Neuromuscular

MYOPATHY WITH IgM ANTI-DECORIN (BJ) ANTIBODIES

Muscle Fiber Pathology


H&E stain
  • Muscle fiber size is variable.
  • Small muscle fibers
    • Are rounded or polygonal
    • May have abnormal internal architecture
  • Endomysial connective tissue is increased.
  • Multiple, large internal nuclei may be present within muscle fibers (Right)

Muscle Fiber Internal Architcture


NADH stain
  • Irregular, moth-eaten, internal architecture.



Immuno-Pathology
  • IgM (brown) stains vessels, and muscle basal lamina, endomysium and perimysium, but not muscle fibers.

Return to Anti-Decorin (BJ) Myopathy

1/13/2001