Neuromuscular

PHOSPHORYLASE DEFICIENCY (McARDLE'S)



H & E stain
Subsarcolemmal vacuoles


Phosphorylase stains
McArdle's disease
Some phosphorylase staining is present in vessels & regenerating muscle fibers

Phosphorylase stain: Normal

Type II fibers stain more darkly than type I  

Phosphorylase stain: McArdle's disease

Myophosphorylase deficiency
Muscle fibers stain yellow


NADH stains

NADH stain

Linearized internal architecture

In type II (paler) muscle fibers.
Occurs in ~50% of biopsies.


Subsarcolemmal bleb

Longitudinal section


PAS stain



Increased glycogen

Glycogen is increased all through cytoplasm.
Type II fibers stain more darkly than type I.
ATPase pH 9.4 stain



Vacuoles are present in both type I and type II muscle fibers



Also see Acid maltase deficiency: Adult; Child

Return to Glycogen storage disorders
Return to Muscle biopsies

2/23/05