Maruff P, Tyler P, Burt T, Currie B, Burns C, Currie J
Neurophysiology and Neurovisual Research Unit, Mental Health Research Institute of Victoria, Parkville, Melbourne, Australia.
Ann Neurol. 1996 Sep;40(3):421-7. doi: 10.1002/ana.410400311.
Cognitive function was examined in 6 patients with genetically confirmed Machado-Joseph disease (MJD) and 15 age- and ethnically matched controls using a series of subtests from the Cambridge Neuropsychological Test Automated Battery (CANTAB), a touch screen-based testing system previously validated in a number of movement and neurodegenerative disorders. The MJD patients had deficits in visual attentional function that were characterized by a slowing of the processing of visual information when task demands were high and an inability to shift attention to previously irrelevant stimulus dimensions to discriminate between complex stimuli. Tests of learning and visual memory were normal. These results demonstrate that specific cognitive deficits occur in patients with MJD, independent of motor dysfunction, and these deficits may reflect disruption to frontosubcortical pathways.
使用剑桥神经心理测试自动成套系统（CANTAB）的一系列子测试，对6例基因确诊的马查多-约瑟夫病（MJD）患者和15名年龄及种族匹配的对照者的认知功能进行了检查。CANTAB是一个基于触摸屏的测试系统，此前已在多种运动和神经退行性疾病中得到验证。MJD患者存在视觉注意力功能缺陷，其特征是在任务要求较高时视觉信息处理速度减慢，并且无法将注意力转移到先前无关的刺激维度以区分复杂刺激。学习和视觉记忆测试结果正常。这些结果表明，MJD患者存在特定的认知缺陷，与运动功能障碍无关，这些缺陷可能反映了额叶皮质下通路的破坏。
