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RYR3 Myopathy: Nemaline rods ¹⁴⁴

All images from: Yalda Nilipour MD

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H&E stain

Fiber sizes: Bimodal Distribution
  Type 1 fibers: Small & Intermediate sized
  Type 2 fibers: Large
Necrosis & Immaturity
  Few muscle fibers (Below)
Myonuclei
  Internal in some fibers

H&E stain

Fiber sizes: Bimodal Distribution
  Type 1 fibers: Small & Intermediate sized
  Type 2 fibers: Large
Fiber types
  Type 1: Predominant

NADH stain

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H&E stain

Muscle fiber morphology
  Aggregates
    Eosinophilic
    Location: Often subsarcolemmal
  Myonuclei
    Oten near, or within, aggregates
    Irregular shapes
    May be clustered

H&E stain

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Gomori trichrome stain

Nemaline Rods
  Present in many larger & intermediate-sized muscle fibers
  Occur in clusters

Gomori trichrome stain

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Gomori trichrome stain

Nemaline Rods
  Present in many larger & intermediate-sized muscle fibers
  Occur in clusters

Gomori trichrome stain

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NADH stain

Muscle fibers: Internal architecture
  Irregular-shaped inclusions in some darker stained muscle fibers

NADH stain

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NADH stain

Muscle fibers: Internal architecture
  Irregular in darker stained muscle fibers

NADH stain

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8/13/2021