Diamond R, White R F, Myers R H, Mastromauro C, Koroshetz W J, Butters N, Rothstein D M, Moss M B, Vasterling J
Department of Neurology, Boston University School of Medicine, MA 02118-2394.
J Clin Exp Neuropsychol. 1992 Nov;14(6):961-75. doi: 10.1080/01688639208402547.
Asymptomatic persons at risk for Huntington's disease (HD) (N = 28) were assessed with neuropsychological, psychiatric, and neurologic tests while undergoing genetic linkage studies to determine their probability of carrying the HD gene. Those participants who were subsequently identified as probable gene carriers did not differ on neurologic or psychiatric examination from those subsequently identified as probable noncarriers. Neuropsychological data are presented for a subset of participants free of other conditions (such as alcoholism) putting them at risk for cognitive deficits. Among these subjects, probable gene carriers were inferior to probable noncarriers on the neuropsychological battery as a whole and on several individual tests involving learning and memory. The results suggest the presence of cognitive decline prior to identifiable motor impairments in HD.
对有患亨廷顿舞蹈症（HD）风险的无症状个体（N = 28）进行了神经心理学、精神病学和神经学测试，同时进行基因连锁研究以确定他们携带HD基因的可能性。那些随后被确定为可能的基因携带者的参与者在神经学或精神病学检查方面与那些随后被确定为可能的非携带者没有差异。本文呈现了一部分未患有其他导致认知缺陷风险疾病（如酒精中毒）的参与者的神经心理学数据。在这些受试者中，总体而言，可能的基因携带者在神经心理学测试组以及几项涉及学习和记忆的单项测试中表现不如可能的非携带者。结果表明，在HD患者出现可识别的运动障碍之前就存在认知衰退。
