Institute of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Hannover, Germany.
J Magn Reson Imaging. 2012 Apr;35(4):926-32. doi: 10.1002/jmri.23517. Epub 2011 Nov 29.
To find imaging signs of active degenerative processes in vanishing white matter disease (VWM), six VWM patients and six matched controls underwent MR examinations. The data were analyzed with modified Scheltens scales for morphological findings and determined quantitatively for apparent diffusion coefficient (ADC). Single-voxel MR spectra were acquired at the parietal white matter and analyzed with LCModel. Typical VWM brain lesions were found in all patients accompanied by proton diffusion abnormalities: Increased ADC appeared in brain regions with severe myelin destruction in all patients, and reduced ADC in two of six younger patients in remaining white matter adjacent to the lesions or at the borders around the lesions, who had a short history of the disease (≤ 1 year). The MR spectroscopy revealed reductions of NAA, Cho, and Cr, which correlate to the grade of white matter abnormalities. An increase of myo-inositol as marker of reactive gliosis was missing. Thus, restricted proton diffusion was evident in younger VWM patients with short history of disease, which in combination with lack of reactive gliosis may reflect early white matter degeneration in VWM. The multimodal MR methods are useful for characterizing such tissue degeneration in brain in vivo.
为了在进行性白质减少症（VWM）中寻找活跃退化过程的影像学征象，6 名 VWM 患者和 6 名匹配的对照者接受了磁共振检查。使用改良的 Scheltens 量表对形态学发现进行了数据分析，并对表观扩散系数（ADC）进行了定量测定。在顶叶白质采集单体素磁共振波谱，并使用 LCModel 进行分析。所有患者均发现典型的 VWM 脑病变，并伴有质子扩散异常：所有患者的严重脱髓鞘区域的 ADC 增加，6 名年轻患者中有 2 名在病变相邻的残留白质或病变周围边界处的 ADC 降低，他们的疾病史较短（≤1 年）。磁共振波谱显示 NAA、Cho 和 Cr 减少，与白质异常程度相关。作为反应性神经胶质增生标志物的肌醇增加缺失。因此，在疾病史较短的年轻 VWM 患者中，质子扩散受限明显，这与缺乏反应性神经胶质增生可能反映了 VWM 中的早期白质退化。多模态磁共振方法可用于在体内对脑内这种组织退化进行特征描述。
