Error Processing in Huntington's Disease
Error Processing in Huntington's Disease
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DOI：
10.1371/journal.pone.0000086
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发表时间：
2006-12-20
期刊：
影响因子：
3.7
通讯作者：
Falkenstein, Michael
中科院分区：
文献类型：
--
作者：
Beste, Christian;Saft, Carsten;Falkenstein, Michael
背景。亨廷顿病（HD）是一种遗传性疾病，表现为基底神经节变性，尤其伴有多巴胺能改变。这些多巴胺能改变与遗传因素（即 CAG 重复扩增）有关。错误（相关）负性（Ne/ERN）是一种与表现监测相关的认知事件相关电位，在前扣带皮层（ACC）中产生，并且被认为依赖于多巴胺能系统。帕金森病 (PD) 中的 Ne 会减少。由于 HD 中多巴胺能缺陷，Ne 也可能减少。此外，假设运动功能障碍是由于错误反馈处理功能失调而出现的。由于多巴胺能的改变与 CAG 重复有关，因此 Ne 的减少也可能与遗传病负荷有关。方法/原则发现。我们在考虑潜在的遗传异常的情况下评估了快速反应任务中的错误负性（Ne）。 HD 患者表现出 Ne 的特定减少，这表明这些患者的错误处理能力受损。此外，Ne与CAG重复扩增密切相关。结论/意义。 Ne 的减少可能是多巴胺能病理学的影响。该结果类似于帕金森病的研究结果。因此，Ne 可能是纹状体多巴胺能输出功能完整性的衡量标准。与 CAG 重复扩展的关系表明 Ne 可以作为 HD 中与基因相关的“认知”生物标志物。
Background. Huntington's disease (HD) is a genetic disorder expressed by a degeneration of the basal ganglia inter alia accompanied with dopaminergic alterations. These dopaminergic alterations are related to genetic factors i.e., CAG-repeat expansion. The error (related) negativity (Ne/ERN), a cognitive event-related potential related to performance monitoring, is generated in the anterior cingulate cortex (ACC) and supposed to depend on the dopaminergic system. The Ne is reduced in Parkinson's Disease (PD). Due to a dopaminergic deficit in HD, a reduction of the Ne is also likely. Furthermore it is assumed that movement dysfunction emerges as a consequence of dysfunctional error-feedback processing. Since dopaminergic alterations are related to the CAG-repeat, a Ne reduction may furthermore also be related to the genetic disease load. Methodology/Principle Findings. We assessed the error negativity (Ne) in a speeded reaction task under consideration of the underlying genetic abnormalities. HD patients showed a specific reduction in the Ne, which suggests impaired error processing in these patients. Furthermore, the Ne was closely related to CAG-repeat expansion. Conclusions/Significance. The reduction of the Ne is likely to be an effect of the dopaminergic pathology. The result resembles findings in Parkinson's Disease. As such the Ne might be a measure for the integrity of striatal dopaminergic output function. The relation to the CAG-repeat expansion indicates that the Ne could serve as a gene-associated "cognitive'' biomarker in HD.