摘要
目的 探索肝豆状核变性患者大脑皮层功能损伤。方法 收集24例临床确诊的肝豆患者及27名健康志愿者，进行MRI扫描，采集结构像与功能像。选取左右侧苍白球为ROI，计算全脑功能连接，比较肝豆患者与健康志愿者脑功能连接状态。结果 与健康志愿者相比较，肝豆患者大脑左侧苍白球与脑干及双侧丘脑区域功能连接减弱，而右侧苍白球与多个脑区功能连接减弱，包括双侧丘脑、双侧额中回（MFG）、双侧岛叶、左侧前扣带回（ACC）、左侧中扣带回（MCC）、脑干和左侧小脑。结论 结果显示HLD组苍白球功能连接异常脑区分布广泛，包括小脑-丘脑-皮层回路以及凸显网络、共青网络等运动、情感、认知相关网络，这可能是导致HLD患者临床相关症状的重要因素。
关键词： 肝豆状核变性，磁共振成像，基底节区，苍白球
Abstract
Objective To explore the damage of cerebral cortex in patients with hepatolenticular degeneration (HLD). Methods Twenty four patients with HLD and 27 healthy volunteers were enrolled in this study. The left and right globus pallidus were selected as ROI, the whole brain functional connectivity was calculated. The brain functional connectivity between patients with HLD and healthy volunteers was compared. Results Compared with healthy volunteers, the functional connections of patients with HLD between the left globus pallidus and the brain stem and bilateral thalamus were decreased, while the functional connections between the right globus pallidus and several brain regions were decreased, including bilateral thalamus, bilateral middle frontal gyrus (MFG), bilateral insula, left anterior cingulate gyrus (ACC), left middle cingulate gyrus (MCC), brainstem and left cerebellum. Conclusion The results show that the abnormal connectivity of globus pallidus is widely distributed in HLD group, including cerebellar thalamic cortical circuit, motor, emotional and cognitive networks such as salience network and Communist Youth Network, which may be an important factor leading to clinical symptoms of HLD patients.
Key words： Hepatolenticular Degeneration, Magnetic Resonance Imaging, Basal Ganglia, Globus Pallidus
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