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CONGENITAL MUSCULAR DYSTROPHY

Laminin-α2 (Merosin) Negative



H&E stain
Congenital muscular dystrophy: Myopathic changes
  Fiber size: Variability
  Immature muscle fibers: Internal nuclei; Basophilia
  Connective tissue: Increased in endomysium & perimysium


H&E stain

 

 
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H&E stain

H&E stain


GT stain
CMD: Laminin-α2 (Merosin) Negative
  Endomysial connective tissue: Increased
  Muscle fiber size: Varied

VvG stain

Congenital muscular dystrophy: Other changes

Many immature (Type 2C) intermediate-staining muscle fibers

ATPase pH 4.3 stain

Muscle Fiber Necrosis, Minor
  Occasional acid phosphatase positive cells

Acid phosphatase stain

Laminin-α2 (Merosin) staining
Laminin-α2: Normal muscle

Merosin is normally located in:
  Basal lamina: Muscle fiber surface
  Intramuscular nerves


Laminin-α2: Congenital muscular dystrophy

  Laminin-α2 staining is absent from muscle fiber surfaces



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5/23/2017