Abnormal Insulin Secretion in Myotonic Dystrophy

Abstract

MYOTONIC dystrophy is a hereditary disorder that involves multiple organ systems.^{1 , 2} The muscle disturbance is characterized by myotonia and a unique pattern of skeletal-muscle atrophy (head, anterior neck, trunk, diaphragm and distal limbs). The striking frequency of cataracts, frontal baldness and gonadal atrophy has suggested that this disease is related to some endocrine or metabolic disturbance.^{1 , 3 , 4} Numerous investigations of endocrine function in patients with myotonic dystrophy have failed to reveal any consistent abnormality of function of the pituitary, adrenal or thyroid gland.^{1 2 3 4 5 6} The basal metabolic rate is often markedly reduced, and testicular atrophy, with a histologic picture of tubular degeneration . . .