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Abnormal Cytokine and Adrenocortical Hormone Regulation in Myotonic Dystrophy¹

Departments of Medicine (Å.J., T.O.), Clinical Chemistry (Å.J.), and Clinical Genetics (K.Ce.), Umeå University Hospital, 901 85 Umeå; Department of Obstetrics and Gynecology and Clinical Research Center (K.Ca.), Karolinska Institute, Huddinge University Hospital, 141 86 Huddinge; Department of Medicine (B.A.), Malmö University Hospital, 205 02 Malmö; and Department of Internal Medicine (E.K., H.F.), Boden Hospital, 961 85 Boden, Sweden

Address correspondence and requests for reprints to: Åsa Johansson, Department of Clinical Chemistry, Umeå University Hospital, 901 85 Umeå, Sweden. E-mail: asa.johansson{at}medicin.umu.se

Metabolic-endocrine dysfunctions, including hyperinsulinemia, hypertriglyceridemia, increased fat mass, and dysregulation of the hypothalamic-pituitary-adrenal axis, are common in myotonic dystrophy (MD). We hypothesized that increased production of interleukin-6 (IL-6) and tumor necrosis factor- (TNF-) may be important underlying mechanisms.

We studied the diurnal rhythmicity of cytokines and cortisol, ACTH, and dehydroepiandrosterone in 18 men with adult onset MD and 18 controls. Morning levels of androstenedione, 17-hydroxyprogesterone, testosterone, and insulin were also determined. Genetic analyses were performed, including calculation of allele sizes.

Median circulating 24-h levels of IL-6 (P < 0.001), TNF- (P = 0.05), ACTH (P < 0.05), and cortisol (P < 0.05) were all significantly increased in MD, whereas dehydroepiandrosterone levels were decreased (P < 0.001). The diurnal rhythms of these cytokines/hormones were disturbed in patients. Morning testosterone levels were decreased and insulin levels increased (P < 0.01 for both). Patients with high body fat mass had significantly increased insulin levels and decreased morning levels of cortisol, ACTH, and testosterone.

IL-6 and TNF- levels are increased and adrenocortical hormone regulation is disturbed in MD. Adiposity may contribute to these disturbances, which may be of importance for decreased adrenal androgen hormone production and metabolic, muscular, and neuropsychiatric dysfunction in MD.

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