Neuromuscular

CENTRAL CORE DISEASE: Pathology


Cores: Central clear zone ("core") in muscle fibers
  NADH stain: Well defined central region of reduced staining
  Cores run whole length of muscle fibers

NADH stain

Central Core Disease: Adult


H&E stain

ATPase, pH 9.4

H&E stain

ATPase, pH 9.4
Adult: Myopathic changes in mild central core disease
  Fiber size: Variability
  Connective tissue: Normal or Mildly increased
  Internal nuclei: Some muscle fibers
  Muscle fiber types
    Marked type I muscle fiber predominence
    Some cores have loss of central myofibrillar structure

NADH

VvG
Some cores are visible on VvG or Gomori trichrome stains

Gomori trichrome

All fibers are Type I: Central cores are visible

ATPase, pH 9.4


SDH stain

PAS stain
Mitochondria
Not present in cores
Glycogen
No glycogen in cores


Central Core Disease with Myopathy


H&E stain

H&E stain

NADH stain

Central Core Disease: Young Child


H&E stain

H&E stain

H&E stain

Gomori trichrome stain

NADH stain

ATPase pH 9.4 stain

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12/23/2014