Neuromuscular

MITOCHONDRIAL DISEASE: Megaconial Myopathy

Congenital Myopathy
Selenium deficiency



H&E stain

H&E stain

H&E stain
Myopathy: Varied muscle fiber size, Internal nuclei, Regeneration Stippling, Basophilic


Gomori trichrome stain

VvG stain

AMPDA stain
Stippling: Toward periphery of muscle fibers


COX stain

COX stain

COX stain
Mitochondria: Large; Non-uniform distribution


SDH stain

SDH stain
Mitochondria: Large; Non-uniform distribution


ATPase pH 9.4 stain

ATPase pH 4.3 stain

Acid Phosphatase stain
Type 2 fibers are larger than type 1
Large mitochondria: More common in type 2 fibers
Scattered type 2C muscle fibers
 
Necrotic Muscle Fibers
 


Sudan stain

PAS stain
Muscle fibers: Lipid mildy increased; Glycogen increased

Return to Megaconial Myopathy
Return to Mitochondrial syndromes
Return to Muscle biopsies

7/11/2011