Department of Radio-diagnosis, Faculty of human medicine, Zagazig University, Zagazig, Egypt.
Department of Radio-diagnosis, Faculty of human medicine, Ain Shams University, Cairo, Egypt.
Eur J Radiol. 2019 Feb;111:41-46. doi: 10.1016/j.ejrad.2018.12.013. Epub 2018 Dec 14.
Although brain magnetic resonance spectroscopy (MRS) imaging findings in adult Wilson disease (WD) have been explained in extensive details, a paucity of information currently exists regarding brain MRS imaging findings in pediatric WD. The purpose of this study was to clarify the role of brain MRS in detecting early metabolite abnormalities in children with WD.
A case-controlled prospective study included 26 children with WD and 26 healthy controls. All children were subjected to examination on a 1.5 T MRI scanner. The spectra of N-acetyl aspartate (NAA), choline (Cho), and creatine (Cr), as well as the metabolite ratios of NAA/Cho, NAA/Cr, and Cho/Cr, were measured and compared between two groups.
Eight patients revealed increased signal intensity in the basal ganglia at T1-weighted images. When compared with healthy controls, WD patients showed a significant decrease (p < 0.05) in NAA (63.8 ± 9.6 vs 97.6 ± 3.8), Cho (46.7 ± 8.9 vs 87.3 ± 4.7), Cr (44 ± 10.1 vs 81.9 ± 4.05), NAA/Cho (1.92 ± 1.2 vs 3.34 ± 0.55), NAA/Cr (1.29 ± 0.7 vs 2.46 ± 0.34), and Cho/Cr (0.78 ± 0.4 vs 2 ± 0.13). Patients complicated with liver cell failure showed a significant decrease in all previous parameters (p < 0.05) than patients without complications. Patients with mixed neurological and hepatic diseases showed a severe reduction in NAA, NAA/Cr, and NAA/Cho compared with patients with hepatic disease only.
MRS in pediatric WD detects early neurological changes even with normal MRI.
尽管成人威尔逊病（WD）的脑磁共振波谱（MRS）成像结果已得到广泛详细的解释，但目前关于儿科 WD 脑 MRS 成像结果的信息很少。本研究旨在阐明脑 MRS 在检测 WD 儿童早期代谢物异常中的作用。
一项病例对照前瞻性研究纳入了 26 例 WD 患儿和 26 例健康对照者。所有患儿均在 1.5T MRI 扫描仪上进行检查。测量并比较两组的 N-乙酰天冬氨酸（NAA）、胆碱（Cho）和肌酸（Cr）谱，以及 NAA/Cho、NAA/Cr 和 Cho/Cr 的代谢物比值。
8 例患者在 T1 加权图像上显示基底节区信号强度增加。与健康对照组相比，WD 患儿的 NAA（63.8 ± 9.6 比 97.6 ± 3.8）、Cho（46.7 ± 8.9 比 87.3 ± 4.7）、Cr（44 ± 10.1 比 81.9 ± 4.05）、NAA/Cho（1.92 ± 1.2 比 3.34 ± 0.55）、NAA/Cr（1.29 ± 0.7 比 2.46 ± 0.34）和 Cho/Cr（0.78 ± 0.4 比 2.0 ± 0.13）明显降低（p＜0.05）。伴有肝细胞衰竭的患者所有上述参数均显著降低（p＜0.05），而无并发症患者则无明显变化。与仅有肝病史的患者相比，合并神经和肝疾病的患者的 NAA、NAA/Cr 和 NAA/Cho 明显减少。
即使 MRI 正常，MRS 也能在儿科 WD 中检测到早期神经变化。
