Hamedani Ali G, Bardakjian Tanya, Balcer Laura J, Gonzalez-Alegre Pedro
Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Departments of Neurology, Ophthalmology and Population Health, New York University School of Medicine, New York, New York, USA.
Neuroophthalmology. 2019 Nov 25;44(4):219-225. doi: 10.1080/01658107.2019.1669668. eCollection 2020.
Saccadic eye movement abnormalities are among the earliest manifestations of Huntington's disease (HD) but are difficult to quantify at the bedside. Similarly, afferent visual pathway involvement in HD is poorly characterised. The objective was to evaluate afferent and efferent visual function in HD. Participants with manifest HD (n = 19) and healthy controls (n = 20) performed the King-Devick test, a timed test of rapid number naming. Binocular high and low-contrast (2.5% and 1.25%) acuities were measured using low-contrast Sloan letter charts, and pupillometric recordings were made using a handheld NeurOptics PLR-3000 pupillometer. The NEI-VFQ-25 questionnaire with 10-item neuro-ophthalmic supplement were also completed. Unified Huntington's Disease Rating Scale (UHDRS) motor score and other clinical and demographic variables were collected. Comparisons between manifest HD and controls were performed using linear regression adjusted for confounders. Mean King-Devick time scores were 102.9 seconds in patients with manifest HD and 48.2 seconds in controls (p < .01, t-test). In unadjusted analyses, binocular high contrast acuity was seven letters (one Snellen line equivalent) lower in manifest HD than controls (p = .043). This effect was similar for low-contrast acuity, but only low-contrast acuity remained statistically significant after adjusting for covariates. Low-contrast acuity also correlated with UHDRS motor score. There were no differences in pupillary reactivity or self-reported vision-related quality of life. In conclusion, HD is associated with reduced low-contrast acuity and abnormal performance on the King-Devick test of rapid number naming. These tests are easy to administer, providing an objective quantitative measure of visual function which could be incorporated into optimised rating scales.
眼球扫视运动异常是亨廷顿舞蹈症（HD）最早出现的症状之一，但在床边难以进行量化评估。同样，HD患者传入视觉通路的受累情况也鲜为人知。本研究旨在评估HD患者的传入和传出视觉功能。对19例显性HD患者和20例健康对照者进行了King-Devick测试，这是一项快速数字命名的计时测试。使用低对比度斯隆字母视力表测量双眼高对比度（2.5%）和低对比度（1.25%）视力，并使用手持NeurOptics PLR-3000瞳孔计进行瞳孔测量记录。还完成了包含10项神经眼科补充内容的NEI-VFQ-25问卷。收集了统一亨廷顿舞蹈症评定量表（UHDRS）运动评分以及其他临床和人口统计学变量。采用针对混杂因素进行调整的线性回归对显性HD患者和对照组进行比较。显性HD患者的平均King-Devick时间得分是102.9秒，对照组为48.2秒（p < .01，t检验）。在未经调整的分析中，显性HD患者的双眼高对比度视力比对照组低7个字母（相当于1行斯内伦视力表）（p = .043）。低对比度视力的情况类似，但在对协变量进行调整后，只有低对比度视力仍具有统计学意义。低对比度视力也与UHDRS运动评分相关。瞳孔反应性或自我报告的视觉相关生活质量没有差异。总之，HD与低对比度视力降低以及在快速数字命名的King-Devick测试中表现异常有关。这些测试易于实施，能够提供视觉功能的客观定量测量，可纳入优化的评定量表中。
