Neuromuscular

LGMD 2D: α-Sarcoglycan deficiency


Muscle: General features


H&E stain

Varied size of muscle fibers
Hypercontracted muscle fibers
Increased endomysial connective tissue

Muscle Fiber Degeneration


H&E stain
Group of degenerated muscle fibers

NADH stain
Degenerating muscle fibers: Reduced NADH stain


Acid phosphatase stain
Acid phosphatase-stained cells
in region of degenerated muscle fibers

Alkaline phosphatase stain
Alkaline phosphatase staining
in region of degenerated muscle fibers


H & E stain
Necrotic muscle fiber replaced by cells: Longitudinal view


H&E stain
Degenerating & regenerating muscle fibers

Muscle Fiber Regeneration


H&E stain
Group of regenerated muscle fibers

NADH stain
Small regenerated muscle fibers: Dark on NADH stain


ATPase stain, pH 4.3
Immature muscle fibers: Intermediate staining

Alkaline phosphatase stain
Immature muscle fibers: Alkaline phosphatase

LGMD 2D: Dystrophy-related protein staining

α-Sarcoglycan


α-Sarcoglycan
LGMD 2D: Markedly reduced

α-Sarcoglycan
Normal levels: Present around rim of muscle fibers

Dystrophin


Dystrophin
LGMD 2D: Mildly reduced

Dystrophin
Normal: Present around rim of muscle fibers



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8/11/2003