BMD Pathology

DYSTROPHINOPATHIES: Becker

Myopathic grouping

Clusters of neighboring necrotic or regenerating muscle fibers all at same stage Most distinctive myopathic change in Becker's muscular dystrophy. Most prominent in patients aged < 12 years. Early change (top) Necrosis of several neighboring muscle fibers. Esterase-positive phagocytic cells Regeneration (middle): Numerous small rounded NADH-positive muscle fibers. Later stage (bottom): A group of intermediate-sized, basophilic muscle fibers
Cluster of necrotic muscle fibers replaced by esterase positive cells (Biopsy from 7 year old boy)
H & E stain	Esterase stain

Grouped regenerating muscle fibers (Biopsy from 7 year old boy)
H & E stain	NADH stain

H & E stain

Esterase stain

Becker Muscular Dystrophy: Biopsy from 27 year old male

Older Patients

Findings are typical of chronic dystrophies
Increased endomysial connective tissue
Variable fiber size: Small muscle fibers are rounded
Internal nuclei
The largest muscle fibers are hypertrophied
Occasional fibers: Degeneration; Regeneration; Hypercontraction; Split

Becker Muscular Dystrophy: Dystrophin staining


Normal dystrophin staining around the rim of muscle fibers.	Reduced dystrophin staining

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4/23/2004