Department of Neurobiology and Anatomy, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA.
J Neurol Sci. 2012 Feb 15;313(1-2):35-41. doi: 10.1016/j.jns.2011.09.035. Epub 2011 Oct 21.
Huntington disease (HD) is a genetic, neurodegenerative disorder characterized by chorea, behavioral co-morbidities, cognitive deficits, and eye movement abnormalities. We sought to evaluate whether reflexive and voluntary orienting prove useful as biomarkers of disease severity in HD.
Eleven HD subjects were evaluated with the motor subscale of the Unified Huntington Disease Rating Scale (UHDRS) and the Montreal Cognitive Assessment. Using an infrared eye tracker, we also measured latency and error rates of horizontal and vertical saccades using prosaccade and antisaccade eye movement tasks. We calculated simple and age-controlled correlations between eye movement and clinical parameters.
Prosaccade latency correlated with total chorea score. HD patients with greater clinical severity were significantly slower in the prosaccade task. Antisaccade error rate also correlated with UHDRS motor score and total chorea score. HD patients with greater clinical severity as measured by either measure made significantly more errors in the antisaccade task. All these correlations remained significant even when age was taken into account.
The results of the present age-controlled study show for the first time that both reflexive and voluntary eye motor control in HD patients decrease with increase in disease severity suggesting declines in both motor and cognitive function. Thus, relatively simple eye movement parameters (latency and error rate) obtained from simple tasks (prosaccade and antisaccade) may serve as quantitative biomarkers of sub-cortical and cortical disease severity in HD and could aid in predicting onset, distinguishing subtypes, or evaluating disease progression and novel therapies.
亨廷顿病（HD）是一种遗传性神经退行性疾病，其特征为舞蹈症、行为共病、认知缺陷和眼球运动异常。我们试图评估反射性和自主性定向是否可作为 HD 疾病严重程度的生物标志物。
11 名 HD 受试者接受了统一亨廷顿病评定量表（UHDRS）运动量表和蒙特利尔认知评估的评估。使用红外眼动追踪器，我们还通过正性和负性眼动任务测量了水平和垂直扫视的潜伏期和错误率。我们计算了眼动与临床参数之间的简单和年龄校正相关性。
正性扫视潜伏期与总舞蹈评分相关。临床严重程度较高的 HD 患者在正性扫视任务中明显较慢。负性扫视错误率也与 UHDRS 运动评分和总舞蹈评分相关。无论是用哪种措施来衡量，临床严重程度较高的 HD 患者在负性扫视任务中都会犯更多的错误。即使考虑到年龄，这些相关性仍然显著。
本年龄对照研究的结果首次表明，HD 患者的反射性和自主性眼球运动控制均随疾病严重程度的增加而降低，提示运动和认知功能均下降。因此，来自简单任务（正性和负性扫视）的相对简单的眼动参数（潜伏期和错误率）可能作为 HD 皮质下和皮质疾病严重程度的定量生物标志物，并有助于预测发病、区分亚型或评估疾病进展和新疗法。
