Rippling muscle disease

Six patients from two families with an autosomal dominantly inherited disease, apparently a myopathy, are described. Their major complaint was muscle stiffness, primarily in the legs. The muscles displayed an unusual sensitivity to stretch, manifested by rippling waves of muscle contraction. These rippling contractions were not accompanied by muscle fiber action potentials. Nonspecific, mild abnormalities were seen on muscle biopsy. These findings raise the possibility that there is an intracellular derangement in the muscle fiber responsible for the muscle rippling; further studies are necessary to establish the underlying pathophysiologic condition.