Neuromuscular

Myopathy with deficient Chondroitin Sulfate C in Connective tissue

Myopathy
  Myonuclei Δ
  Atrophy
Inflammation
Perimysium
  Histiocytes
  Alkaline phosphatase
Chrondroitin SO4 C


H&E stain

H&E stain
Muscle fiber morphology

H&E stain


H&E stain
Muscle fibers
  Myonuclei with irregular shapes
  Varied fiber size

H&E stain

Congo red stain
Muscle fibers: Myonuclei with irregular shapes


Gomori trichrome stain
Muscle fibers: Varied size


H&E stain
Perifascicular muscle fiber atrophy

Type 2 muscle fibers: Very small
Type 1 muscle fibers: Mild atrophy; Varied size

ATPase pH 9.4 stain

ATPase pH 9.4 stain


Congo red stain
Inflamatory pathology
  Cells near & around Perimysial vessel

Congo red stain

H&E stain

H&E stain


Acid phosphatase stain
Perimysium
  Acid phosphatase positive histiocytic cells

Acid phosphatase stain

Alkaline phosphatase stain

Alkaline phosphatase stain
Perimysium
  Increased staining by alkaline phosphatase

Alkaline phosphatase stain
Connective tissue changes
Chondroitin sulfate C: Patterns of Staining

Chondroitin sulfate C stain
Normal control
  Dark staining on endomysum & capillaries (Arrow)

Chondroitin sulfate C stain
Myopathy
  Reduced staining on endomysum & capillaries


Chondroitin sulfate C stain
Myopathy
  Reduced staining on endomysum & capillaries; Increased staining in muscle fiber cytoplasm


Chondroitin sulfate C stain
Patient muscle: Pre-Rx

Chondroitin sulfate C stain
Patient: Post steroid Rx
Chondroitin sulfate staining in muscle endomysium


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1/28/2019