Neuromuscular

ACOX1: Mitchell syndrome



H&E stain
Nerve Morphology: Normal Perineurium, Epineurium & Vessels


Gomori trichrome stain
Axon loss: Varied within some fasicles

VvG stain


Neurofilament stain
Axon loss: Small axons relatively preserved

Neurofilament stain


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


VvG stain
Epineurial artery: Fibrils mildly reduced

Myelinated axons: Morphology & Pathology

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


Toluidine blue stain
Wallerian Degeneration
  Scattered cells containing myelin debris & lipid droplets

Toluidine blue stain

Myelin debris in phagocytic Schwann cells



Lipid droplets & Debris in Endoneurial macrophage


Toluidine blue stain
Myelin sheath: Irregular folding around some axons


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


Neurofilament (Green) + MBP (Red) stains

Axon loss: Acute
  MBP regions (Red) with no associated axon



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