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Neuromuscular Research Laboratory and the Department of Neurology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota.
Ultrastructural studies have shown plasmalemmal defects in a proportion of non-necrotic muscle fibers in Duchenne dystrophy, suggesting that intracellular calcium overloading may be an important mechanism of muscle fiber degeneration. To investigate this assumption, we studied the localization of calcium with the von Kössa method, with alizarin red, and with glyoxalbis-(O-hydroxyanil) in serial, fresh-frozen sections of 114 biopsy specimens. The biopsy material included Duchenne dystrophy (24 cases), other dystrophies (27 cases), inflammatory myopathies (47 cases), and normal controls (11 cases). Counted in each specimen were every muscle fiber, the so-called large-dark fibers, and all calcium-positive fibers. Separate counts were made of the large-dark, necrotic, and other types of calcium-positive fibers. In Duchenne dystrophy, non-necrotic calcium-positive fibers occurred with a mean frequency of 4.83 percent. For all other groups, the corresponding value was 0.57 percent, with a range of 0.21 percent (normals) to 1.76 percent (scleroderma), p < 0.001. Large-dark fibers were 12 times more common in Duchenne dystrophy than in all other cases. Forty-three percent of the fibers were calcium-positive in Duchenne dystrophy, whereas calcium-positive large-dark fibers were extremely rare in the other cases.
This work was supported in part by Research Grant NS-6277 from the National Institutes of Health and by a Research Center Grant and a Clinical Fellowship Grant from the Muscular Dystrophy Association.
Presented at the twenty-ninth annual meeting of the American Academy of Neurology, Atlanta, April 28, 1977.
Reprint requests should be addressed to Dr. Engel, Department of Neurology, Mayo Clinic, Rochester, MN 55901.
Accepted for publication June 24, 1977.
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