EFFECT OF EXOGENOUS ARGININE VASOPRESSIN ON ADRENOCORTICOTROPIN AND CORTISOL RELEASE IN MYOTONIC-DYSTROPHY PATIENTS - DELAYED-RESPONSES OF NORMAL MAGNITUDE

We administered intramuscular arginine vasopressin (AVP) to ten normal controls and eight myotonic dystrophy patients. By measuring plasma AVP levels in five of the myotonics and all the normals, we showed that absorption and distribution of AVP was not delayed or significantly reduced in myotonics. The magnitude of the mean plasma adrenocorticotropin (ACTH) response to AVP in the myotonics was not different from that of normals, although it was significantly delayed (mean peak time, 37.5 +/- 4.9 versus 17.0 +/- 3.2 min). We propose that this delay was caused by a significantly reduced ACTH secretion rate in myotonics, because the maximum rate of detection of ACTH in plasma is reduced in myotonics (0.6 +/- 0.2 versus 1.7 +/- 0.5 pmol/L/min), whose corticotropes, while having the same capacity to respond to the AVP stimulus, are slower to attain that capacity. The mean integrated cortisol response (AUC) was significantly smaller for myotonics (8072 +/- 2017 versus 13049 +/- 1630 nmol.min/L). This may be due to the slower rate of ACTH delivery to the adrenal in myotonics. The timing of the adrenal response does not appear to be impaired in myotonic dystrophy, with the cortisol peak following the ACTH peak by approximately 15 min in both groups. The normal magnitude ACTH response to AVP in myotonics is in contrast to that seen to ACTH secretagogues acting via corticotropin-releasing hormone-initiated pathways, where a rapid hypersecretion is seen. We propose a mechanism of defective calcium transport to account for these observations.