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Progressive External Ophthalmoplegia: EOM pathology

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H&E stain

PEO: Myopathic Muscle

• Muscle fibers
  • Sizes: Varied
  • Internal architecture: Coarse; NADH dark
  • Fiber types: Mostly type II
  • Mitochondrial stains
    • COX & SDH: Dark in most areas
    • Ragged red fibers: Few
  • Lysosomal granules
  • NMJs: Multisegmented
• Endomysial connective tissue
  • Increased amounts: May have prominent fibril content
  • Capillaries: Sinusoid shapes; Alkaline phosphatse stain prominent
• Intramuscular nerves: Normal

H&E stain

VvG stain

PEO: Myopathic Muscle

• Muscle fibers
  • Sizes: Varied
  • Internal architecture: Coarse
• Endomysial connective tissue: Increased
• Intramuscular nerves: Normal

VvG stain

Gomori trichrome stain

H&E stain

PEO: Myopathic Muscle

• Muscle fibers
  • Sizes: Varied
• Endomysial connective tissue: Increased

H&E stain

Gomori trichrome stain

Gomori trichrome stain

PEO: Myopathic Muscle

• Muscle fibers
  • Sizes: Varied
  • Internal architecture: Coarse; NADH dark
  • Mitochondrial stains
    • Ragged red fibers: Few
• Endomysial connective tissue: Increased

Gomori trichrome stain

VvG stain

PEO: Myopathic Muscle
  Endomysial connective tissue: Increased; Fibrillar

VvG stain

NADH stain

PEO: Myopathic Muscle
  NADH stains sarcoplasm dark

NADH stain

ATPase pH 4.3 stain PEO: Myopathic Muscle   Must muscle fibers are type II See: Control EOM

ATPase 9.4 stain

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PEO: Myopathic Muscle
  Muscle fiber cytoplasm: Patchy Acid phosphatase staining

Acid phosphatase stain

COX stain

PEO: Mitochondria
  Dark staining in must fibers for SDH & COX

SDH stain

Esterase stain

PEO: Neuromuscular Junctions
  Multisegmented
  Dark stained
See: Control EOM

Esterase stain

VvG stain

PEO: Myopathic Muscle
  Endomysial connective tissue: Increased; Contains Alkaline phosphatase positive capillaries

Alkaline phosphatase stain

Alkaline phosphatase stain

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11/13/2022