Kruse B, Barker P B, van Zijl P C, Duyn J H, Moonen C T, Moser H W
Kennedy Krieger Institute, Baltimore, MD 21205.
Ann Neurol. 1994 Oct;36(4):595-608. doi: 10.1002/ana.410360408.
Multislice proton magnetic resonance spectroscopic imaging permits metabolic analysis of brain tissue in vivo by data acquisition in four oblique axial slices, each 15-mm thick and divided into 0.8-ml single-volume elements. We applied this technique to the systematic study of 25 patients with adrenoleukodystrophy: 3 with the severe childhood or adult cerebral form of the disease, 5 with adrenomyeloneuropathy, 12 with no demonstrable neurological involvement, and 5 women heterozygous for adrenoleukodystrophy who had some degree of neurological disability. Abnormalities on magnetic resonance spectroscopic imaging included a reduction in N-acetyl aspartate, an increase in choline-containing compounds, and at times, an increase in lactate. Five patients showed abnormalities in the presence of normal-appearing magnetic resonance images, and in 8 other patients the alterations on spectroscopic images were more severe than those demonstrable by magnetic resonance imaging. Correlation with clinical course suggests that an increase in the choline-containing compounds is associated with an active demyelinative process, whereas such compounds are not elevated in lesions that are stable. We conclude that magnetic resonance spectroscopic imaging is a more sensitive indicator of early neurological involvement than is magnetic resonance imaging, and that the character of abnormalities detected by the former technique may serve as a gauge of the degree of activity of the demyelinating process and as a guide to the selection and evaluation of therapeutic approaches.
多层质子磁共振波谱成像技术可通过采集四个斜轴位层面的脑组织数据，对活体脑组织进行代谢分析。每个层面厚15毫米，划分为0.8毫升的单一体积单元。我们运用该技术对25例肾上腺脑白质营养不良患者进行了系统研究：其中3例为严重的儿童期或成年期脑型疾病患者，5例为肾上腺脊髓神经病患者，12例无明显神经受累表现，5例为肾上腺脑白质营养不良杂合子女性，有一定程度的神经功能障碍。磁共振波谱成像异常表现包括N - 乙酰天门冬氨酸减少、含胆碱化合物增加，有时还有乳酸增加。5例患者在磁共振图像表现正常的情况下出现波谱成像异常，另有8例患者波谱图像的改变比磁共振成像显示的更为严重。与临床病程的相关性表明，含胆碱化合物增加与活跃的脱髓鞘过程相关，而在稳定的病变中这些化合物并未升高。我们得出结论，磁共振波谱成像比磁共振成像更能敏感地显示早期神经受累情况，而且前一种技术检测到的异常特征可作为脱髓鞘过程活动程度的指标，并指导治疗方法的选择和评估。
