Hu Xiaopeng, Chen Siyi, Huang Chang-Bing, Qian Yinfeng, Yu Yongqiang
Department of Radiology, First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China.
Key Laboratory of Behavioral Science, Institute of Psychology, Chinese Academy of Sciences, Beijing, 100101, China.
Metab Brain Dis. 2017 Jun;32(3):685-692. doi: 10.1007/s11011-016-9946-3. Epub 2017 Jan 24.
To investigate the frequency-dependent changes in the amplitude of low-frequency fluctuations (ALFF) in patients with Wilson's disease (WD). Resting-state function magnetic resonance imaging (R-fMRI) were employed to measure the amplitude of ALFF in 28 patients with WD and 27 matched normal controls. Slow-5 (0.01-0.027 Hz) and slow-4 (0.027-0.073 Hz) frequency bands were analyzed. Apart from the observation of atrophy in the cerebellum, basal ganglia, occipital gyrus, frontal gyrus, precentral gyrus, and paracentral lobule, we also found widespread differences in ALFF of the two bands in the medial frontal gyrus, inferior temporal gyrus, insula, basal ganglia, hippocampus/parahippocampal gyrus, and thalamus bilaterally. Compared to normal controls, WD patients had increased ALFF in the posterior lobe of the cerebellum, inferior temporal gyrus, brain stem, basal ganglia, and decreased ALFF in the anterior lobe of the cerebellum and medial frontal gyrus. Specifically, we observed that the ALFF abnormalities in the cerebellum and middle frontal gyrus were greater in the slow-5 than in the slow-4 band. Correlation analysis showed consistently positive correlations between urinary copper excretion (Cu), serum ceruloplasmin (CP) and ALFFs in the cerebellum. Our study suggests the accumulation of copper profoundly impaired intrinsic brain activity and the impairments seem to be frequency-dependent. These results provide further insights into the understanding of the pathophysiology of WD.
为研究肝豆状核变性（WD）患者低频波动幅度（ALFF）的频率依赖性变化。采用静息态功能磁共振成像（R-fMRI）测量28例WD患者和27例匹配的正常对照者的ALFF。分析了慢5（0.01-0.027Hz）和慢4（0.027-0.073Hz）频段。除观察到小脑、基底神经节、枕叶、额叶、中央前回和中央旁小叶萎缩外，我们还发现双侧内侧额叶回、颞下回、岛叶、基底神经节、海马/海马旁回和丘脑这两个频段的ALFF存在广泛差异。与正常对照相比，WD患者小脑后叶、颞下回、脑干、基底神经节的ALFF增加，小脑前叶和内侧额叶回的ALFF降低。具体而言，我们观察到小脑和额中回的ALFF异常在慢5频段比慢4频段更明显。相关性分析显示尿铜排泄量（Cu）、血清铜蓝蛋白（CP）与小脑ALFF之间始终呈正相关。我们的研究表明铜的蓄积严重损害了大脑的内在活动，且这种损害似乎具有频率依赖性。这些结果为深入了解WD的病理生理学提供了进一步的见解。
