Lasker A G, Zee D S, Hain T C, Folstein S E, Singer H S
Department of Ophthalmology, Johns Hopkins University, School of Medicine, Baltimore, MD.
Neurology. 1988 Mar;38(3):427-31. doi: 10.1212/wnl.38.3.427.
Eye movements were recorded from 20 mildly affected patients with Huntington's disease (HD) who were divided into two groups, 10 patients with onset of symptoms before age 30 and 10 with onset of symptoms after age 30. In the younger onset group (HD less than 30), peak saccade velocities were low (less than 255 deg/sec for 20-deg saccades) in six of the 10 patients, whereas none of the 10 patients in the older onset group (HD greater than 30) had peak saccade velocities lower than 300 deg/sec. Latencies for volitional saccades were greater than normal in the HD greater than 30 group, but were normal for the HD less than 30 group. The ability to maintain steady fixation in the face of a distracting visual stimulus was decreased, to the same degree, in both groups of HD patients. In addition, 70% of the HD less than 30 group had an affected father, while 70% of the HD greater than 30 group had an affected mother. These findings suggest that the pathophysiology of the slow saccades, initiation deficit, and excessive distractibility in HD are different.
对20名轻度亨廷顿舞蹈症（HD）患者的眼球运动进行了记录，这些患者被分为两组，10名症状在30岁之前出现，10名症状在30岁之后出现。在发病年龄较小的组（HD小于30岁）中，10名患者中有6名的扫视峰值速度较低（20度扫视时小于255度/秒），而在发病年龄较大的组（HD大于30岁）中，10名患者中没有一人的扫视峰值速度低于300度/秒。在HD大于30岁的组中，随意性扫视的潜伏期大于正常水平，但在HD小于30岁的组中潜伏期正常。两组HD患者在面对干扰性视觉刺激时保持稳定注视的能力均同等程度下降。此外，HD小于30岁组中有70%的患者父亲患病，而HD大于30岁组中有70%的患者母亲患病。这些发现表明，HD中扫视缓慢、起始缺陷和过度易分心的病理生理学机制有所不同。
