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

项目摘要 项目2：功能连接，大脑发育，和基亚里I型畸形的结果 摘要 基亚里畸形（CM）是一种具有挑战性的疾病。尽管它的解剖学定义明确的病理 其特征在于小脑扁桃体通过枕骨大孔疝出，其临床症状和 手术干预的适应症是高度可变的。在这里，我们提出了先进的神经成像， 在两种手术（后颅窝减压术前/后）中纵向采集个性化连接切除术 和非手术CM患者，以确定成像标记物，以更好地描述疾病特征和结局 预测.我们使用静息态功能性MRI（rs-fMRI）和弥散张量成像（DTI）来测量 静息态功能连接性（RSFC）和分数各向异性（FA）。我们正在利用 青少年大脑认知发展（ABCD）研究（n = 11，874）的协议，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受试者进行比较， 认知/情感指标。此外，我们正在收集基亚里严重程度指数（CSI）和基亚里健康指数， 儿科（CHIP）数据，这两种数据都是由我们的团队专门为CM开发和验证的。的 该项目的纵向臂包括一个成年CM队列，这将扩大参与者的数量， 更大的功率，并允许我们测试儿童队列中的发育CM效应。成像和认知 在任何干预之前，将在基线处比较测量值。然后，参与者内部比较将 在后颅窝减压（PFD）手术前后进行，以确定新的影像学标记物预测 疾病的结果和对手术的反应。

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.