Bachoud-Lévi A C, Maison P, Bartolomeo P, Boissé M F, Dalla Barba G, Ergis A M, Baudic S, Degos J D, Cesaro P, Peschanski M
Département de Neurosciences Médicales, CHU Henri Mondor, Créteil, France.
Neurology. 2001 Apr 24;56(8):1052-8. doi: 10.1212/wnl.56.8.1052.
To assess the natural progression of cognitive impairment in Huntington's disease (HD) and to reveal factors that may mask this progression.
Although numerous cross-sectional studies reported cognitive deterioration at different stages of the disease, progressive cognitive deterioration has been, up to now, difficult to demonstrate in neuropsychological longitudinal studies.
The authors assessed 22 patients in early stages of HD at yearly intervals for 2 to 4 years (average, 31.2 +/- 10 months), using a comprehensive neuropsychological battery based on the Core Assessment Program for Intracerebral Transplantation in Huntington's Disease (CAPIT-HD).
The authors observed a significant decline in different cognitive functions over time: these involved primarily attention and executive functions but also involved language comprehension, and visuospatial immediate memory. Episodic memory impairment that was already present at the time of enrollment did not show significant decline. The authors found a significant retest effect at the second assessment in many tasks.
Many attention and executive tasks adequately assess the progression of the disease at an early stage. For other functions, the overlapping of retest effects and disease progression may confuse the results. High interindividual and intraindividual variability seem to be hallmarks of the disease.
评估亨廷顿舞蹈病（HD）认知障碍的自然进展情况，并揭示可能掩盖这种进展的因素。
尽管众多横断面研究报告了该疾病不同阶段的认知衰退，但迄今为止，在神经心理学纵向研究中，渐进性认知衰退仍难以得到证实。
作者使用基于亨廷顿舞蹈病脑内移植核心评估项目（CAPIT-HD）的综合神经心理学测试组，对22例HD早期患者进行了为期2至4年（平均31.2±10个月）的年度评估。
作者观察到不同认知功能随时间显著下降：主要涉及注意力和执行功能，但也涉及语言理解和视觉空间即刻记忆。入组时就已存在的情景记忆障碍未显示出显著下降。作者发现在第二次评估时，许多任务存在显著的重测效应。
许多注意力和执行任务能充分评估疾病早期的进展情况。对于其他功能，重测效应和疾病进展的重叠可能会混淆结果。个体间和个体内的高度变异性似乎是该疾病的特征。
