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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 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)
Inflammatory myopathies • Dermatomyositis: • Polymyositis • Inclusion body myositis
Muscular Dystrophies Characterized by: • Necrosis of individual muscle fibers • Accumulation of connective tissue around individual muscle fibers • Usually structural protein missing (genetic defect)
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