Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
Eur J Neurol. 2011 Aug;18(8):1105-8. doi: 10.1111/j.1468-1331.2010.03309.x. Epub 2011 Jan 25.
The P3 event-related potential (ERP) is presumably partly generated by the basal ganglia. Because degeneration of these brain structures starts many years before clinical disease onset in Huntington's disease (HD), studying the interplay between P3 characteristics and basal ganglia volumes in 'premanifest' carriers might lead to new insights into the disease process.
Fourteen premanifest\ HD mutation carriers and twelve non-mutation carriers underwent clinical, MRI and P3-ERP investigations. The P3 was measured during the Sustained Attention to Response Task.
P3 amplitude and latency did not differ between groups. In carriers, longer P3 latency during Go-trials was strongly associated with smaller caudate, putamen and globus pallidus volumes (r values up to -0.827, P ≤ 0.001).
The exceptionally strong relations of P3 latency with basal ganglia volumes in carriers suggest that the P3 may provide a marker for disease progression in HD.
P3 事件相关电位（ERP）可能部分由基底神经节产生。由于这些脑结构的退化在亨廷顿病（HD）的临床疾病发作前许多年就开始了，因此研究“前显症”携带者中 P3 特征与基底神经节体积之间的相互作用可能会为疾病过程提供新的见解。
14 名前显症 HD 突变携带者和 12 名非突变携带者接受了临床、MRI 和 P3-ERP 检查。P3 在持续注意反应任务中进行测量。
组间 P3 振幅和潜伏期无差异。在携带者中，Go 试验时较长的 P3 潜伏期与尾状核、壳核和苍白球体积较小呈强烈相关（r 值高达-0.827，P≤0.001）。
携带者中 P3 潜伏期与基底神经节体积之间的异常强关系表明，P3 可能为 HD 疾病进展提供一个标志物。
