Myofibrillar Myopathy 4 (MFM4): ZASP

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H&E stain

Myopathic muscle
  Fiber sizes
    Varied
    Hypertrophy & Moderate atrophy
    Small fibers may occur in clusters (Below)
  Internal nuclei
  Vacuoles & Aggregates (Arrow): In scattered muscle fibers

H&E stain

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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

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Gomori trichrome stain

Vacuoles: Contain red-stained material at edges

Gomori trichrome stain

Gomori trichrome stain

Gomori trichrome stain

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

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VvG stain

Aggregates: Dark-stained; Irregular shapes
Vacuoles: Irregular shape; Gray border; Several in single muscle fiber (Right)

VvG stain

VvG stain

VvG stain

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AMPDA stain

Aggregates: Irregular; Cytoplasm; Large or Small

AMPDA stain

AMPDA stain

AMPDA stain

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Congo red stain

Congo red stain

Aggregates: Congophilic (Above right)
Vacuoles: Clear; Well defined border; May have basophilic debris (Below)

Congo red stain

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NADH stain

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

NADH stain

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Aggregates
  Displace myofibrillar apparatus
  Present in fiber types I & II

ATPase pH 9.4 stain

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Desmin: Mildly irregular distribution in cytoplasm

Desmin stain

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Caveolin-3 stain

Caveolin-3: Present in some vacuoles & aggregates

Caveolin-3 stain

Caveolin-3 stain

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Dystrophin: Present in few splits & amp; aggregates

Dystrophin (Dys2) stain

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5/10/2023