Studies of Plasma Insulin in Myotonic Dystrophy

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Abstract
Plasma insulin response to oral glucose has been studied in 12 patients with myotonic dystrophy. In 7 of 12 patients the insulin response markedly exceeded the normal range. The markedly exaggerated insulin response was observed after arginine stimulation; tolbutamide infusion produced an abnormal but less exaggerated response than seen with glucose or arginine. The exaggerated insulin response occurred in young patients with very mild disease as well as in older patients with more severe muscle involvement. Excess body weight, severity of the muscle disease, glucocorticoids and growth hormone were excluded as a cause of the hyperinsulinemia. The patients exhibited nearly normal sensitivity to exogenous and endogenous insulin, and no abnormalities were detected in the form of plasma insulin in these patients. No correlation was found in the type of muslce fiber atrophy and insulin secretion. The data appear most compatible with an increased sensitivity of the β-cell to a variety of stimuli in myotonic dystrophy.

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