de Blank Peter, Berman Jeffrey I, Prelack Marisa, Sollee John R, Lane Adam, Waldman Amy T, Fisher Michael J
University of Cincinnati Medical Center Department of Pediatrics, Cincinnati, Ohio, USA.
Division of Oncology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Neurooncol Adv. 2020 Jun 25;2(Suppl 1):i150-i158. doi: 10.1093/noajnl/vdaa037. eCollection 2020 Jul.
Adults with neurofibromatosis type 1 (NF1) have decreased white matter integrity, but differences in children with NF1 have not been described. Defining normal values for diffusion tensor imaging (DTI) measures, especially in the optic radiations, is important to the development of DTI as a potential biomarker of visual acuity in children with optic pathway glioma. This study examines the effect of age and NF1 status on DTI measures in children.
In this retrospective study, MR imaging including DTI was conducted in 93 children (40 children with NF1 and 53 healthy controls) between 0 and 14 years of age. Regression models of age, sex, and NF1 status on DTI measures were evaluated, and tract-based spatial statistics (TBSS) compared DTI measures in age-matched NF1 to non-NF1 cohorts.
Fractional anisotropy, radial diffusivity, and mean diffusivity in white matter tracts of the optic radiations varied with age and were best modeled by a logarithmic function. Age-related DTI measure change was different in NF1 versus non-NF1 subjects. Normal values and 95% confidence intervals for age 0.5-12 years were derived for both groups. Differences in DTI measures between NF1 and non-NF1 groups at a range of ages were shown diffusely throughout the cerebral white matter using TBSS.
Children with NF1 demonstrate increased diffusion throughout the brain compared to children without NF1 suggesting a potentially altered developmental trajectory of optic radiation microstructure. Defining normal values for white matter integrity in children with NF1 may help target early intervention efforts in this vulnerable group.
1型神经纤维瘤病（NF1）成人患者的白质完整性降低，但NF1儿童患者的差异尚未见描述。确定扩散张量成像（DTI）测量的正常值，尤其是在视辐射中的正常值，对于DTI作为视神经通路胶质瘤儿童视力潜在生物标志物的发展至关重要。本研究探讨年龄和NF1状态对儿童DTI测量的影响。
在这项回顾性研究中，对93名0至14岁的儿童（40名NF1儿童和53名健康对照）进行了包括DTI在内的磁共振成像。评估了年龄、性别和NF1状态对DTI测量的回归模型，并采用基于纤维束的空间统计学（TBSS）比较了年龄匹配的NF1组和非NF1组的DTI测量值。
视辐射白质束中的各向异性分数、径向扩散率和平均扩散率随年龄变化，最佳拟合为对数函数。NF1组和非NF1组与年龄相关的DTI测量变化不同。得出了两组0.5至12岁的正常值和95%置信区间。使用TBSS显示，在一系列年龄中，NF1组和非NF1组之间的DTI测量差异广泛分布于整个脑白质。
与无NF1的儿童相比，NF1儿童的全脑扩散增加，提示视辐射微结构的发育轨迹可能改变。确定NF1儿童白质完整性的正常值可能有助于针对这一弱势群体开展早期干预工作。
