Ihara Y, Mori A, Hayabara T, Namba R, Nobukuni K, Sato K, Miyata S, Edamatsu R, Liu J, Kawai M
Clinical Research Institute, National Minamiokayama Hospital, Okayama, Japan.
J Neurol. 1995 Feb;242(3):119-22. doi: 10.1007/BF00936882.
We studied the levels of free radicals, lipid peroxides and antioxidants, as well as superoxide dismutase (SOD) activity in the blood of six patients with myotonic dystrophy (MyD) (mean age 52.8, SD 5.0 years) and seven controls (mean age 48.8, SD 6.3 years). Electron spin resonance was used to assess the free radicals by the spin-trapping method using 5,5-dimethyl-1-pyrroline-1-oxide. The levels of C centre radical (P < 0.05) and H radical (P < 0.05) in blood from the six MyD patients were significantly higher than those in the seven controls. The SOD activities in red blood cells and serum from the six MyD patients showed no significant difference from those in the seven controls. The serum lipid peroxide concentration was increased in five of the MyD patients and tended to increase further as the disease progressed. The serum vitamin E level was low in two patients and in the low normal range in three. Serum coenzyme Q10 was decreased in four patients. The serum selenium level was decreased in two patients and that of serum albumin was decreased in three. Therefore we conclude that increased levels of free radicals and lipid peroxides and decreased antioxidant levels play an important role in the pathogenesis of MyD.
我们研究了6例强直性肌营养不良（MyD）患者（平均年龄52.8岁，标准差5.0岁）和7名对照者（平均年龄48.8岁，标准差6.3岁）血液中的自由基、脂质过氧化物和抗氧化剂水平，以及超氧化物歧化酶（SOD）活性。采用电子自旋共振，通过自旋捕获法，使用5,5-二甲基-1-吡咯啉-1-氧化物评估自由基。6例MyD患者血液中的C中心自由基水平（P<0.05）和H自由基水平（P<0.05）显著高于7名对照者。6例MyD患者红细胞和血清中的SOD活性与7名对照者相比无显著差异。5例MyD患者的血清脂质过氧化物浓度升高，且随着疾病进展有进一步升高的趋势。2例患者血清维生素E水平较低，3例处于低正常范围。4例患者血清辅酶Q10降低。2例患者血清硒水平降低，3例患者血清白蛋白水平降低。因此，我们得出结论，自由基和脂质过氧化物水平升高以及抗氧化剂水平降低在MyD的发病机制中起重要作用。
