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结节性硬化症患儿白质通路中扩散特性的纵向变化

Longitudinal changes in diffusion properties in white matter pathways of children with tuberous sclerosis complex.

作者信息

Baumer Fiona M, Song Jae W, Mitchell Paul D, Pienaar Rudolph, Sahin Mustafa, Grant P Ellen, Takahashi Emi

机构信息

Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

Division of Newborn Medicine, Department of Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.

出版信息

Pediatr Neurol. 2015 Jun;52(6):615-23. doi: 10.1016/j.pediatrneurol.2015.02.004. Epub 2015 Feb 16.

Abstract

BACKGROUND

Abnormal white matter development in patients with tuberous sclerosis complex, a multisystem hamartomatous disorder caused by aberrant neural proliferation and axonal maturation, may be associated with poorer neurocognitive outcomes. The purpose of this study is to identify predictors of longitudinal changes in diffusion properties of white matter tracts in patients with tuberous sclerosis complex.

METHODS

Diffusion magnetic resonance imaging was carried out in 17 subjects with tuberous sclerosis complex (mean age, 7.2 ± 4.4 years) with at least two magnetic resonance imaging scans (mean number of days between scans, 419.4 ± 105.4). There were 10 males; 5 of 17 had autism spectrum disorder and 10 of 17 had epilepsy. Regions of interest were placed to delineate the internal capsule/corona radiata, cingulum, and corpus callosum. The outcomes were mean change in apparent diffusion coefficient and fractional anisotropy. Data were analyzed using Pearson's correlation and multiple linear regression analyses.

RESULTS

Gender was a significant predictor of mean change in apparent diffusion coefficient in the left internal capsule, right and left cingulum bundles, and corpus callosum and a significant predictor of mean change in fractional anisotropy in the corpus callosum. Epilepsy was a significant predictor of mean change in apparent diffusion coefficient in the left internal capsule. Autism spectrum disorder was not predictive of diffusion changes in any of the studied pathways.

CONCLUSION

Clinical variables, including gender and epilepsy, have an effect on the development of white matter pathways. These variables should be taken into consideration when counseling tuberous sclerosis complex patients and in future imaging studies in this population.

摘要

背景

结节性硬化症是一种由异常神经增殖和轴突成熟引起的多系统错构瘤病,患者脑白质发育异常可能与较差的神经认知结果相关。本研究的目的是确定结节性硬化症患者脑白质束扩散特性纵向变化的预测因素。

方法

对17例结节性硬化症患者(平均年龄7.2±4.4岁)进行了扩散磁共振成像检查,这些患者至少接受了两次磁共振成像扫描(扫描间隔天数的平均值为419.4±105.4)。其中男性10例;17例中有5例患有自闭症谱系障碍,17例中有10例患有癫痫。放置感兴趣区域以描绘内囊/放射冠、扣带束和胼胝体。观察指标为表观扩散系数和分数各向异性的平均变化。采用Pearson相关性分析和多元线性回归分析对数据进行分析。

结果

性别是左侧内囊、右侧和左侧扣带束以及胼胝体表观扩散系数平均变化的显著预测因素,也是胼胝体分数各向异性平均变化的显著预测因素。癫痫是左侧内囊表观扩散系数平均变化的显著预测因素。自闭症谱系障碍对任何研究通路的扩散变化均无预测作用。

结论

包括性别和癫痫在内的临床变量会影响脑白质通路的发育。在为结节性硬化症患者提供咨询以及今后对该人群进行影像学研究时,应考虑这些变量。

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