿童眼球震颤患者检测.81例(162眼)患者行Y-Ⅱ眼球震颤仪检测,15例(30眼)行EYELINK眼动仪检测.111例(222眼)患儿行国际标准视力表检测.对75例(150眼)患儿的眼球震颤图检查和视力进行比较.结果 先天特发性眼球震颤121例(242眼),中枢性眼球震颤5例(10眼),前庭性眼球震颤1例(2眼),眼源性眼球震颤21例(42眼).眼球震颤频率为(2.88±1.37) Hz,振幅(7.88±5.22)度,震强(22.68±11.79)度×Hz.111例患儿矫正视力为0.32±0.22.75例患儿矫正视力与眼球震颤频率、振幅及震强无明显相关性(均为P＞0.05).结论 儿童眼球震颤可以使用眼球震颤仪对震频、振幅、震强精确地量化检测,从而对指导病因及治疗进行评价.     

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:     

眼球震颤的临床特征及诊疗进展

眼球震颤根据发病年龄可分为先天性眼球震颤和后天获得性眼球震颤, 复杂多变的临床表现及有限的诊疗措施使其成为眼科诊疗的疑难杂症之一。近年来, 视频眼震电图及数字化眼动仪等用于量化评估眼球震颤的程度及治疗效果;在治疗方面, 除传统的手术方式外, 可采用视光学手段(矫正屈光不正、配戴三棱镜)、生物反馈治疗、药物(加巴喷丁和美金刚胺)治疗及知觉训练等。(国际眼科纵览, 2022, 46:228-235)     

家族性先天性眼球震颤患者眼底病变特点的临床观察

目的：研究家族性先天性眼球震颤患者的眼底病变特点。

方法：对我院就诊的家族性先天性眼球震颤3个家系患者20例40眼进行眼位、屈光状态、眼前节、眼底彩色照相、光学相干断层扫描(OCT)、视网膜电生理(ERG)和视觉诱发电位(VEP)等检查。

结果：家系A共8例患者,均为水平型眼球震颤,其中斜视6例(伴代偿头位2例),核性白内障2例,后极性白内障2例,早产儿视网膜病变1例,高度屈光不正2例。家系B共6例患者,均为水平型眼球震颤,其中白化病3例(伴黄斑发育不良2例),高度屈光不正2例,全色盲1例,核性白内障1例。家系C共6例患者(5例水平型眼球震颤,1例旋转型眼球震颤),其中Leber先天性黑矇2例,家族性渗出性视网膜病变1例,后极性白内障2例,虹膜萎缩1例,斜视2例。

结论：家族性先天性眼球震颤患者要进行全面的眼部检查,尽可能找出病因,改善患者视功能。     

眼球震颤阻滞综合征(附3例报告)

眼球震颤阻滞综合征的特点为内斜视前先有眼球震颤 ,双侧外展神经假性麻痹以及当注视眼由内转位向外转位运动时 ,出现显性眼球震颤。本文报告 3例眼球震颤阻滞综合征 ,对其发病率、临床表现、鉴别诊断和手术方法作了简要讨论。     

眼球震颤诊治进展

眼球震颤是指双眼有节律的、不自主的,常呈对称共轭性的异常摆动,可根据发病年龄分为先天性眼球震颤和后天获得性眼球震颤。由于目前有限的诊治手段使得眼球震颤成为较为复杂的眼科疑难疾病之一。近年来,国际和国内对该类疾病的检测和诊疗水平在不断进步。本文将对眼球震颤的分类及特点,检查记录方法以及治疗的新进展进行综述。     

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.     

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.     

Clinical and Electrophysiological Effects of Extraocular Muscle Surgery on Patients with Infantile Nystagmus Syndrome (INS)

The purpose of this report is to summarize clinical and electrophysiological effects of extraocular muscle surgery in patients with INS. Our hypothesis is that surgery on the extraocular muscles of patients with INS changes their nystagmus resulting in improved vision and visual functions. All patients had all four virgin horizontal recti operated on, either for strabismus alone, nystagmus alone, for a head posture due to an eccentric null zone alone or for a head posture due to an eccentric null zone plus strabismus. All patients have been followed for at least 12 months. Subjective outcome measures include the pre- and post-operative binocular best optically corrected null zone acuity (NZA) in 75 patients and gaze dependent acuity (GDA) in 12 patients. Objective outcome measure included null zone width (NZW) in 75 patients. The results are summarized as follows: NZA increased. 1 LogMar or greater in 75% with those patients ≤8 years significantly better. Subjective GDA and NZW measured from eye movement recordings showed persistent, significant increases. This report adds to the evidence that surgery on the extraocular muscles in patients with INS has independent neurological and visual results, from simply reposition the head, eye(s) or visual axis.     

Soft Contact Lenses to Optimize Vision in Adults with Idiopathic Infantile Nystagmus: A Pilot Parallel Randomized Controlled Trial

Purpose: The optimal management of infantile nystagmus syndrome (INS) is still unclear. Contact lenses (CL) may be superior to glasses in improving visual function in INS but it is not known whether their beneficial effects are due to optical correction alone, or to an additional proprioceptive effect, and whether soft CLs would be as effective as rigid CLs. There is little data on feasibility and and the present study aimed to provide this information.

Methods: We completed a pilot Randomized Control Trial (RCT) at a single tertiary referral centre in London, UK. We enrolled 38 adults with idiopathic INS and randomised them to either plano CL (with corrective spectacles if required) or to corrective CL. CL wear was required for a minimum of 2 weeks. Primary outcome measures were feasibility and safety of CL wear in INS; secondary outcome measures were visual acuity and nystagmus waveform parameters.

Results: 27 completed the study (27/38,71%). 4 partcipants withdrew due to difficulty with CL insertion/removal and 7 were lost to follow up. CL tolerability was high (24/27,89%) - 2 found the CLs irritant, and 1 had an exacerbation of allergic eye disease. At two weeks, mean improvement in binocular visual acuity from baseline with plano CLs was 0.07 logMAR (95% confidence interval (CI: 0.03-0.11) and 0.06 logMAR with fully corrective CLs (95% CI:0.02-0.1). Mean improvement in the eXpanded Nystagmus Acuity Function (NAFX, a nystagmus acuity function based on eye movement recording) with plano CLs was -0.04(95% CI: -0.08-0.005) and -0.05 with fully corrective CLs(95% CI: -0.09–0.003).

Conclusions: CLs are well tolerated, with a low risk profile. Whilst our study was not powered to detect significant changes in BCVA and waveform parameters between treatment arms, we observed a trend towards an improvement in visual function at two weeks from baseline with CLs.     

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.     

眼球震颤阻滞综合征的临床研究

眼球震颤阻滞综合征的特点为内斜视前先有眼球震颤,双侧外展神经假性麻痹以及当注视眼由内转位向外转位运动时,出现显性眼球震颤。本文结合临床资料就其发病率、临床表现、鉴别诊断和手术方法作了简要讨论。     

Being "slow to see" is a dynamic visual function consequence of infantile nystagmus syndrome: model predictions and patient data identify stimulus timing as its cause

The objective of this study was to investigate the dynamic properties of infantile nystagmus syndrome (INS) that affect visual function; i.e., which factors influence latency of the initial reflexive saccade (Ls) and latency to target acquisition (Lt). We used our behavioral ocular motor system (OMS) model to simulate saccadic responses (in the presence of INS) to target jumps at different times within a single INS cycle and at random times during multiple cycles. We then studied the responses of 4 INS subjects with different waveforms to test the model's predictions. Infrared reflection was used for 1 INS subject, high-speed digital video for 3. We recorded and analyzed human responses to large and small target-step stimuli. We evaluated the following factors: stimulus time within the cycle (Tc), normalized Tc (Tc%), initial orbital position (Po), saccade amplitude, initial retinal error (e(i)), and final retinal error (e(f)). The ocular motor simulations were performed in MATLAB Simulink environment and the analysis was performed in MATLAB environment using OMLAB software. Both the OMS model and OMtools software are available from http://http:www.omlab.org. Our data analysis showed that for each subject, Ls was a fixed value that is typically higher than the normal saccadic latency. Although saccadic latency appears somewhat lengthened in INS, the amount is insufficient to cause the "slow-to-see" impression. For Lt, Tc% was the most influential factor for each waveform type. The main refixation strategies employed by INS subjects made use of slow and fast phases and catch-up saccades, or combinations of them. These strategies helped the subjects to foveate effectively after target movement, sometimes at the cost of increased target acquisition time. Foveating or braking saccades intrinsic to the nystagmus waveforms seemed to disrupt the OMS' ability to accurately calculate reflexive saccades' amplitude and refoveate. Our OMS model simulations demonstrated this emergent behavior and predicted the lengthy target acquisition times found in the patient data.     

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.     

Nystagmus induced by intermittent photic stimulation (flash induced nystagmus (FIN))

In man, as has been shown previously in rabbits and monkeys, a nystagmus can be elicited by monocular intermittent photic stimulation. The direction of nystagmus (FIN) is always towards the stimulated eye.This nystagmus strongly resembles latent nystagmus. Here the fast phase is always directed towards the non-occluded eye. The FIN may, therefore, be used as a model for the study of mechanisms underlying these pathological conditions.For further elucidation of FIN, studies were made in rabbits.