Neuromuscular

Myofibrillar Myopathy 4 (MFM4): ZASP


Myopathic muscle
  Fiber size: Varied; Hypertrophy & Moderate atrophy
  Internal nuclei
  Vacuoles & Aggregates: In scattered muscle fibers

H&E stain


H&E stain

H&E stain
Muscle fiber Morphology
  Vacuoles
  Aggregates
  Myonuclei: Irregular shapes; Increased numbers at edge of fibers

H&E stain

H&E stain

Aggregates: Dark stained; Large, Small or Cytoplasmic bodies
Vacuoles: Contain red-stained material at edges

Gomori trichrome stain

Gomori trichrome stain

Gomori trichrome stain

Aggregates: Dark-stained; Irregular shapes
Vacuoles: Irregular shape; Several in single muscle fiber

VvG stain

VvG stain

VvG stain


AMPDA stain
Aggregates: Irregular; Cytoplasm; Large or Small

AMPDA stain

AMPDA stain

AMPDA stain

Vacuoles: Clear; Well defined border; No basophilic debris
Aggregates: Congophilic

Congo red stain

Congo red stain


NADH stain
Aggregates: Displace internal membranes
Internal membranes: Clustered in some regions

NADH stain

Aggregates
  Displace myofibrillar apparatus
  Present in fiber types I & II

ATPase pH 9.4 stain

Desmin: Mildly irregular distribution in cytoplasm

Desmin stain


Caveolin-3 stain
Caveolin-3: Present in some vacuoles & aggregates

Caveolin-3 stain

Caveolin-3 stain

Dystrophin: Present in few splits & amp; aggregates

Dystrophin (Dys2) stain

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2/26/2016