Rajanayagam V, Balthazor M, Shapiro E G, Krivit W, Lockman L, Stillman A E
Department of Radiology, University of Minnesota, Minneapolis 55455, USA.
AJNR Am J Neuroradiol. 1997 Nov-Dec;18(10):1909-14.
To determine early signs of disease in patients with childhood-onset cerebral adrenoleukodystrophy (COCALD) with the use of proton MR spectroscopy.
Eleven children with posterior COCALD involvement and three children with anterior COCALD involvement were studied with single-voxel proton MR spectroscopy and neuropsychological testing. Findings were compared with those in five healthy control subjects.
Areas of abnormal T2 signal intensity in children with COCALD showed abnormal metabolite ratios relative to those of control subjects as follows: decreased N-acetylaspartate (NAA)/Creatine (Cr) and NAA/Choline (Ch) and increased Ch/Cr. Metabolite ratios from normal-appearing brain regions in the same patients also were abnormal, with reduced NAA/Cr and NAA/Ch and increased Ch/Cr values. The mean metabolite ratios in normal-appearing regions were between those in the abnormal regions and those found in the control subjects. Statistical comparison of these ratios with neuropsychological test scores, which are specific for anterior and posterior brain functions, showed a significant correlation with the abnormal metabolite ratios. Our results indicate that the normal-appearing brain regions in these patients are metabolically abnormal.
Proton MR spectroscopy could be a useful noninvasive tool to evaluate extent of disease in patients with COCALD.
利用质子磁共振波谱法确定儿童期起病的脑肾上腺脑白质营养不良（COCALD）患者的疾病早期迹象。
对11例后部受累的COCALD患儿和3例前部受累的COCALD患儿进行了单体素质子磁共振波谱检查和神经心理学测试。将结果与5名健康对照者的结果进行比较。
COCALD患儿T2信号强度异常区域的代谢物比率相对于对照者异常，如下所示：N-乙酰天门冬氨酸（NAA）/肌酸（Cr）和NAA/胆碱（Ch）降低，Ch/Cr升高。同一患者正常脑区的代谢物比率也异常，NAA/Cr和NAA/Ch降低，Ch/Cr值升高。正常脑区的平均代谢物比率介于异常脑区和对照者之间。将这些比率与针对前后脑功能的神经心理学测试分数进行统计学比较，结果显示与异常代谢物比率存在显著相关性。我们的结果表明，这些患者正常脑区在代谢上是异常的。
质子磁共振波谱法可能是评估COCALD患者疾病程度的一种有用的非侵入性工具。
