ACOX1: Mitchell syndrome
![](../pics/biopsy/nerve/acox1/acox1help.jpg)
H&E stain
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Nerve Morphology: Normal Perineurium, Epineurium & Vessels
![](../pics/biopsy/nerve/acox1/acox1gtlp.jpg)
Gomori trichrome stain
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Axon loss: Varied within some fasicles
![](../pics/biopsy/nerve/acox1/acoxvvglp.jpg)
VvG stain
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Neurofilament stain
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Large Axon loss: Small axons relatively preserved
Neurofilament stain
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![](../pics/biopsy/nerve/acox1/acox1ncam.jpg)
NCAM stain
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Non-myelinating Schwnn cells: Present in clusters around small axons
![](../pics/biopsy/nerve/acox1/acoxvessvvg.jpg)
VvG stain
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Epineurial artery: Fibrils mildly reduced
Myelinated axons: Morphology & Pathology
![](../pics/biopsy/nerve/acox1/acox1tbmp.jpg)
Toluidine blue stain
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Myelinated Axons
Myelin sheaths: Normal thickness
Axon numbers: Loss of large & small myelinated axons
Wallerian degeneration
![](../pics/biopsy/nerve/acox1/acox1tbaxdegsm.jpg)
Toluidine blue stain
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Wallerian Degeneration
Scattered cells containing myelin debris & lipid droplets
![](../pics/biopsy/nerve/acox1/acox1wdtb.jpg)
Toluidine blue stain
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Myelin debris in phagocytic Schwann cells
Lipid droplets & Debris in Endoneurial macrophage
![](../pics/biopsy/nerve/acox1/acox1tbirregmyel.jpg)
Toluidine blue stain
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Myelin sheath: Irregular folding around some axons
![](../pics/biopsy/nerve/acox1/acox1nfgp0rsm.jpg)
Neurofilament (Green) + P0 (Red) stains
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Myelinated axons (Yellow): Normal to reduced numbers
Acute axon loss: Some P0 regions (Red) with no axons
![](../pics/biopsy/nerve/acox1/acox1nfgmbprsm.jpg)
Neurofilament (Green) + MBP (Red) stains
Axon loss: Acute
MBP regions (Red) with no associated axon
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![](../pics/biopsy/nerve/acox1/acox1nfmbpsm.jpg)
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![](../pics/biopsy/nerve/acox1/acox1ncamgp0rsm.jpg)
NCAM (Green) + P0 (Red) stains
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Axon loss: Chronic
Schwann cells, scattered, (Yellow) that co-stain for both NCAM & P0
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7/25/2020