Functional Connectivity, Brain Development, and Outcomes in Chiari Type I Malformation

项目总结 项目2：功能连接、脑发育和Chiari I型畸形的结局 摘要 Chiari畸形(CM)是一种具有挑战性的疾病。尽管它在解剖学上定义明确的病理 以小脑扁桃体通过枕大孔突出为特征，其临床症状和 手术干预的适应症是高度可变的。在这里，我们建议使用高级神经成像技术 两种手术(后颅窝减压术前/术后)纵向获得的个性化连接 和非手术的CM患者，以确定更好的疾病特征和结果的成像标志物 预测。我们使用静息状态功能磁共振成像(rs-fmri)和扩散张量成像(Dti)来测量。 静息状态功能连接性(RSFC)和分数各向异性(FA)。我们正在利用 青少年脑认知发展(ABCD)研究方案(n=11874)，以促进直接 与来自ABCD数据集的年龄和人口统计学匹配的对照组受试者进行比较，包括成像和 认知/情感指标。此外，我们正在收集Chiari严重程度指数(CSI)和Chiari健康指数 儿科(芯片)数据，这两个数据都是由我们的团队专门为CM开发和验证的。这个 该项目的纵向臂包括一个成人CM队列，这将扩大以下项目的参与者数量 更大的力量，并允许我们在儿科队列中测试发育的CM效应。想象与认知 在进行任何干预之前，将在基线上对这些措施进行比较。那么参与者内部的比较将是 在后颅窝减压术(PFD)手术前后进行以确定新的预测成像标志物 疾病结局和对手术的反应。

PROJECT SUMMARY Project 2: Functional Connectivity, Brain Development, and Outcomes in Chiari Type I Malformation Abstract Chiari malformation (CM) is a challenging disease. Despite its anatomically well-defined pathology characterized by herniation of the cerebellar tonsils through the foramen magnum, its clinical symptoms and indications for surgical intervention are highly variable. Here we propose advanced neuroimaging with personalized connectomics acquired longitudinally, in both surgical (pre/post posterior fossa decompression) and non-surgical CM patients, to identify imaging markers for better disease characterization and outcome prediction. We are using resting state functional MRI (rs-fMRI) and diffusion tensor imaging (DTI) to measure resting state functional connectivity (RSFC) and fractional anisotropy (FA), respectively. We are leveraging the protocols of the Adolescent Brain Cognitive Development (ABCD) Study (n = 11,874), to facilitate direct comparisons to age and demographic matched control subjects from the ABCD data set, for both imaging and cognitive/emotional metrics. In addition, we are collecting Chiari Severity Index (CSI) and Chiari Health Index in Pediatrics (CHIP) data, both of which have been developed and validated by our team specifically for CM. The longitudinal arm of this project includes an adult CM cohort, which will expand the number of participants for greater power, and allow us to test developmental CM effects in the pediatric cohort. The imaging and cognitive measures will be compared at baseline before any intervention. Then within-participant comparisons will be made before and after posterior fossa decompression (PFD) surgery to identify novel imaging markers predictive of disease outcomes and response to surgery.