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Welcome. Myotubular Myopathy is a rare muscle condition which affects mostly boys at birth, and sometimes an even rarer form develops later in life affecting both boys and girls. It causes profound muscle weakness.
Characteristics of the Disease. Limb-girdle muscular dystrophy type 2B (LGMD2B) is a degenerative myopathy characterized by weakness and atrophy. Individuals with LGMD generally show weakness and wasting restricted to the limb musculature, proximal greater than distal.Proximal weakness refers to weakness of the muscles closer to the center of the body, including the shoulder, pelvic girdle, upper thighs, and upper arms.ndividuals with LGMD generally show weakness and wasting restricted to the limb musculature, proximal greater than distal. Proximal weakness refers to weakness of the muscles closer to the center of the body, including the shoulder, pelvic girdle, upper thighs, and upper arms.