Robins Wahlin Tarja-Brita, Lundin Anders, Dear Keith
Karolinska Institutet, Department of Neurobiology, Caring Sciences and Society, Stockholm, Sweden.
Neuropsychology. 2007 Jan;21(1):31-44. doi: 10.1037/0894-4105.21.1.31.
The primary focus of this study was to examine whether there is early neuropsychological impairment in presymptomatic Huntington's disease (HD). A broad neuropsychological assessment battery was administered to 24 asymptomatic gene carriers (HD+) and 31 noncarriers (HD-). The gene carriers revealed inferior cognitive functioning as compared with the noncarriers in memory and executive functions. When the gene carriers were assigned to 2 groups based on predicted years to onset (with 15 and over being HD+ late and under 15 being HD+ near), the HD+ near group performed significantly worse than the HD+ late group in all domains but ability to shift conceptually and visuospatial memory. Results suggest that early cognitive deficits are detectable prior to motor symptoms, first in memory functions and then in executive functions and perceptual motor speed.
本研究的主要重点是检查症状前亨廷顿舞蹈病（HD）患者是否存在早期神经心理损害。对24名无症状基因携带者（HD+）和31名非携带者（HD-）进行了广泛的神经心理评估。与非携带者相比，基因携带者在记忆和执行功能方面表现出较差的认知功能。当根据预测发病年限将基因携带者分为两组时（15年及以上为HD+晚期，15年以下为HD+早期），HD+早期组在所有领域的表现均显著差于HD+晚期组，但在概念转换能力和视觉空间记忆方面除外。结果表明，在运动症状出现之前就可以检测到早期认知缺陷，首先是在记忆功能方面，然后是在执行功能和感知运动速度方面。
