Rajanayagam V, Grad J, Krivit W, Loes D J, Lockman L, Shapiro E, Balthazor M, Aeppli D, Stillman A E
Department of Radiology, University of Minnesota, Minneapolis 55455, USA.
AJNR Am J Neuroradiol. 1996 Jun-Jul;17(6):1013-24.
To determine the potential of proton MR spectroscopy to monitor patients with childhood-onset cerebral adrenoleukodystrophy (COCALD).
Single-voxel MR spectroscopy was performed in 16 children with COCALD (24 examinations) who had had no treatment and in 7 children (13 examinations) who had had bone marrow transplantation.
In the untreated children with clinically active COCALD, the metabolite ratios N-acetyl-aspartate (NAA)/creatine (Cr) and NAA/choline (Ch) were decreased while Ch/Cr was increased. This trend agrees well with those reported by other researchers, although different experimental sequences and parameters were used in our study. Comparison of these ratios with those from a control group yielded significant differences in the occipital region. In the children who were clinically stable after bone marrow transplantation, the mean levels of the three ratios were between those of the control subjects and the patients with untreated COCALD: the differences in these ratios approached significance. In patients who had been monitored periodically, MR spectroscopy metabolite ratios correlated well with the dementia rating score, reflecting clinical status.
There is good correlation between MR spectroscopy metabolite ratios and a patient's clinical status. MR spectroscopy appears to be a useful, noninvasive tool to monitor patients with adrenoleukodystrophy, and it increases the overall sensitivity of MR techniques in clinical applications.
确定质子磁共振波谱技术监测儿童期起病的脑肾上腺白质营养不良（COCALD）患者的潜力。
对16例未经治疗的COCALD患儿（24次检查）和7例接受过骨髓移植的患儿（13次检查）进行单体素质子磁共振波谱分析。
在未经治疗且临床症状活跃的COCALD患儿中，代谢物比率N-乙酰天门冬氨酸（NAA）/肌酸（Cr）和NAA/胆碱（Ch）降低，而Ch/Cr升高。尽管本研究使用了不同的实验序列和参数，但这一趋势与其他研究者报道的结果高度吻合。将这些比率与对照组的比率进行比较，发现枕叶区域存在显著差异。在骨髓移植后临床症状稳定的患儿中，这三个比率的平均水平介于对照组和未经治疗的COCALD患者之间：这些比率的差异接近显著水平。在接受定期监测的患者中，磁共振波谱代谢物比率与痴呆评分密切相关，反映了临床状态。
磁共振波谱代谢物比率与患者的临床状态之间存在良好的相关性。磁共振波谱似乎是监测肾上腺白质营养不良患者的一种有用的非侵入性工具，并且它提高了磁共振技术在临床应用中的整体敏感性。
