Neuromuscular

DYSTROPHINOPATHIES: Becker


Myopathic grouping

  • Clusters of neighboring necrotic or regenerating muscle fibers all at same stage
  • Most distinctive myopathic change in Becker's muscular dystrophy.
  • Most prominent in patients aged < 12 years.
  • Early change (top)
    • Necrosis of several neighboring muscle fibers.
    • Esterase-positive phagocytic cells
  • Regeneration (middle): Numerous small rounded NADH-positive muscle fibers.
  • Later stage (bottom): A group of intermediate-sized, basophilic muscle fibers

Cluster of necrotic muscle fibers replaced by esterase positive cells (Biopsy from 7 year old boy)

H & E stain

Esterase stain

Grouped regenerating muscle fibers (Biopsy from 7 year old boy)

H & E stain

NADH stain

H & E stain

Esterase stain


Becker Muscular Dystrophy: Biopsy from 27 year old male
Older Patients
  • Findings are typical of chronic dystrophies
  • Increased endomysial connective tissue
  • Variable fiber size: Small muscle fibers are rounded
  • Internal nuclei
  • The largest muscle fibers are hypertrophied
  • Occasional fibers: Degeneration; Regeneration; Hypercontraction; Split


Becker Muscular Dystrophy: Dystrophin staining
Normal dystrophin Reduced dystrophin
Normal dystrophin staining
around the rim of muscle fibers.
Reduced dystrophin staining



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4/23/2004