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婴儿眼球震颤综合征(INS)的诊断与治疗。

The diagnosis and treatment of infantile nystagmus syndrome (INS).

作者信息

Khanna Sangeeta, Dell'Osso Louis F

机构信息

Daroff-Dell'Osso Ocular Motility Laboratory, Louis Stokes Cleveland Department of Veterans Affairs Medical Center and CASE Medical School, Cleveland, OH, USA.

出版信息

ScientificWorldJournal. 2006 Oct 30;6:1385-97. doi: 10.1100/tsw.2006.248.

DOI:10.1100/tsw.2006.248
PMID:17086344
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5917301/
Abstract

The successful treatment of infantile nystagmus syndrome (INS) depends primarily on accurate and repeatable diagnosis of the type(s) of nystagmus present as well as their variation with gaze and convergence angles or fixating eye. Research over the past 40 years has demonstrated that the only way to achieve both is by making and analyzing ocular motility recordings. Determination of the direct effects of peripheral and central INS therapies can only be made by pre- and post-therapy comparisons of the nystagmus characteristics, specifically of the quality of the foveation periods within each cycle. If one is only interested in cosmetic improvements, diminution of the nystagmus amplitude is all that need be measured. However, if improvement of visual function is the primary goal of therapy, then measurement of the pre- and post-therapy foveation quality must be made, both in primary position and over a broad range of gaze angles. The use of the eXpanded Nystagmus Acuity Function (NAFX) on nystagmus data yields both an accurate measure of foveation quality and a prediction of maximum potential acuity for the patient's waveform. When used with the patient's measured, pre-therapy visual acuity, the NAFX demonstrates the amount of visual acuity loss that is due to sensory abnormalities, demonstrates the amount due to the nystagmus waveform, and estimates the measured post-therapy acuity for all values of improved NAFX and gaze angles measured. The ability to predict visual acuity improvement was not possible before the use of the NAFX. The failure to incorporate accurate measures of nystagmus waveform and foveation quality into their diagnostic evaluation continues to deprive patients of the best possible standard of care and results in mistaken diagnoses as well as inappropriate and, in some cases, unneeded multiple surgeries.

摘要

婴儿眼球震颤综合征(INS)的成功治疗主要取决于对所存在的眼球震颤类型进行准确且可重复的诊断,以及了解其随注视和集合角度或注视眼的变化情况。过去40年的研究表明,实现这两点的唯一方法是进行并分析眼球运动记录。只有通过治疗前后眼球震颤特征的比较,特别是每个周期内注视期质量的比较,才能确定外周和中枢INS治疗的直接效果。如果仅关注外观改善,那么只需测量眼球震颤幅度的减小情况。然而,如果治疗的主要目标是改善视觉功能,那么就必须在初始位置以及广泛的注视角度范围内测量治疗前后的注视质量。对眼球震颤数据使用扩展的眼球震颤视力功能(NAFX),既能准确测量注视质量,又能预测患者波形的最大潜在视力。当与患者治疗前测得的视力一起使用时,NAFX可显示出由于感觉异常导致的视力损失量、由于眼球震颤波形导致的视力损失量,并能针对所有改善的NAFX值和测得的注视角度估计治疗后的视力。在使用NAFX之前,无法预测视力改善情况。在诊断评估中未能纳入眼球震颤波形和注视质量的准确测量,继续使患者无法获得最佳的护理标准,并导致误诊以及不适当的、在某些情况下甚至是不必要的多次手术。