NEURAL ACTIVITY IN CONGENITAL CENTRAL HYPOVENTILATION

描述(改编自申请者的描述)：目标是 确定患有脑部疾病的患者的大脑结构缺陷 先天性中枢低通气综合征(CCHS)，一种以 由于缺乏中枢化学感受器，睡眠时失去呼吸动力， 偶尔还会出现清醒时的低眼压。大脑区域将通过 正常人和CCHS患者的功能性磁共振检查 患者在基线条件下和轻度低氧挑战期间， 高碳酸血症和一过性血压升高。血氧水平 相关(粗体)功能磁共振图像将从 基线期间15名CCHS患者和15名年龄匹配的对照组，100% 02%，5%二氧化碳，在95%02%和15%02%呼吸机挑战中，以及在Valsalva期间 升压动作，同时呼吸、心率和变异性， 无创血压，交感神经流出指数(出汗) 都是经过测量的。基线条件下的脑部图像将从 在实验挑战期间收集的图像。激活的程度 以及在挑战和训练过程中激活的脑区的变化 将比较CCHS患者和对照组的恢复情况。时间的选择 不同大脑部位的激活将与生理相关 伴随着挑战而来的变化。

DESCRIPTION (Adapted from applicant's description): The objective is to identify the brain structures that are deficient in patients afflicted with Congenital Central Hypoventilation Syndrome (CCHS), a disorder characterized by absence of central chemoreception, loss of breathing drive during sleep, and occasional waking hypotonia. Brain areas will be visualized with functional magnetic resonance procedures in normal subjects and CCHS patients during baseline conditions and during challenges of mild hypoxia, hypercapnia, and transient elevation of blood pressure. Blood oxygen level dependent (BOLD) functional magnetic resonance images will be collected from fifteen CCHS patients and 15 age-matched controls during baseline, and 100% 02, 5% CO2 in 95% 02, and 15% 02 ventilatory challenges, and during Valsalva pressor maneuvers, while respiration, heart rate and variability, non-invasive blood pressure, and an index of sympathetic outflow (sweating) are measured. Brain images from baseline conditions will be subtracted from images collected during experimental challenges. The extent of activation and time course of changes in activated brain regions during challenges and recovery will be compared in CCHS patients and controls. The timing of activation of different brain sites will be correlated with physiological changes accompanying the challenges.