Department of Neurobiology, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA 90095, USA.
Neuroscience. 2009 Nov 10;163(4):1373-9. doi: 10.1016/j.neuroscience.2009.07.038. Epub 2009 Jul 24.
Congenital central hypoventilation syndrome (CCHS) children show cognitive and affective deficits, in addition to state-specific loss of respiratory drive. The caudate nuclei serve motor, cognitive, and affective roles, and show structural deficits in CCHS patients, based on gross voxel-based analytic procedures. However, the magnitude and regional sites of caudate injury in CCHS are unclear. We assessed global caudate nuclei volumes with manual volumetric procedures, and regional volume differences with three-dimensional surface morphometry in 14 CCHS (mean age+/-SD: 15.1+/-2.3 years; 8 male) and 31 control children (15.1+/-2.4 years; 17 male) using brain magnetic resonance imaging (MRI). Two high-resolution T1-weighted image series were collected using a 3.0 Tesla MRI scanner; images were averaged and reoriented (rigid-body transformation) to common space. Both left and right caudate nuclei were outlined in the reoriented images, and global volumes calculated; surface models were derived from manually-outlined caudate structures. Global caudate nuclei volume differences between groups were evaluated using a multivariate analysis of covariance (covariates: age, gender, and total intracranial volume). Both left and right caudate nuclei volumes were significantly reduced in CCHS over control subjects (left, 4293.45+/-549.05 vs. 4626.87+/-593.41 mm(3), P<0.006; right, 4376.29+/-565.42 vs. 4747.81+/-578.13 mm(3), P<0.004). Regional deficits in CCHS caudate volume appeared bilaterally, in the rostral head, ventrolateral mid, and caudal body. Damaged caudate nuclei may contribute to CCHS neuropsychological and motor deficits; hypoxic processes, or maldevelopment in the condition may underlie the injury.
先天性中枢通气不足综合征（CCHS）患儿除了出现呼吸驱动的特定状态丧失外，还表现出认知和情感障碍。尾状核在运动、认知和情感方面发挥作用，并且根据基于体素的分析程序，在 CCHS 患者中显示出结构缺陷。然而，CCHS 患者尾状核损伤的程度和区域部位尚不清楚。我们使用手动体积测量程序评估了整体尾状核体积，并使用三维表面形态计量学对 14 名 CCHS（平均年龄+/-标准差：15.1+/-2.3 岁；8 名男性）和 31 名对照组儿童（15.1+/-2.4 岁；17 名男性）的区域体积差异进行了评估，使用脑磁共振成像（MRI）。使用 3.0T MRI 扫描仪采集了两组高分辨率 T1 加权图像序列；图像进行平均处理并重新定向（刚体变换）到共同空间。在重新定向的图像中勾勒出左右尾状核，并计算出总体积；从手动勾勒的尾状核结构中得出表面模型。使用协方差的多变量分析评估了组间尾状核核体积差异（协变量：年龄、性别和总颅内体积）。CCHS 患者的左、右尾状核体积均明显小于对照组（左侧，4293.45+/-549.05 与 4626.87+/-593.41mm³，P<0.006；右侧，4376.29+/-565.42 与 4747.81+/-578.13mm³，P<0.004）。CCHS 尾状核体积的区域性缺陷在双侧、头部前、腹外侧中部和体部均有出现。受损的尾状核可能导致 CCHS 神经心理学和运动缺陷；缺氧过程或该病症的发育不良可能是损伤的基础。
