Gray matter atrophy patterns within the cerebellum-neostriatum-cortical network in SCA3

Gray matter atrophy patterns within the cerebellum-neostriatum-cortical network in SCA3
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SCA3 中小脑-新纹状体-皮质网络内的灰质萎缩模式

DOI:
10.1212/wnl.0000000000010986
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发表时间:
2020-12-01
期刊:
影响因子:
9.9
通讯作者:
Chen, Huafu
Chen, Huafu
中科院分区:
医学1区
文献类型:
--
作者:
Guo, Jing;Chen, Hui;Chen, Huafu

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目的探讨脊髓小脑性共济失调3型(SCA 3)患者脑灰质萎缩的空间分布特点及可能的发生顺序。方法47例SCA 3患者和49例年龄、性别匹配的健康对照者参加研究。对所有参与者进行高分辨率T1加权MRI检查。我们使用结构协方差因果网络(CasCN)来识别灰质萎缩模式的序列。这是通过将格兰杰因果关系分析应用于从网络水平通过基于体素的形态测量进行的灰质萎缩分期方案来实现的。结果表现前期患者表现为小脑蚓部局灶性灰质萎缩。随着病程的延长,小脑、新纹状体、额叶和顶叶的灰质萎缩逐渐加重。SCA 3患者还表现出从小脑蚓部到主要位于小脑-新纹状体-皮质网络的区域的近端和远端皮质萎缩序列。结论SCA 3的灰质萎缩具有不同的解剖学特点,并扩展到更多的区域,主要分布在小脑-新纹状体-皮质网络中。这些发现推进了我们对SCA 3结构损伤自然史的理解,同时证实了已知的临床特征。这可以为针对特定网络的区域性脑萎缩的有序顺序过程提供独特的见解。
Objective To investigate the spatial patterns and the probable sequences of gray matter atrophy in spinocerebellar ataxia type 3 (SCA3). Methods A total of 47 patients with SCA3 and 49 age- and sex-matched healthy controls participated in the study. High-resolution T1-weighted MRI were examined in all participants. We used the causal network of structural covariance (CasCN) to identify the sequence of gray matter atrophy patterns. This was achieved by applying Granger causality analysis to a gray matter atrophy staging scheme performed by voxel-based morphometry from the network level. Results Participants in the premanifest stage of the disease showed the presence of focal gray matter atrophy in the vermis. As the disease duration increased, there was progressive gray matter atrophy in the cerebellar, neostriatum, frontal lobe, and parietal lobe. The patients with SCA3 also showed proximal and distal cortical atrophy sequences exerting from the vermis to the regions mainly located in the cerebellum-neostriatum-cortical network. Conclusion Our results, although preliminary in nature, indicate that the gray matter atrophy in SCA3 lies and extends to involve more regions according to distinct anatomical patterns, mainly in the cerebellum-neostriatum-cortical network. These findings advance our understanding on the natural history of structural damage in SCA3, while confirming known clinical features. This could provide unique insight into the ordered sequential process of regional brain atrophy that targets a particular network.