Biologically relevant models of infantile nystagmus syndrome: the requirement for behavioral ocular motor system models.
Biologically relevant models of infantile nystagmus syndrome: the requirement for behavioral ocular motor system models.
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DOI：
10.1080/08820530600613795
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发表时间：
2006-04-01
影响因子：
1.7
通讯作者：
Dell'Osso, L F
中科院分区：
文献类型：
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作者：
Dell'Osso, L F
婴儿眼球震颤综合征(INS)是几种类型的眼球震颤的组合，每种类型的眼球震颤都代表眼球运动系统(OMS)的一个子系统功能障碍，并有独特的波形。眼球运动记录是唯一确定的识别IN并与其他类型区分的方法。使用了30年的波形分类方案既准确、包容，又暗示了潜在的子系统不稳定性。在不同的个体中，它们可能出现在出生时(硬连接)或早期婴儿(发育中)。假设IN的主要子系统不稳定存在于正常减弱的平滑追踪系统；也可能存在前庭功能障碍(失衡)。少数情况下，视束核可能会受累。过去40年的眼球运动研究表明，眼跳和凝视保持在INS中是正常的，而IN波形中包含的眼跳总是正确的；即它们不是导致IN的起始运动。由于有无限多的解决方案来模拟特定的波形，仅产生孤立地类似IN的波形的模型在临床上或在增加我们对IN的潜在机制的理解方面用处不大。INS的生物相关模型应该是完整的OMS模型的一部分，并在其中操作，能够在仍在振荡的情况下再现这些人的正常眼运动行为；即，该模型必须像患者一样，不能有震颤，并且能够对各种目标输入做出正确的反应。
Infantile nystagmus syndrome (INS) is a combination of several types of nystagmus, each representing dysfunction in one subsystem of the ocular motor system (OMS) and having characteristic waveforms. Eye-movement recordings are the only certain way to identify IN and differentiate it from other types. The waveform classification scheme in use for 30 years is both accurate, inclusive, and suggests the underlying subsystem instabilities. In different individuals, they may appear at birth (hard wired) or in early infancy (developmental). The primary subsystem instability in IN is hypothesized to lie in the normally underdamped smooth pursuit system; vestibular dysfunction (imbalance) may also be present. Less often, the nucleus of the optic tract may be involved. Ocular motility studies over the past 40 years have demonstrated that saccades and gaze holding are normal in the INS and saccades contained within IN waveforms are always corrective; i.e., they cannot be the initiating movement responsible for IN. Because there are an infinite number of solutions to simulating specific waveforms, models that merely generate waveforms that resemble IN in isolation are of little use, either clinically or to increase our understanding of the underlying mechanisms of IN. A biologically relevant model of the INS should be part of, and operate within, a complete OMS model, capable of reproducing the normal ocular motor behavior of these individuals while still oscillating; i.e., the model, like the patient, must not have oscillopsia and be able to respond correctly to various target inputs.