Rippling muscle disease is a rare autosomal dominant disorder that may occur sporadically. In this report two patients presenting with rippling muscles followed by myasthenia gravis are described. Our first patient developed rippling muscles about 1 month after infection with Yersinia enterocolitica. Two years later myasthenia gravis appeared. Our second patient had a 2-year history of asthma prior to the onset of rippling muscles which preceded the myasthenic symptoms by 4-8 weeks. Acetylcholine receptor and anti-skeletal muscle antibody titers were positive in both patients. In both patients the rippling phenomena worsened with pyridostigmine treatment but markedly improved after immunosuppression with azathioprine.
[Show abstract][Hide abstract] ABSTRACT: Rippling muscle disease (RMD) is a benign myopathy with symptoms and signs of muscular hyperexcitability. The typical finding is electrically silent muscle contractions provoked by mechanical stimuli and stretch. After the first description in 1975, there have been several publications on this disorder. Although RMD most often is reported with autosomal dominant inheritance, some sporadic cases are found, and an association with other diseases such as myasthenia gravis has also been reported. The pathophysiological mechanism is still not clarified. Abnormalities in calcium homeostasis in the sarcoplasmic reticulum have been proposed as the most probable causes. However, recent genetic studies make a primary channelopathy unlikely. In this article, a review of this curious disease is presented.
[Show abstract][Hide abstract] ABSTRACT: A 46-year-old woman presented with rippling muscle phenomenon. She complained of uncomfortable muscular stiffness of extremities and abdominal wall. Muscle contraction was easily elicited by percussion, which was visible from the surface and propagated in a rolling manner. The mounding (or myoedema) phenomenon was also remarkable. Three years later, malignant lymphoma (histologically, lymphoplasmacytoid lymphoma) was found in the sacrum. The lymphoma subsided with treatment by vincristine, cyclophosphamide, doxorubicin and prednisolone. Serum IgG as well as creatine kinase values were normalized. The rippling phenomenon also responded to the treatment. The present rippling muscle syndrome might be of a paraneoplastic or autoimmune origin related to lymphoma, although the evidence seemed indirect. We discussed the role of the internal membrane system of the skeletal muscle in the pathogenesis of rippling muscle.
Internal Medicine 03/2002; 41(2):147-50. DOI:10.2169/internalmedicine.41.147 · 0.90 Impact Factor
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