文章摘要
Wilson病患者的丘脑核体积与神经功能损害程度的相关性分析
The relationship between patients with Wilson's disease and neurological manifestations
投稿时间:2023-01-20  
DOI:10.3969/j.issn.1000-0399.2023.06.003
中文关键词: Wilson 病  丘脑核  神经功能损害程度
英文关键词: Wilson's disease  Thalamic nucleus  Neurological impairment
基金项目:国家自然科学基金项目(编号:81472535),安徽中医药大学重大项目(编号:2021sfylc01)
作者单位E-mail
张兵 230012 安徽 合肥 安徽中医药大学研究生院  
胡文彬 230012 安徽 合肥 安徽中医药大学研究生院
230001 安徽 合肥 安徽中医药大学神经病学研究所附属医院神经内科二病区 
hbwzhx@163.com 
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中文摘要:
      目的 探讨Wilson病(WD)的丘脑核体积,以及其与神经功能损害程度的关系。方法 选取2022年1月至9月安徽中医药大学神经病学研究所附属医院住院的61例WD患者(WDs)纳入WDs组。同期,在本院中招募33例健康对照(HCs)纳入HCs组。通过影像学处理软件Freesurfer对两组对象的结构像进行丘脑核的分割处理并获得相应的体积。采用统一肝豆状核变性评定量表中的神经功能评分(UWDRS-N)从言语、静止性震颤、面部表情、肌僵直、步态以及姿势等运动症状评估患者的神经功能的损伤程度。采用独立样本t检验或者Mann-Whitney U检验比较两组对象的丘脑核体积,采用多元线性回归模型分析WDs组的丘脑核体积与UWDRS-N得分的关系。P值通过错误发现率(FDR)校正,且<0.05。结果 WDs组的双侧腹后外侧核(VPL)、腹内侧核(VM)、腹外侧核后部(VLp)、腹外侧核前部(VLa)、腹前核大细胞部(VAmc)、腹前核(VA)、界核(L-Sg)、下侧枕(PuM)、内侧枕(PuI)、前枕(PuA)、带旁核(Pt)、束旁核(Pf)、中央旁核(Pc)、腹内侧重聚区(MV (Re))、内侧大细胞部(MDm)、内侧小细胞部(MDl)、外侧膝状体(LGN)、中央外侧核(CM)、中央内侧核(CeM)、丘脑前腹侧核(AV)、后侧核(LP)、背外侧核(LD)、中央外侧核(CL)以及右侧外侧枕(PuL)的体积较HCs组均缩小,且差异有统计学意义。右侧MDm (β=-4.29,FDR-P<0.001)的体积与UWDRS-N得分呈负相关。结论 WDs的丘脑核萎缩涉及到丘脑前核、以及丘脑外侧、腹侧、板内、内侧和后侧的多个核群。其中,MDm与神经功能损害程度关系密切,可能在WDs运动症状的发生和发展中扮演重要角色。
英文摘要:
      Objective This study aims to investigate the volume atrophy of thalamic nuclei in Wilson's Disease (WD) and its relationship with the degree of neurological impairment. Methods A total of 61 WD patients (WDs) who were admitted to the Affiliated Hospital of the Institute of Neurology, Anhui University of Chinese Medicine from January to September 2022, were included in the WDs group. During the same period, 33 healthy controls (HCs) were recruited from the same hospital and included in the HCs group. The structural images of the two groups were processed using the imaging software Freesurfer to segment the thalamic nuclei and obtain the corresponding volumes. The degree of neurological impairment in WD patients was assessed using the Unified Wilson's Disease Rating Scale -Neurological subscore (UWDRSN), which includes assessments of speech, resting tremor, facial expression, muscle rigidity, gait, and posture. The volumes of thalamic nuclei were compared between two groups using independent samples t-test or Mann-Whitney U test. The relationship between thalamic nucleus volume and UWDRS-N score in the WDs group was analyzed using a multivariate linear regression model. Results The volumes of bilateral ventral posterolateral nucleus (VPL), ventral posteromedial nucleus (VM), ventrolateral nucleus posterior part (VLp), ventrolateral nucleus anterior part (VLa), ventral anterior nucleus magnocellular part (VAmc), ventral anterior nucleus (VA), intergeniculate leaflet (L-Sg), lateroposterior nucleus (PuM), lateroinferior nucleus (PuI), anterior nucleus (PuA), parataenial nucleus (Pt), parafascicular nucleus (Pf), centromedian nucleus (Pc), medial ventral nucleus reuniens (MV(Re)), medial dorsal nucleus magnocellular part (MDm), medial dorsal nucleus parvocellular part (MDl), lateral geniculate nucleus (LGN), centrolateral nucleus (CM), centromedial nucleus (CeM), anteroventral nucleus of the thalamus (AV), lateral posterior nucleus (LP), lateral dorsal nucleus (LD), centrolateral nucleus (CL), as well as the volume of right lateral posterior nucleus (PuL) were significantly reduced in the WDs group compared to the HCs group (FDR-P <0.05). The volume of right MDm (β = -4.29, FDR-P<0.001) was negatively correlated with UWDRS-N scores. Conclusion Thalamic nuclei atrophy in WD involves several nuclei groups, including the anterior thalamic nuclei and various medial, lateral, ventral, intralaminar, and posterior nuclei. Among these, the MDm nucleus is closely related to the degree of neurological impairment and may play an important role in the occurrence and development of motor symptoms in WDs.
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