胡曼诗,赵静,初建平,吴超,黄子桓,丘海珊.脊髓小脑共济失调3型患者脑白质及灰质微结构改变[J].中国医学影像技术,2023,39(10):1566~1570
脊髓小脑共济失调3型患者脑白质及灰质微结构改变
Microstructure changes of brain white and gray matter in patients with spinocerebellar ataxia type 3
投稿时间:2023-06-20  修订日期:2023-08-20
DOI:10.13929/j.issn.1003-3289.2023.10.027
中文关键词:  脊髓小脑共济失调  灰质  白质  弥散磁共振成像
英文关键词:spinocerebellar ataxias  gray matter  white matter  diffusion magnetic resonance imaging
基金项目:广东省基础与应用基础研究基金项目(2021A1515012279)、广州市科技计划项目(202201011244)。
作者单位E-mail
胡曼诗 中山大学附属第一医院放射科, 广东 广州 510080  
赵静 中山大学附属第一医院放射科, 广东 广州 510080  
初建平 中山大学附属第一医院放射科, 广东 广州 510080  
吴超 中山大学附属第一医院神经科, 广东 广州 510080  
黄子桓 中山大学附属第一医院放射科, 广东 广州 510080  
丘海珊 中山大学附属第一医院放射科, 广东 广州 510080 qiuhsh3@mail.sysu.edu.cn 
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中文摘要:
      目的 观察脊髓小脑共济失调3型(SCA3)患者脑白质及灰质微结构改变。方法 前瞻性招募37例SCA3患者(症状组)、15例SCA3患者直系亲属(症状前组)及35名健康志愿者(对照组),采集全脑3D T1WI及弥散峰度成像(DKI);对白质及灰质结构进行组间两两比较,针对存在差异脑区提取相关指标,分析脑白质及灰质结构改变与共济失调等级量表(SARA)的相关性。结果 症状前组纤维束受损主要见于双侧小脑上、中、下脚及大脑脚等处,其平均弥散峰度(MK)、径向弥散峰度(Kr)、轴向弥散峰度(Ka)及部分各向异性(FA)均低于对照组(P均<0.02);症状组受损纤维束广泛存在于双侧小脑上、中、下脚、大脑脚、内囊后肢、胼胝体、丘脑后辐射(包括视辐射)及上放射冠,其MK、Kr、Ka及FA均低于症状前组及对照组(P均<0.02)。相比对照组,症状组双侧小脑半球(前叶、后叶)、脑桥、延髓、双侧壳核及豆状核灰质体积(GMV)显著减少、双侧丘脑GMV显著增加(P均<0.02);相比症状前组,症状组脑桥GMV显著减低(P<0.02)。症状前组与对照组全脑GMV差异均无统计学意义(P均>0.02)。症状组及症状前组双侧小脑上、中、下脚及大脑脚部分弥散值与患者SARA评分相关(P均<0.05)。结论 SCA3患者脑白质及灰质微结构发生改变,且白质受损更显著并早于临床出现症状。
英文摘要:
      Objective To observe microstructure changes of brain white and gray matter in patients with spinocerebellar ataxia type 3 (SCA3). Methods Thirty-seven SCA3 patients (symptom group), 15 direct relatives of them (pre-symptom group) and 35 healthy volunteers (control group) were prospectively recruited. Brain 3D T1WI and diffusion kurtosis imaging (DKI) were collected. Structures of brain white and gray matter were compared between each 2 groups, and relative indicators of brain areas being significant different between groups were extracted to explore the correlations of brain microstructure changes with results of scale assessment and rating of ataxia (SARA). Results In pre-symptom group, damaged fiber bundles mainly distributed in bilateral superior, middle, inferior cerebellar peduncles and cerebral peduncles, with decreased mean kurtosis (MK), radial kurtosis (Kr), axial kurtosis (Ka) and fractional anisotropy (FA) compared with those in control group (all P<0.02). In symptom group, extensive damaged fiber bundles were found in bilateral superior, middle, inferior cerebellar peduncles, cerebral peduncles, posterior limbs of the internal capsule, corpus callosum, posterior thalamic radiation (including visual radiation) and superior corona radiata, with decreased MK, Kr, Ka and FA of damaged fiber bundles compared with those in pre-symptom group and control group (all P<0.02). Compared with control group, the gray matter volume (GMV) of bilateral cerebellar hemispheres (anterior and posterior lobes), pons, medulla oblongata, putamen and lenticular nuclei in symptom group was significantly reduced, while that of bilateral thalamus was significantly increased (all P<0.02). Compared with that in pre-symptom group, pontine GMV significantly reduced in symptom group (P<0.02). No significant difference of whole brain GMV was found between pre-symptom group and control group (all P>0.02). The partial diffusion values of bilateral cerebellar superior, middle, inferior and cerebral peduncles in patients in symptom group and pre-symptom group were correlated with SARA scores (all P<0.05). Conclusion Microstructure changes of brain white and gray matter existed in SCA3 patients, and more significant damages of white matter occurred earlier than onset of clinical symptoms.
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