Neurology, 10623Stanford Hospital and Clinics, Palo Alto, CA, USA.
Neonatal and Developmental Medicine, 10624Stanford University School of Medicine, Stanford, CA, USA.
J Child Neurol. 2021 Sep;36(10):894-900. doi: 10.1177/08830738211008736. Epub 2021 May 28.
Children with neurofibromatosis type 1 (NF1) often report cognitive challenges, though the etiology of such remains an area of active investigation. With the advent of treatments that may affect white matter microstructure, understanding the effects of age on white matter aberrancies in NF1 becomes crucial in determining the timing of such therapeutic interventions. A cross-sectional study was performed with diffusion tensor imaging from 18 NF1 children and 26 age-matched controls. Fractional anisotropy was determined by region of interest analyses for both groups over the corpus callosum, cingulate, and bilateral frontal and temporal white matter regions. Two-way analyses of variance were done with both ages combined and age-stratified into early childhood, middle childhood, and adolescence. Significant differences in fractional anisotropy between NF1 and controls were seen in the corpus callosum and frontal white matter regions when ages were combined. When stratified by age, we found that this difference was largely driven by the early childhood (1-5.9 years) and middle childhood (6-11.9 years) age groups, whereas no significant differences were appreciable in the adolescence age group (12-18 years). This study demonstrates age-related effects on white matter microstructure disorganization in NF1, suggesting that the appropriate timing of therapeutic intervention may be in early childhood.
患有神经纤维瘤病 1 型（NF1）的儿童经常报告认知挑战，尽管这种病因仍在积极研究中。随着可能影响白质微观结构的治疗方法的出现，了解 NF1 中年龄对白质异常的影响对于确定此类治疗干预的时机至关重要。对 18 名 NF1 儿童和 26 名年龄匹配的对照者进行了一项横断面研究，采用弥散张量成像。对两组的胼胝体、扣带回和双侧额颞叶白质区域进行了感兴趣区分析，以确定各向异性分数。对年龄和年龄分层（幼儿期[1-5.9 岁]、儿童中期[6-11.9 岁]和青春期[12-18 岁]）进行了双向方差分析。当年龄合并时，NF1 与对照组在胼胝体和额叶白质区域的各向异性分数存在显著差异。按年龄分层，我们发现这种差异主要是由幼儿期（1-5.9 岁）和儿童中期（6-11.9 岁）年龄组驱动的，而在青春期年龄组没有明显差异。本研究表明 NF1 中白质微观结构紊乱存在与年龄相关的影响，提示治疗干预的适当时机可能在幼儿期。
