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Muscle

Muscle. Normal muscle. Muscle diseases. Primary Changes: Inflammation Structural (dystrophies and congenital myopathies) Metabolic diseases (eg glycogenoses, mitochondrial diseases) Secondary Changes: Neurogenic atrophy (due to loss of innervation and reiinervation). Neurogenic Atrophy.

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Muscle

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  1. Muscle

  2. Normal muscle

  3. Muscle diseases • Primary Changes: • Inflammation • Structural (dystrophies and congenital myopathies) • Metabolic diseases (eg glycogenoses, mitochondrial diseases) • Secondary Changes: • Neurogenic atrophy (due to loss of innervation and reiinervation)

  4. Neurogenic Atrophy

  5. Inflammatory myopathies

  6. Inflammatory myopathies • Dermatomyositis: • Polymyositis • Inclusion body myositis

  7. Differences

  8. Dermatomyositis

  9. Polymyositis

  10. Muscular Dystrophies Characterized by: • Necrosis of individual muscle fibers • Accumulation of connective tissue around individual muscle fibers • Usually structural protein missing (genetic defect)

  11. Duchenne’s Muscular Dystrophy

  12. DMD • X-linked (males affected, female carriers) • Early onset (around 4 yoa) • Weakness Quadriceps and Gastrocnemius first followed by all proximal muscles • Often swollen gastrocnemius region early in disease. • If untreated (respiratory support), usual age of death around 18 yoa

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