Hashimoto T, Tayama M, Yoshimoto T, Miyazaki M, Harada M, Miyoshi H, Tanouchi M, Kuroda Y
Department of Pediatrics, University of Tokushima School of Medicine.
No To Hattatsu. 1995 May;27(3):177-83.
Volume selective proton MR spectroscopy (1H-MRS) of the brain was performed on a 1.5T magnet in five patients with congenital muscular dystrophy (CMyD), 46 healthy children, and one healthy adult. Peaks of N-acetylaspartate (NAA), choline (Cho) and creatine (Cr) were observed in both patients and control groups on 1H-MRS, but lactate was not observed. 1H-MRS revealed an increase in the ratio of NAA/Cho and NAA/Cr, and a decrease in Cho/Cr ratio with age in control subjects. In patients with CMyD, the values of NAA/Cho ratio did not increase with age. The values of NAA/Cr ratio decreased with age. The values of Cho/Cr ratio decreased with age in both patient and control groups. At age more than 4 years, the values of NAA/Cho and NAA/Cr ratios were lower in patients with CMyD than in controls. The values of Cho/Cr ratio did not differ between CMyD and controls. These results suggest that in patients with CMyD, there is an NAA decrease and a developmental disorder of neurons in the brain.
对5例先天性肌营养不良（CMyD）患者、46名健康儿童和1名健康成人在1.5T磁体上进行了脑部容积选择性质子磁共振波谱（1H-MRS）检查。在1H-MRS上，患者组和对照组均观察到N-乙酰天门冬氨酸（NAA）、胆碱（Cho）和肌酸（Cr）的波峰，但未观察到乳酸峰。1H-MRS显示，对照组中NAA/Cho和NAA/Cr比值随年龄增加，Cho/Cr比值随年龄降低。在CMyD患者中，NAA/Cho比值不随年龄增加。NAA/Cr比值随年龄降低。患者组和对照组的Cho/Cr比值均随年龄降低。在4岁以上时，CMyD患者的NAA/Cho和NAA/Cr比值低于对照组。CMyD患者和对照组的Cho/Cr比值无差异。这些结果表明，CMyD患者存在脑内NAA减少和神经元发育障碍。
