Muscular dystrophy

Gastrointestinal involvement may occur in a number of forms of muscular dystrophy including myotome muscular dystrophy and the dystro-phinopathies Duchenne and Becker muscular dystrophy. Involvement of the smooth muscle of the gastrointestinal tract and bladder is well described in myotonic muscular dystrophy, and seemingly motor abnormalities can be found throughout the whole gastrointestinal tract.23 Duchenne and the milder variety Becker muscular dystrophy are due to abnormalities of dysrophin.24 The predominant effects are on skeletal and cardiac muscle. Abnormality of particularly the smooth muscle of the foregut also occurs, with impairment of gastric emptying and disordered proximal small intestinal motor activity demonstrable on manometry. Histological studies show that the intestinal smooth muscle cells become swollen, then destroyed, and progressively replaced by fat.25

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