Muscular dystrophy



Muscular dystrophy





Description



  • Hereditary disorder characterized by progressive symmetrical wasting of skeletal muscles


  • No neural or sensory defects


  • Four main types: Duchenne’s (pseudohypertrophic), Becker’s (benign pseudohypertrophic), Landouzy-Dejerine (facioscapulohumeral), and Erb’s (limb-girdle) dystrophies


  • Duchenne’s beginning in early childhood; death within 10 to 15 years


Pathophysiology



  • Muscle fibers necrotize and regenerate in various states.


  • As regeneration slows down, degeneration dominates.


  • Fat and connective tissue replace muscle fibers, with ensuing weakness.


Causes



  • Duchenne’s and Becker’s X-linked recessive


  • Erb’s usually autosomal recessive


  • Landouzy-Dejerine autosomal dominant


  • Various genetic mechanisms (band Xp 21)


Assessment findings


Duchenne’s and Becker’s

Jul 20, 2016 | Posted by in NURSING | Comments Off on Muscular dystrophy

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