Neuromuscular

Myopathy with deficient chondroitin sulfate C in connective tissue


H&E stain

ATPase pH 9.4 stain
Muscle fiber morphology
  • Variability in fiber size
  • Small fibers are angular & type 2 (Dark on ATPase pH 9.4)
  • No active myopathic changes



H&E stain

Acid phosphatase stain

Alkaline phosphatase stain
Connective tissue changes
  • Perivascular inflammatory infiltrate: Rare
  • Perimysial cells staining with acid phosphatase: Occasional
  • Perimysium staining with alkaline phosphatase



Antibody to Chondroitin sulfate C:
Immunoperoxidase stain


Patient muscle before treatment

Antibody to Chondroitin sulfate C:
Immunoperoxidase stain


Normal muscle

Antibody to Chondroitin sulfate C:
Immunoperoxidase stain


Patient muscle after
corticosteroid treatment
Chondroitin sulfate staining in muscle endomysium
  • Normally abundant in skeletal muscle endomysium
  • Absent in patient muscle before treatment
  • Returns to near normal after corticosteroid treatment



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6/29/2002