EXCESSIVE PLASMA K+ INCREASE AFTER ISCHEMIC EXERCISE IN MYOTONIC MUSCULAR-DYSTROPHY

Changes in plasma electrolyte levels upon ischemic forearm exercise were studied in myotonic muscular dystrophy (MyD) patients, disease control groups, and healthy volunteers. Significant differences were observed in the pH and the concentrations of creatine kinase and Na+ before exercise between healthy volunteers and MyD patients. In comparison with healthy volunteers a lower pH and higher concentrations of both CK and Na+ were found in MyD patients. The concentrations of K+, inorganic phosphate, lactate, and ammonia increase upon exercise in all groups. The mean increase in plasma K+ for healthy volunteers amounted to 0.8 mM (=23%). In MyD patients a significantly higher increase in plasma K+ was found [mean 2.2 mM (= 65%)]. No abnormal release of K+ from muscular tissue was found in the disease control groups. Data on the postexercise increase in the concentration of other muscular constituents such as creatine kinase, inorganic phosphate, or creatine exclude the possibility of a generally increased membrane permeability in MyD. The abnormally high increase of plasma K+ upon muscular exercise seems to be specific for MyD and may be related to the biochemical defect in this disease. Copyright © 1990 John Wiley & Sons, Inc.