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Muscle insulin resistance in myotonic dystrophy

Effect of supraphysiologic insulinization

Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY.

In myotonic dystrophy, there is resistance of forearm muscle to physiologic concentrations of insulin. To determine whether this resistance is due to a shift in the dose-response curve resulting from decreased numbers of insulin receptors, we measured the effects of supraphysiologic insulinization (200 µU per kilogram per minute) on the uptake of glucose by forearm muscle in three patients with myotonic dystrophy and five denervated patients with comparable wasting. Although insulin levels were higher in patients with myotonic dystrophy than in controls, peak glucose uptake remained significantly lower in myotonic dystrophy patients. The findings are consistent with a postinsulin receptor defect of muscle and with altered membrane function in myotonic dystrophy.

Address correspondence and reprint requests to Dr. Moxley, Department of Neurology, University of Rochester School of Medicine and Dentistry, 601 Elmwood Avenue, P. O. Box 673, Rochester, NY 14642.

Accepted for publication December 17, 1979.

This research was supported by research grants from the Muscular Dystrophy Association, Inc., the National ALS Foundation, the Waasdorp Foundation, U. S. Public Health Service Grant No. RR 00044 from the Division of Research Resources of the National Institutes of Health, and by a grant from the National Institute of Arthritis, Metabolism, and Digestive Diseases, No. AM 22048.