基于功能磁共振成像的肝豆状核变性患者纹状体亚区损害及临床分析

Functional magnetic resonance imaging study of striatal subregions in patients with Wilson disease

关键词:  肝豆状核变性  认知障碍  纹状体亚区  磁共振成像

Key words:  hepatolenticular degeneration  cognition disorders  striatum subregion  magnetic resonance imaging

摘要:

目的基于功能磁共振成像分析肝豆状核变性（Wilson disease, WD）患者纹状体亚区神经功能损害及其与临床相关分析，探讨其神经心理学症状可能的致病机制。方法收集WD患者29例和健康志愿者30例，进行3.0 T磁共振功能磁共振成像及临床量表测试，采用mICA工具箱的group ICA功能将壳核和尾状核分成8个亚区，做全脑功能连接，提取功能连接异常区域的值与临床量表测试结果做相关性分析。结果与对照组相比，WD患者左侧尾状核中部与全脑未发现功能增强或减弱区域，余感兴趣区与全脑功能连接不同程度减弱。左侧尾状核前部（ROI5）-中扣带回功能连接值与简易智能状态量表（MMSE）值呈显著负相关（r=-0.47，P=0.009）；右侧尾状核前部（ROI6）-中扣带回功能连接值与MMSE值呈显著负相关（r=-0.46，P=0.011）；右侧尾状核中部（ROI8）-右侧岛叶功能连接值与MMSE值呈显著负相关（r=-0.38，P=0.041）。结论右侧尾状核前中部与背外侧前额叶功能连接减弱，双侧壳核前部与丘脑功能连接减弱，可能破坏皮质-纹状体-丘脑回路引起认知功能损害；参与记忆及动作相关认知的突显网络、默认模式网络的通路连接受阻则可能是WD患者认知障碍的神经病理机制。

Abstract:

ObjectiveTo investigate neurological damage of striatal subregions in patients with Wilson disease(WD) using functional magnetic resonance imaging(fMRI). MethodsTwenty-nine patients with WD and 30 healthy controls were recruited in the study. All subjects underwent fMRI examination; the cognition of WD patients was assessed with Mini-Mental State Examination(MMSE）, Time-Based Prospective Memory(TBPM）and Event-Based Prospective Memory(EBPM）scales. The putamen and caudate nuclei were divided into 8 sub-regions with group ICA function of the mICA toolbox, and the whole brain function connection was analyzed. The values of the abnormal function connection areas were extracted and their correlation with the cognition assessment results was analyzed. ResultsCompared to the control group, the whole brain connectivity in WD group was significantly weaker, except in the middle of the left caudate nucleus. The connection values between ROI5 and cingulate gyrus function, between ROI6 and cingulate gyrus function, between ROI8 and right insular lobe were negatively correlated with the MMSE scores(r=-0.47, P=0.009; r=-0.46, P=0.011 and r=-0.38, P=0.041, respectively). ConclusionThe weakened connection between the anterior-middle part of the right caudate nucleus and the dorsolateral prefrontal lobe, the anterior part of the bilateral putamen and the thalamus may destruct the cortex-striatum-thalamic circuit, leading to cognitive impairment. Blocked pathway connections of the salient network and default mode network involved in memory and action-related cognition may be the pathological mechanism of cognitive impairment in patients with WD.