Progressive gray matter changes in patients with congenital central hypoventilation syndrome.

Progressive gray matter changes in patients with congenital central hypoventilation syndrome.
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DOI:
10.1038/pr.2012.25
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发表时间:
2012-06
期刊:
影响因子:
3.6
通讯作者:
Harper, Ronald M.
Harper, Ronald M.
中科院分区:
医学3区
文献类型:
--
作者:
Kumar, Rajesh;Woo, Marlyn S.;Macey, Paul M.;Woo, Mary A.;Harper, Ronald M.

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先天性中枢性低通气综合征(CCHS)患者在控制化学感觉、自主神经、运动、认知和情绪功能的区域出现脑损伤,这些功能在这种情况下是缺陷的。这些异常特征中有许多是在新生儿期出现的;然而,目前尚不清楚这些特征背后的组织损伤是否会随着时间的推移而逐渐恶化。我们假设CCHS受试者的几个大脑区域会随着时间的推移表现出灰质体积丢失的增加。我们使用3.0Tesla磁共振扫描仪收集了7例CCH(首次研究年龄,16.1±2.7岁,4名男性)和3名对照组(15.9±2.1岁,3名男性)的两次高分辨率T1加权图像(相隔4年),并用基于体素的形态计量学方法评估了区域灰质体积的变化。CCHS的多个脑部位,包括额叶、前额叶、岛叶和扣带回皮质,尾状核和壳核,腹侧颞叶和顶叶皮质,以及小脑皮质,随着时间的推移,灰质体积显著减少。只有有限的大脑区域,包括感觉、颞叶和髓质区域,在晚年出现灰质增加。CCHS患者的自主神经、呼吸和认知调节区域的灰质体积随着年龄的增长而减少,这一结果可能导致随着年龄的增长而出现的综合征功能的恶化。
Congenital central hypoventilation syndrome (CCHS) patients show brain injury in areas that control chemosensory, autonomic, motor, cognitive, and emotion functions, which are deficient in the condition. Many of these abnormal characteristics are present from the neonatal period; however, it is unclear if tissue injury underlying the characteristics progressively worsens with time. We hypothesized that several brain areas in CCHS subjects would show increased gray matter volume loss over time. We collected high-resolution T1-weighted images twice (four years apart) from 7 CCHS (age at first study, 16.1±2.7 years; 4 males) and 3 control subjects (15.9±2.1 years; 3 males) using a 3.0-Tesla MRI scanner, and evaluated regional gray matter volume changes with voxel-based-morphometry procedures. Multiple brain sites in CCHS, including frontal, prefrontal, insular and cingulate cortices, caudate nuclei and putamen, ventral temporal and parietal cortices, and cerebellar cortices showed significantly reduced gray matter volume over time. Only limited brain areas, including sensory, temporal, and medullary regions emerged with increased gray matter at the later age. CCHS patients show reduced gray matter volume with age progression in autonomic, respiratory, and cognitive regulatory areas, an outcome that may contribute to deterioration of functions found in the syndrome with increasing age.
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