Rippling Muscle Syndrome Preceding Malignant Lymphoma.

Abstract

A 46-year-old womanpresented 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)phenomenonwas also remarkable. Three years later, malignant lymphoma (histologically, lymphoplasmacytoid lymphoma) was found in the sacrum. The lymphomasubsided with treatment by vincristine, cyclophosphamide, doxorubicin and prednisolone.SerumIgG as well as creatine kinase values were normalized. The rippling phenomenonalso responded to the treatment. The present rippling muscle syndrome might be of a paraneoplastic or autoimmuneorigin related to lymphoma, although the evidence seemed indirect. Wediscussed the role of the internal membranesystem of the skeletal muscle in the pathogenesis of rippling muscle. (Internal Medicine 41: 147-150, 2002)

Caveolin-3 gene mutation in Japanese with rippling muscle disease

“…Rippling muscle disease, which was reported in patients from various European countries (1–10), is extremely rare in Japan. Only three sporadic cases and one family with similar clinical features to RMD have been reported (11–14). There have not been any reports describing a CAV3 mutation in Japanese patients with RMD.…”

Rippling Muscle Syndrome

“…The symptomof rippling muscles improved after the treatment of myasthenia gravis either by thymectomy or immunosuppression in all acquired RMDpatients. A new case of acquired RMD is reported by Takagi et al (9). This RMDpatient also had malignant lymphoma (lymphoplasmacytoid lymphoma) and improved after treatment with anti-cancer drugs.…”

“…The authors suggested that these after-contractions result from secondaryslow release of Ca2+from the sarcoplasmic reticulum as a result of abnormal sensitivity, perhaps of the Ca2+ channels, to mechanical distortion. Takagi et al (9) performed skinned fiber studies using biopsied muscle. They showed the spreading contraction in the skinned fiber preparation where the surface membrane had already been removed, but the T-tubule and SR remained intact.…”

Immune-mediated rippling muscle disease

“…2 In addition, immune-mediated acquired rippling muscle has been reported. [3][4][5][6][7][8] In some patients, RMD has been associated with antibodies against striated muscle antigens but also antibodies to neuronal ion channels, such as potassium channels. 5,6 We report RMD in a patient with autoantibodies recognizing antigens associated with the sarcolemma and triad and possibly contributing to the pathophysiology of acquired immune-mediated RMD.…”