Premanifest Huntington's disease (pre-HD) individuals typically show increased task-related functional magnetic resonance imaging (fMRI), suggested to reflect compensatory strategies. Despite the evidence, no study has attempted to understand the compensatory process in light of 'formal' models of compensation. We used a quantitative model of compensation - the Compensation-Related Utilization of Neural Circuits Hypothesis (CRUNCH), to characterise compensation in pre-HD using fMRI. Pre-HD individuals (n = 15) and controls (n = 15) performed a modified stop-signal task that incremented in four levels of stop difficulty. Our results did not support the critical assumption of the CRUNCH model - controls did not show increased fMRI activity with increased level of stop difficulty; however, controls showed decreased fMRI activity with increased stop difficulty in right inferior frontal gyrus and right caudate nucleus. Relative to controls, pre-HD individuals showed increased fMRI activity in right inferior frontal gyrus and in right caudate nucleus at higher levels of stop difficulty, which is the opposite effect to that predicted by the model. Our findings suggest a compensatory process of the response inhibition network in pre-HD; however, the pattern of fMRI activity was not in the manner expected by CRUNCH.

中文翻译：

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预示性亨廷顿氏病反应抑制期间的大脑补偿。

亨廷顿病前期（HD）个体通常表现出与任务相关的功能性磁共振成像（fMRI）增加，建议反映补偿策略。尽管有证据，但没有研究试图根据“正式”的补偿模型来理解补偿过程。我们使用了定量的补偿模型-神经回路假说的补偿相关利用（CRUNCH），以使用fMRI来表征HD前期的补偿。HD前个体（n = 15）和对照（n = 15）执行了修改的停止信号任务，该任务以四种难度级别增加。我们的结果不支持CRUNCH模型的关键假设-对照并未显示功能性核磁共振成像活动增加且停止难度增加。然而，对照组显示fMRI活动减少，右下额回和右尾状核停止困难增加。相对于对照，HD前个体在较高的停止难度下在右下额回和右尾状核中显示出功能增强的核磁共振成像活性，这与模型所预测的相反。我们的发现提示HD前反应抑制网络的补偿过程。但是，功能磁共振成像活动的模式与CRUNCH预期的方式不同。我们的发现提示HD前反应抑制网络的补偿过程。但是，功能磁共振成像活动的模式与CRUNCH预期的方式不同。我们的发现提示HD前反应抑制网络的补偿过程。但是，功能磁共振成像活动的模式与CRUNCH预期的方式不同。