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  • Immune-mediated rippling mu...
    Bettini, Mariela; Gonorazky, Hernan; Chaves, Marcelo; Fulgenzi, Ernesto; Figueredo, Alejandra; Christiansen, Silvia; Cristiano, Edgardo; Bertini, Enrico S.; Rugiero, Marcelo

    Journal of neuroimmunology, 10/2016, Letnik: 299
    Journal Article

    Cases of acquired rippling muscle disease in association with myasthenia gravis have been reported. We present three patients with iRMD (immune-mediated rippling muscle disease) and AChR-antibody positive myasthenia gravis. None of them had thymus pathology. They presented exercise-induced muscle rippling combined with generalized myasthenia gravis. One of them had muscle biopsy showing a myopathic pattern and a patchy immunostaining with caveolin antibodies. They were successfully treated steroids and azathioprine. The immune nature of this association is supported by the response to immunotherapies and the positivity of AChR-antibodies. Schematic representation of Caveolin-3 (CAV3) and its relationship with other muscle proteins. Display omitted •Rippling muscle is a self-propagating stiffness induced by percussion.•Immunogenic rippling muscle associated to myasthenia gravis was described.•This uncommon association might be underrecognized between myasthenic patients.•The good response to immunotherapy supports their immunologic origin.