Sporadic, Late Onset Nemaline Myopathy (SLONM)
Muscle features
- Muscle fiber size: Varied
- Myonuclei: Large; May be clumped
- Internal architecture
- Fiber types
- Type I: Predominance
- Type 2: Small; Many 2C
SLONM: Myopathy
Fiber size: Varied
Endomysial connective tissue: Increased
H&E stain
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H&E stain
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SLONM: Myonuclei
Large
May be clumped
H&E stain
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Congo red stain
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Congo red stain
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Gomori trichrome stain
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Gomori trichrome stain
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SLONM: Muscle Fiber Internal Architecture
VvG stain
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VvG stain
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VvG stain
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NADH stain
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SLONM: Nemaline Rods
Gomori trichrome stain
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Gomori trichrome stain
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Gomori trichrome stain
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Gomori trichrome stain
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SLONM: Clustered Rod-like Material
Gomori trichrome stain
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Gomori trichrome stain
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Gomori trichrome stain
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Gomori trichrome stain
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SLONM: AMPDA aggregates
AMPDA stain
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SLONM: Fiber types
Type I predominance
Small fibers mostly 2C
ATPase pH 4.3 stain
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6/13/2019