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
Wide stance and waddling gait
Gowers’ sign when rising from a sitting or supine position
Muscle hypertrophy and atrophy
Calves enlarging because of fat infiltration into the muscle
Posture changes
Lordosis and a protuberant abdomen
Scapular “winging” or flaring when raising arms
Contractures
Tachypnea and shortness of breathStay updated, free articles. Join our Telegram channel
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