We present three women with hypokalemic myopathy in whom serum creatin
e phosphokinase (CPK) was bound to serum immunoglobulin (macro-CPK), I
n all three cases, CPK isozyme electrophoresis demonstrated an extra C
PK band between CPK-MM and CPK-MB, The bound immunoglobulins were iden
tified as IgA/A-kappa and lambda, IgA-lambda and IgA-kappa and lambda,
respectively, In all cases, histological examination of the biopsied
muscles revealed necrotic and/or regenerating fibers, In each patient,
potassium replacement therapy returned the macro-CPK as well as the m
arked elevations of serum muscle enzymes to normal, Taken together wit
h other recent reports, our findings suggested that CPK-immunoglobulin
binding may be related to the emergence of pathophysiology of hypokal
emic myopathy.