Department of Neurobiology, David Geffen School of Medicine at UCLA, University of California at Los Angeles, Los Angeles, CA 90095-1763, USA.
Neurosci Lett. 2009 Dec 25;467(2):139-43. doi: 10.1016/j.neulet.2009.10.024. Epub 2009 Oct 12.
Congenital central hypoventilation syndrome (CCHS) patients show hypoventilation during sleep and severe autonomic impairments, including aberrant cardiovascular regulation. Abnormal sympathetic patterns, together with increased and variable CO(2) levels, lead to the potential for sustained cerebral vasculature changes. We performed high-resolution T1-weighted imaging in 13 CCHS and 31 control subjects using a 3.0-T magnetic resonance imaging scanner, and evaluated resting basilar and bilateral middle cerebral artery cross-sections. Two T1-weighted image series were acquired; images were averaged and reoriented to common space, and regions containing basilar and both middle cerebral arteries were oversampled. Cross-sections of the basilar and middle cerebral arteries were manually outlined to calculate cross-sectional areas, and differences between and within groups were evaluated. Basilar arteries in CCHS were significantly dilated over control subjects, but both middle cerebral artery cross-sections were similar between groups. No significant differences appeared between left and right middle cerebral arteries within either group. Basilar artery dilation may result from differential sensitivity to high CO(2) over other vascular beds, damage to serotonergic or other chemosensitive cells accompanying the artery, or enhanced microvascular resistance, and that dilation may impair tissue perfusion, leading to further neural injury in CCHS.
先天性中枢性肺泡换气不足症（CCHS）患者在睡眠期间表现出换气不足和严重的自主神经损伤，包括异常的心血管调节。异常的交感神经模式，加上增加和变化的二氧化碳水平，导致持续的脑血管变化的潜在风险。我们使用 3.0-T 磁共振成像扫描仪对 13 名 CCHS 患者和 31 名对照受试者进行了高分辨率 T1 加权成像，并评估了静息基底动脉和双侧大脑中动脉的横截面。采集了两个 T1 加权图像序列；对图像进行平均处理并重新定向到公共空间，并对包含基底动脉和双侧大脑中动脉的区域进行过采样。手动勾勒基底动脉和大脑中动脉的横截面以计算横截面积，并评估组间和组内的差异。CCHS 患者的基底动脉明显比对照组扩张，但两组之间的大脑中动脉横截面相似。两组内的左侧和右侧大脑中动脉之间没有出现显著差异。基底动脉扩张可能是由于对高二氧化碳的敏感性不同于其他血管床，伴随着动脉的 5-羟色胺能或其他化学敏感性细胞的损伤，或增强的微血管阻力，并且扩张可能会损害组织灌注，导致 CCHS 中的进一步神经损伤。
