Neuromuscular

MYOPATHIES WITH MYOSIN LOSS


H&E stain

Some patients have small angular fibers on H&E stain.
These small fibers are often basophilic and have large nuclei (Right).


Gomori trichrome stain

NADH stain
The small muscle fibers stain darkly on Gomori trichrome & NADH.

ATPase pH 9.4 stain

 
The small muscle fibers with myosin loss have less staining than type I muscle fibers on ATPase pH 9.4 stain.
Comparison of ATPase pH 9.4 (Left) & 4.3 (Right) in serial sections
Type I fibers (1): Darker staining at pH 9.4 than 4.3
Type II fibers (2): Darker staining at pH 4.3 than 9.4
Myosin loss fibers (0 or arrows): No staining at both pH 9.4 and pH 4.3

ATPase pH 9.4 stain

ATPase pH 4.3 stain


H & E stain


 

Myosin ATPase, pH 9.4 stain
A focal pattern of myosin loss may also occur in muscle fibers.
  Focal regions of myosin loss in type I and II muscle fibers.
  These regions appear basophilic on H & E stain.
  Myosin loss may occur in large as well as small fibers


Anti-myosin antibody

Reduced myosin in many small
  muscle fibers


Ultrastructure

Thick filament loss with relative preservation of thin filaments & Z-lines

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5/17/2005