Y. Ihara et al., FREE-RADICALS, LIPID PEROXIDES AND ANTIOXIDANTS IN BLOOD OF PATIENTS WITH MYOTONIC-DYSTROPHY, Journal of neurology, 242(3), 1995, pp. 119-122
We studied the levels of free radicals, lipid peroxides and antioxidan
ts, 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 resona
nce was used to assess the free radicals by the spin-trapping method u
sing 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 patient
s were significantly higher than those in the seven controls. The SOD
activities in red blood cells and serum from the six MyD patients show
ed no significant difference from those in the seven controls. The ser
um lipid peroxide concentration was increased in five of the MyD patie
nts and tended to increase further as the disease progressed. The seru
m vitamin E level was low in two patients and in the low normal range
in three. Serum coenzyme Q(10) was decreased in four patients. The ser
um selenium level was decreased in two patients and that of serum albu
min was decreased in three. Therefore we conclude that increased level
s of free radicals and lipid peroxides and decreased antioxidant level
s play an important role in the pathogenesis of MyD.