CONGENITAL MUSCULAR DYSTROPHY (Laminin-α2 (Merosin) -): Pathology

Congenital muscular dystrophy: Myopathic changes

  • Fiber size: Variability
  • Immature muscle fibers: Internal nuclei; Basophilia
  • Connective tissue: Increased in endomysium & perimysium

H&E stain

H&E stain

GT stain

VvG stain

Congenital muscular dystrophy: Other changes

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

ATPase pH 4.3 stain

No Necrosis: No acid phosphatase positive cellularity

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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