Myofibrillar Myopathy 4 (MFM4): ZASP
![](../pics/biopsy/zasp/zasphelp.jpg)
H&E stain
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Myopathic muscle
Fiber sizes
Varied
Hypertrophy & Moderate atrophy
Small fibers may occur in clusters (Below)
Internal nuclei
Vacuoles & Aggregates (Arrow): In scattered muscle fibers
![](../pics/biopsy/zasp/zasphear.jpg)
H&E stain
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![](../pics/biopsy/zasp/zaspfibhehp1.jpg)
H&E stain
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![](../pics/biopsy/zasp/zaspfibhehp3.jpg)
H&E stain
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Muscle fiber Morphology
Vacuoles
Aggregates
Myonuclei: Irregular shapes; Increased numbers at edge of fibers
![](../pics/biopsy/zasp/zaspfibhehhp4.jpg)
H&E stain
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![](../pics/biopsy/zasp/zaspfibhehp2.jpg)
H&E stain
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![](../pics/biopsy/zasp/zaspvacgt.jpg)
Gomori trichrome stain
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Vacuoles: Contain red-stained material at edges
![](../pics/biopsy/zasp/zaspfibgthp3a.jpg)
Gomori trichrome stain
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![](../pics/biopsy/zasp/zaspfibgthp2a.jpg)
Gomori trichrome stain
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![](../pics/biopsy/zasp/zaspfibgthp1a.jpg)
Gomori trichrome stain
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Aggregates: Dark stained; Large, Small or Cytoplasmic bodies
Vacuoles: Contain red-stained material at edges
![](../pics/biopsy/zasp/zaspvvg.jpg)
VvG stain
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Aggregates: Dark-stained; Irregular shapes
Vacuoles: Irregular shape; Gray border; Several in single muscle fiber (Right)
![](../pics/biopsy/zasp/zaspfibvvghp1.jpg)
VvG stain
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![](../pics/biopsy/zasp/zaspfibvvghp3.jpg)
VvG stain
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![](../pics/biopsy/zasp/zaspfibvvghp2.jpg)
VvG stain
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![](../pics/biopsy/zasp/zaspfibampdalp.jpg)
AMPDA stain
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Aggregates: Irregular; Cytoplasm; Large or Small
![](../pics/biopsy/zasp/zaspfibampda2.jpg)
AMPDA stain
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![](../pics/biopsy/zasp/zaspfibampda3.jpg)
AMPDA stain
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![](../pics/biopsy/zasp/zaspfibampda1a.jpg)
AMPDA stain
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![](../pics/biopsy/zasp/zaspfibcrhp1.jpg)
Congo red stain
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![](../pics/biopsy/zasp/zaspfibcrhp2.jpg)
Congo red stain
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Aggregates: Congophilic (Above right)
Vacuoles: Clear; Well defined border; May have basophilic debris (Below)
![](../pics/biopsy/zasp/zaspcr2.jpg)
Congo red stain
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![](../pics/biopsy/zasp/zaspfibnadh1.jpg)
NADH stain
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Aggregates: Displace internal membranes
Internal membranes: Clustered in some regions
![](../pics/biopsy/zasp/zaspfibnadh2.jpg)
NADH stain
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Aggregates
Displace myofibrillar apparatus
Present in fiber types I & II
![](../pics/biopsy/zasp/zaspatp94.jpg)
ATPase pH 9.4 stain
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Desmin: Mildly irregular distribution in cytoplasm
![](../pics/biopsy/zasp/zaspdesmin.jpg)
Desmin stain
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![](../pics/biopsy/zasp/zaspcav3.jpg)
Caveolin-3 stain
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Caveolin-3: Present in some vacuoles & aggregates
![](../pics/biopsy/zasp/zaspfibcav31.jpg)
Caveolin-3 stain
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![](../pics/biopsy/zasp/zaspfibcav32.jpg)
Caveolin-3 stain
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Dystrophin: Present in few splits & amp; aggregates
![](../pics/biopsy/zasp/zaspfibdys2.jpg)
Dystrophin (Dys2) stain
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Myopathies with wasting
5/10/2023