Neuromuscular

MYH7 disorders

Childhood
Gowers-Laing: Adult

Gowers-Laing: Adult

Muscle fiber sizes
  Varied: Small to Hypertrophied
  Bimodal distribution

H & E stain


H & E stain
Small muscle fibers
  Often present in clusters
  May be intermediate sized or very small
  May be round, polygonal or angular

H & E stain

H & E stain


Gomori trichrome stain
Small muscle fibers: Varied shapes & sizes

Gomori trichrome stain

VvG stain


NADH stain
Small muscle fibers: Stain dark with NADH

NADH stain


ATPase pH 4.3 stain
Small muscle fibers: Most are type I (Dark stained)

ATPase pH 4.3 stain


ATPase pH 9.4 stain
Type I muscle fibers (Pale stained)
  May be large or small
  May be present in groups or clusters

ATPase pH 9.4 stain

Serial sections with different stains

ATPase pH 4.3 stain


ATPase pH 9.4 stain


NADH stain


PAS stain


Cytochrome oxidase stain

MYH7 mutation: Childhood



H&E stain
Fiber sizes
  Varied
  Bimodal distribution

Congo red stain

Muscle fiber internal architecture
  Irregular
  "Smudged" regions with reduced staining

Gomori trichrome stain


NADH stain
Muscle fiber internal architecture
  May be coarse with variable dark & light-stained areas

NADH stain

Muscle fiber internal architecture
  Reduced mitochondrial staining in some areas

Cytochrome oxidase stain


ATPase pH 9.4 stain
Muscle fiber types
  Type 2, especially 2B, fibers tens to be smaller than type 1

ATPase pH 4.3 stain


ATPase pH 4.6 stain

Neuromuscular junctions
  Large & Dark stained for esterase

Esterase stain

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4/11/2016