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Kenneth Ricker, MD; Richard T. Moxley, MD; Reinhard Rohkamm, MD

Arch Neurol. 1989;46(4):405-408.

Abstract
• 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.

Author Affiliations
From the Department of Neurology, the University of Würzburg, West Germany (Drs Ricker and Rohkamm), and the Departments of Neurology and Pediatrics, University of Rochester (NY) School of Medicine and Dentistry (Dr Moxley).

Footnotes
Accepted for publication Sept 3, 1988.

Reprint requests to Neurologische Universitäts-Klinik, Josef-Schneider-Strasse 11, D-8700 Würzburg, West Germany (Dr Ricker).

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