Josiassen R C, Curry L M, Mancall E L
Arch Neurol. 1983 Dec;40(13):791-6. doi: 10.1001/archneur.1983.04050120041005.
Patients with more recently diagnosed Huntington's disease (RHD) were compared on a neuropsychological test battery with patients who had had the disease one to eight years (moderate HD) and with asymptomatic offspring. Though the patients with HD showed deficits in motor and motor-independent abilities, the patients with RHD were less impaired on factors assessing visuospatial ability, cognitive flexibility, and motor steadiness. Of particular interest was the finding that some asymptomatic offspring showed deficits in visuospatial ability and auditory memory that resembled the deficits of patients with RHD. The findings suggest that deficits in HD do not develop at a uniform rate; deficits in cognitive flexibility occur in later stages of the disease; and visuospatial and auditory memory deficits are very early signs and are present in some asymptomatic offspring.
将近期诊断出患有亨廷顿舞蹈症（RHD）的患者与患病一至八年的患者（中度HD）以及无症状的后代进行了一系列神经心理学测试对比。尽管患有HD的患者在运动和非运动能力方面存在缺陷，但在评估视觉空间能力、认知灵活性和运动稳定性的因素上，RHD患者受损程度较轻。特别值得关注的是，一些无症状的后代在视觉空间能力和听觉记忆方面表现出与RHD患者相似的缺陷。研究结果表明，HD的缺陷并非以统一的速度发展；认知灵活性缺陷出现在疾病后期；视觉空间和听觉记忆缺陷是非常早期的症状，且在一些无症状的后代中也存在。
