Dysfunction of the Lenticular Nucleus Is Associated with Dystonia in Wilson's Disease.
Dysfunction of the Lenticular Nucleus Is Associated with Dystonia in Wilson's Disease.
复制标题
胸部核的功能障碍与威尔逊氏病中的肌张力障碍有关。
DOI：
10.3390/brainsci13010007
复制
发表时间：
2022-12-20
期刊：
影响因子：
3.3
通讯作者：
Ding, Yufeng
中科院分区：
文献类型：
--
作者：
Yang, Yulong;Wei, Taohua;Yang, Wenming;Hu, Sheng;Jiang, Hailin;Dong, Wei;Hao, Wenjie;Yang, Yue;Qian, Nannan;Ding, Yufeng
关键词：
豆状核功能障碍被认为与肝豆状核病(WD)的神经系统症状有关。然而，关于豆状核是否以及如何通过与大脑皮层和小脑相互作用来影响肌张力障碍，我们知之甚少。为了解决这一问题，我们招募了37例WD患者(男性20例，年龄23.95±6.95岁，年龄范围12-37岁)和37例年龄和性别匹配的健康对照组(HCS)(25例男性，年龄25.19±1.88岁，年龄20-30岁)，每个受试者都进行了静息状态功能磁共振成像(RS-fMRI)扫描。采用肌肉生物力学参数和统一的威尔逊病评分量表(UWDRS)分别评价肌张力障碍程度和临床表现。根据背侧、腹侧、前侧和后侧的定位，将豆状核壳核和苍白球分为12个亚区，并计算每个亚区基于种子的功能连接性(FC)。通过相关分析进一步探讨豆状核内Fc变化与肌张力水平及临床表现的关系。通过将所有WD肌肉生物力学参数与健康对照组(HCS)进行比较来诊断肌张力障碍。与HCS相比，WD患者除右侧腹后部至中扣带回(MCC)外，壳核各亚区FC均降低，除左侧小脑腹前部外，其余各亚区FC均降低。WD患者左侧苍白球FC减少，主要分布于MCC和小脑，右侧苍白球至小脑FC减少。从壳核到MCC的FC与精神症状显著相关。从壳核到小脑的FC与肌张力和神经症状显著相关。此外，从苍白球到小脑的FC也与肌肉张力有关。总之，这些发现强调了豆状核-小脑环路可能作为肌张力障碍的神经生物标志物，并为WD肌张力障碍的神经机制提供了启示。
Dysfunction of the lenticular nucleus is thought to contribute to neurological symptoms in Wilson’s disease (WD). However, very little is known about whether and how the lenticular nucleus influences dystonia by interacting with the cerebral cortex and cerebellum. To solve this problem, we recruited 37 WD patients (20 men; age, 23.95 ± 6.95 years; age range, 12–37 years) and 37 age- and sex-matched healthy controls (HCs) (25 men; age, 25.19 ± 1.88 years; age range, 20–30 years), and each subject underwent resting-state functional magnetic resonance imaging (RS-fMRI) scans. The muscle biomechanical parameters and Unified Wilson Disease Rating Scale (UWDRS) were used to evaluate the level of dystonia and clinical representations, respectively. The lenticular nucleus, including the putamen and globus pallidus, was divided into 12 subregions according to dorsal, ventral, anterior and posterior localization and seed-based functional connectivity (FC) was calculated for each subregion. The relationships between FC changes in the lenticular nucleus with muscle tension levels and clinical representations were further investigated by correlation analysis. Dystonia was diagnosed by comparing all WD muscle biomechanical parameters with healthy controls (HCs). Compared with HCs, FC decreased from all subregions in the putamen except the right ventral posterior part to the middle cingulate cortex (MCC) and decreased FC of all subregions in the putamen except the left ventral anterior part to the cerebellum was observed in patients with WD. Patients with WD also showed decreased FC of the left globus pallidus primarily distributed in the MCC and cerebellum and illustrated decreased FC from the right globus pallidus to the cerebellum. FC from the putamen to the MCC was significantly correlated with psychiatric symptoms. FC from the putamen to the cerebellum was significantly correlated with muscle tension and neurological symptoms. Additionally, the FC from the globus pallidus to the cerebellum was also associated with muscle tension. Together, these findings highlight that lenticular nucleus–cerebellum circuits may serve as neural biomarkers of dystonia and provide implications for the neural mechanisms underlying dystonia in WD.
登录
查看更多内容
影响因子：
2.7
作者：
Ikemoto, Satoshi;Yang, Chen;Tan, Aaron
通讯作者：
Tan, Aaron
DOI：
10.1016/s1474-4422(14)70190-5
发表时间：
2015-01
期刊：
The Lancet. Neurology
影响因子：
--
作者：
Bandmann O;Weiss KH;Kaler SG
通讯作者：
Kaler SG
DOI：
10.12659/msm.909550
发表时间：
2018-10-23
期刊：
Medical science monitor : international medical journal of experimental and clinical research
影响因子：
--
作者：
Huang J;Qin K;Tang C;Zhu Y;Klein CS;Zhang Z;Liu C
通讯作者：
Liu C
影响因子：
8.6
作者：
Albanese, Alberto;Bhatia, Kailash;Bressman, Susan B.;DeLong, Mahlon R.;Fahn, Stanley;Fung, Victor S. C.;Hallett, Mark;Jankovic, Joseph;Jinnah, Hyder A.;Klein, Christine;Lang, Anthony E.;Mink, Jonathan W.;Teller, Jan K.
通讯作者：
Teller, Jan K.
影响因子：
2.9
作者：
Hu S;Xu C;Dong T;Wu H;Wang Y;Wang A;Kan H;Li C
通讯作者：
Li C