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随机侧面硬化症基因型中的渐进性thalamo-cortical断路:thalamus-relayed circuits的结构退化和网络功能障碍

Marlene Tahedl¹, Jana Kleinerova¹, Mark A Doherty²

¹Computational Neuroimaging Group (CNG), School of Medicine, Trinity College Dublin, Dublin, Ireland.

European journal of neurology

|May 10, 2025

概括

肌缩性侧面硬化症 (ALS) 涉及状皮质电路的广泛退化,特别是影响额头区域和前中心. 这种网络范围内的损伤突出显示了ALS是一种渐进的网络疾病.

科学领域:

神经科学是一个神经科学.
神经学神经学
医疗成像医学成像

背景情况:

丘陵体是认知,感官和运动功能的关键皮质下枢纽.
肌缩性侧面硬化症 (ALS) 的thalamic病理越来越被认可,但通常是孤立地研究.
需要一个网络智慧的视角来评估膜退化及其在ALS的皮质投影.

研究的目的:

为了研究ALS.在thalamo-cortical电路的结构和功能完整性.
评估ALS患者的纵向体积变化.
根据遗传分层 (C9orf72) 探索基于乳头皮层网络退化的差异.

主要方法:

对基因分层的ALS患者进行前性成像研究.
通过神经成像来评估thalamic投影的白质完整性.
灰色物质和功能连接的纵向体积分析.

主要成果:

在ALS的基线上,多个皮层区域的乳头突出的白质完整性受到损害,特别是在C9orf72载体中.
纵向观察到 thalamus 和额头区域 (DLPFC,MFG) 之间的逐渐断裂.
脑前中心环和小脑中的灰质体积减少,特别是在C9orf72患者中.

结论:

ALS的特点是广泛而渐进的甲状腺皮层连接性改变.
甲状腺突起的退化支持ALS作为一个网络疾病的观点.
这些发现与相互连接的神经结构一起退化的原则保持一致.

关键词:

骨髓缩侧面硬化症 (ALS) 是一种磁共振成像技术的使用运动神经元疾病运动神经元疾病神经成像是一种神经成像.

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