Ishikawa Maho, Sawaya Reika, Hirayama Miki, Ueda Junpei, Saito Shigeyoshi
Department of Medical Physics and Engineering, Area of Medical Imaging Technology and Science, Division of Health Sciences, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan.
Department of Medical Technology, Division of Radiology, Osaka University Hospital, Osaka 565-0871, Japan.
Diagnostics (Basel). 2022 Aug 2;12(8):1864. doi: 10.3390/diagnostics12081864.
Pelizaeus−Merzbacher disease (PMD) is an X-linked recessive disorder of the central nervous system. We performed 7 Tesla magnetic resonance imaging of the brain in Tama rats, a rodent PMD model, and control rats, as well as evaluated the diagnostic values. In the white matter of the Tama rats, the T2 values were prolonged, which is similar to that observed in patients with PMD (60.7 ± 1.8 ms vs. 51.6 ± 1.3 ms, p < 0.0001). The apparent diffusion coefficient values in the white matter of the Tama rats were higher than those of the control rats (0.68 ± 0.03 × 10−3 mm2/s vs. 0.64 ± 0.03 × 10−3 mm2/s, p < 0.05). In proton magnetic resonance spectroscopy, the N-acetylaspartate (6.97 ± 0.12 mM vs. 5.98 ± 0.25 mM, p < 0.01) and N-acetylaspartate + N-acetylaspartylglutamate values of the Tama rats were higher (8.22 ± 0.17 mM vs. 7.14 ± 0.35 mM, p < 0.01) than those of the control rats. The glycerophosphocholine + phosphocholine values of the Tama rats were lower than those of the control rats (1.04 ± 0.09 mM vs. 1.45 ± 0.04 mM, p < 0.001). By using Luxol fast blue staining, we confirmed dysmyelination in the Tama rats. These results are similar to those of patients with PMD and other PMD animal models.
佩利措伊斯-梅茨巴赫病（PMD）是一种X连锁隐性中枢神经系统疾病。我们对啮齿类PMD模型塔玛大鼠和对照大鼠进行了7特斯拉脑部磁共振成像，并评估了其诊断价值。在塔玛大鼠的白质中，T2值延长，这与PMD患者中观察到的情况相似（60.7±1.8毫秒对51.6±1.3毫秒，p<0.0001）。塔玛大鼠白质中的表观扩散系数值高于对照大鼠（0.68±0.03×10−3平方毫米/秒对0.64±0.03×10−3平方毫米/秒，p<0.05）。在质子磁共振波谱分析中，塔玛大鼠的N-乙酰天门冬氨酸（6.97±0.12毫摩尔对5.98±0.25毫摩尔，p<0.01）和N-乙酰天门冬氨酸+N-乙酰天门冬氨酰谷氨酸值更高（8.22±0.17毫摩尔对7.14±0.35毫摩尔，p<0.01）。塔玛大鼠的甘油磷酸胆碱+磷酸胆碱值低于对照大鼠（1.04±0.09毫摩尔对1.45±0.04毫摩尔，p<0.001）。通过使用Luxol固蓝染色，我们证实了塔玛大鼠存在髓鞘形成异常。这些结果与PMD患者和其他PMD动物模型的结果相似。
