Vaca-Palomares Israel, Díaz Rosalinda, Rodríguez-Labrada Roberto, Medrano-Montero Jacqueline, Aguilera-Rodríguez Raúl, Vázquez-Mojena Yaimeé, Fernandez-Ruiz Juan, Velázquez-Pérez Luis
1Laboratory of Neuropsychology,Department of Physiology, Faculty of Medicine,National Autonomous University of Mexico,Coyoacan,Mexico City 04510,Mexico City,Mexico.
2Centre for the Investigation and Rehabilitation of Hereditary Ataxias,Holguin,Cuba.
J Int Neuropsychol Soc. 2015 Mar;21(3):214-20. doi: 10.1017/S1355617715000132. Epub 2015 Mar 23.
Our goal was to improve spinocerebellar ataxia type 2 (SCA2) cognitive profile characterization by testing the hypothesis that strategy, planning and rule acquisition capacities are affected in SCA2. Forty one patients with SCA2 were evaluated with the Spatial Working Memory (SWM), the Stockings of Cambridge (SOC), and the Intra-Extra Dimensional Shift (IED) tests of the Executive module of the Cambridge Neuropsychological Testing Automated Battery (CANTAB). Paired Associates Learning (PAL) and Delayed Matching to Sample (DMS) from the CANTAB memory module were also assessed to corroborate previous findings. Motor deterioration was measured using the Scale for the Assessment and Rating of Ataxia (SARA). We found significant SCA2 related deficits in strategy, planning, and rule acquisition. Our results also corroborated significant memory deficits in these patients with SCA2. Further analysis also showed that patients with large motor deterioration had poorer associative learning and spatial planning scores. Patients with SCA2 show strategy, planning, and rule acquisition deficits as revealed with the CANTAB battery. These deficits should be noted when planning an effective therapy for these patients.
我们的目标是通过检验脊髓小脑共济失调2型（SCA2）中策略、计划和规则习得能力受到影响这一假设，来改善对SCA2认知特征的描述。对41例SCA2患者进行了剑桥神经心理测试自动成套系统（CANTAB）执行模块中的空间工作记忆（SWM）、剑桥长袜（SOC）和维度内-维度间转换（IED）测试评估。还对CANTAB记忆模块中的配对联想学习（PAL）和延迟匹配样本（DMS）进行了评估，以证实先前的研究结果。使用共济失调评估与分级量表（SARA）测量运动功能恶化情况。我们发现SCA2患者在策略、计划和规则习得方面存在显著缺陷。我们的结果也证实了这些SCA2患者存在显著的记忆缺陷。进一步分析还表明，运动功能恶化严重的患者在联想学习和空间计划方面的得分较低。CANTAB测试显示，SCA2患者存在策略、计划和规则习得缺陷。在为这些患者制定有效治疗方案时应注意这些缺陷。
