Neuromuscular

DYSTROPHINOPATHIES: Becker

Myopathic grouping

  • This is the most distinctive myopathic change in Becker's muscular dystrophy.
  • It is most prominent in patients aged < 12 years.
  • Early change (top)
    • Degeneration of several neighboring muscle fibers.
    • Esterase-positive phagocytic cells
  • Regeneration (middle): Numerous small rounded NADH-positive muscle fibers.
  • Later (bottom): A group of intermediate-sized, basophilic muscle fibers
Cluster of degenerated 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