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ACOX1: Mitchell syndrome

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H&E stain

Nerve Morphology: Normal Perineurium, Epineurium & Vessels

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Gomori trichrome stain

Axon loss: Varied within some fasicles

VvG stain

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Neurofilament stain

Large Axon loss: Small axons relatively preserved

Neurofilament stain

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NCAM stain

Non-myelinating Schwnn cells: Present in clusters around small axons

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VvG stain

Epineurial artery: Fibrils mildly reduced

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Myelinated axons: Morphology & Pathology

Toluidine blue stain

Myelinated Axons
  Myelin sheaths: Normal thickness
  Axon numbers: Loss of large & small myelinated axons
  Wallerian degeneration

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Toluidine blue stain

Wallerian Degeneration
  Scattered cells containing myelin debris & lipid droplets

Toluidine blue stain

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Myelin debris in phagocytic Schwann cells

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Lipid droplets & Debris in Endoneurial macrophage

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Toluidine blue stain

Myelin sheath: Irregular folding around some axons

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Neurofilament (Green) + P0 (Red) stains

Myelinated axons (Yellow): Normal to reduced numbers
  Acute axon loss: Some P0 regions (Red) with no axons

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Neurofilament (Green) + MBP (Red) stains Axon loss: Acute   MBP regions (Red) with no associated axon

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NCAM (Green) + P0 (Red) stains

Axon loss: Chronic
  Schwann cells, scattered, (Yellow) that co-stain for both NCAM & P0

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7/25/2020