Faculty of Medicine, Maastricht University, Maastricht, the Netherlands.
Rett Expertise Center - Governor Kremers Center, Maastricht University Medical Center, Maastricht, the Netherlands.
Eur J Paediatr Neurol. 2019 Mar;23(2):262-269. doi: 10.1016/j.ejpn.2018.12.003. Epub 2018 Dec 18.
Rett syndrome (RTT) is a neurological disorder characterized by a broad spectrum of symptoms. Communication is a major area of difficulty. Use of eye tracking technology offers a potentially effective method of communication when underpinned by intact oculomotor function. In this study, oculomotor function was assessed using electronystagmography (ENG). However, challenges were encountered when examining individuals with RTT.
To improve oculomotor examination in individuals with RTT by evaluating the challenges encountered during ENG examination.
Oculomotor function was examined in 17 girls and young women with RTT and 16 typically developing (TD) individuals using ENG. Observational analysis of both performance and results indicated that challenges in examination were mainly related to quality of attention and quality of signals. Subsequently these outcome values were explored quantitatively according to percentage looking time for attention and drift for signal quality.
A significantly reduced level of attention and suboptimal electrode signals were evident in the RTT group when compared with the TD group for all tests except torsion swing.
The challenges in testing confirm that regular oculomotor examination should be adjusted to meet the needs of individuals with RTT. It is hypothesized that the RTT group's higher quality of attention on the torsion swing can be explained by the more forceful vestibular rather than visual-ocular stimulus operating in this test. Suggested adaptations include reducing the number of electrodes, changing the picture stimuli and bringing them closer, performing observational assessments rather than ENG, and using virtual reality goggles.
雷特综合征（RTT）是一种神经发育障碍，其症状表现广泛。沟通是其主要的困难领域之一。当眼动追踪技术得到完整的眼球运动功能的支持时，它提供了一种潜在有效的交流方法。在这项研究中，使用眼电图（ENG）评估眼球运动功能。然而，在检查 RTT 患者时遇到了挑战。
通过评估 ENG 检查中遇到的挑战，改善 RTT 患者的眼动检查。
使用 ENG 对 17 名患有 RTT 的女孩和年轻女性以及 16 名典型发育（TD）个体的眼球运动功能进行了检查。对表现和结果进行观察性分析表明，检查中的挑战主要与注意力质量和信号质量有关。随后，根据注意力的注视时间百分比和信号质量的漂移，对这些结果值进行了定量探索。
与 TD 组相比，所有测试（除扭转摆动外）中，RTT 组的注意力水平明显降低，电极信号质量不佳。
测试中的挑战证实，应根据 RTT 患者的需求调整常规眼动检查。假设 RTT 组在扭转摆动测试中具有更高质量的注意力，可以用更强烈的前庭刺激而不是视觉-眼动刺激来解释。建议的调整包括减少电极数量、改变图片刺激并使其更接近、进行观察评估而不是 ENG 检查，以及使用虚拟现实护目镜。
