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LGMD 2D: α-Sarcoglycan deficiency

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Muscle: General features

H&E stain Muscle fibers: Features   Varied sizes   Hypercontraction   Myopathic groups: Clusters of smaller muscle fibers Endomysial connective tissue: Increased between muscle fibers

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Muscle Fiber Degeneration: Grouped Pathology

H&E stain Group of degenerated muscle fibers

NADH stain Degenerating muscle fibers: Reduced NADH stain

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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

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

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H&E stain Necrotic & Regenerating (Immature) muscle fibers

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Muscle Fiber Regeneration

H&E stain Group of regenerated muscle fibers

NADH stain Small regenerated muscle fibers: Dark on NADH stain

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ATPase stain, pH 4.3 Immature muscle fibers: Intermediate staining

Alkaline phosphatase stain Immature muscle fibers: Alkaline phosphatase

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LGMD 2D: Dystrophy-related protein staining

α-Sarcoglycan

α-Sarcoglycan LGMD 2D: Markedly reduced

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

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Dystrophin

Dystrophin LGMD 2D: Mildly reduced

Dystrophin Normal: Present around rim of muscle fibers

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7/28/2023