Myopathy, Distal, with Rimmed Vacuoles, also known as multisystem proteinopathy 4, is related to nonaka myopathy and miyoshi muscular dystrophy. An important gene associated with Myopathy, Distal, with Rimmed Vacuoles is SQSTM1 (Sequestosome 1). Affiliated tissues include skeletal muscle, and related phenotypes are emg: myopathic abnormalities and babinski sign