Lasker A G, Zee D S
Department of Neurology, Johns Hopkins Hospital, Baltimore, MD 21267, USA.
Vision Res. 1997 Dec;37(24):3639-45. doi: 10.1016/S0042-6989(96)00169-1.
We review here the eye movements in patients with Huntington's disease (HD), concentrating upon saccades as they show the most prominent abnormalities. Inability to suppress reflexive glances to suddenly appearing novel visual stimuli and delayed initiation of voluntary saccades, including predictive saccades, are early and consistent findings. These two abnormalities can be interpreted in the context of a model, based upon the idea that the frontal lobes and basal ganglia contribute more to the control of voluntary than to reflexive types of saccades. Most patients eventually also show slow saccades but they are most prominent when the disease is early-onset. Slowing of saccades may reflect involvement of both the higher-level cerebral centers that trigger saccades and the areas in the brain stem that produce premotor saccade commands. The study of eye movements in HD has led to a fruitful interaction between basic science and clinical investigation, and has served as a paradigm for examining higher-level defects in saccadic eye movement control in patients with various degenerative, neurological diseases or with focal cerebral hemispheral lesions.
我们在此回顾亨廷顿舞蹈症（HD）患者的眼球运动，重点关注扫视，因为它们表现出最显著的异常。无法抑制对突然出现的新视觉刺激的反射性扫视以及包括预测性扫视在内的自主扫视启动延迟，是早期且一致的发现。这两种异常可以在一个模型的背景下进行解释，该模型基于额叶和基底神经节对自主扫视控制的贡献比对反射性扫视的贡献更大这一观点。大多数患者最终也会出现缓慢扫视，但在疾病早发时最为明显。扫视减慢可能反映了触发扫视的高级脑中枢以及产生运动前扫视指令的脑干区域均受到影响。对HD患者眼球运动的研究促成了基础科学与临床研究之间富有成效的互动，并成为检查患有各种退行性、神经性疾病或局灶性脑半球病变患者扫视眼运动控制中高级缺陷的范例。
