Ho A K, Sahakian B J, Brown R G, Barker R A, Hodges J R, Ané M-N, Snowden J, Thompson J, Esmonde T, Gentry R, Moore J W, Bodner T
University of Cambridge, UK.
Neurology. 2003 Dec 23;61(12):1702-6. doi: 10.1212/01.wnl.0000098878.47789.bd.
To examine the pattern of cognitive decline in early Huntington's disease (HD).
The authors studied 61 patients with mild to moderate HD who had at least three annual neuropsychological assessments using the Core Assessment Program for Intracerebral Transplantation in Huntington's Disease short battery. A subset of 34 patients had additional neuropsychological tests, and another subset of 21 patients was assessed annually on the Cambridge Neuropsychological Test Automated Battery. Neuropsychological measures that changed significantly over time were submitted to a multiple analysis of covariance to explore associations with demographic and neurologic indices.
Patients showed a progressive impairment in attention, executive function, and immediate memory, with timed tests of psychomotor skill being particularly sensitive to decline. In contrast, general cognition, semantic memory, and delayed recall memory were relatively unaffected.
The profile of cognitive performance shows selective and progressive dysfunction of attention and executive function in patients with mild to moderate HD, consistent with frontostriatal pathology at this stage of disease.
研究早期亨廷顿舞蹈症（HD）的认知衰退模式。
作者对61例轻度至中度HD患者进行了研究，这些患者至少接受过三次年度神经心理学评估，采用亨廷顿舞蹈症脑内移植核心评估程序简版。34例患者的一个子集接受了额外的神经心理学测试，另外21例患者的一个子集每年接受剑桥神经心理学测试自动成套测验评估。对随时间显著变化的神经心理学指标进行多元协方差分析，以探索与人口统计学和神经学指标的关联。
患者在注意力、执行功能和即时记忆方面表现出进行性损害，心理运动技能的定时测试对衰退尤为敏感。相比之下，一般认知、语义记忆和延迟回忆记忆相对未受影响。
认知表现概况显示，轻度至中度HD患者存在注意力和执行功能的选择性和进行性功能障碍，与疾病该阶段的额纹状体病理学一致。
