Neuromuscular

Neuropathy with IgM antibody vs GD1b (CANOMAD)


No inflammation
Axon loss

H&E stain


Gomori trichrome stain
Loss of myelinated axons: Severe

VvG stain


Toluidine blue stain
Loss of myelinated axons: Severe

Toluidine blue stain


Neurofilament stain
Large Axons: Severe loss
Small axons: Relative preservation
  See: Control nerve: Many large axons

Neurofilament stain


Neurofilament (Green) & Myelin basic protein (MBP) (Red) stains
Large Myelinated Axons
  Severe loss
  Only a few remaining axons have associated myelin basic protein
  Normals: Largest myelinated axons commonly have myelin containing MBP

Neurofilament (Green) & Myelin basic protein (MBP) (Red) stains

p0
  In myelin around a few remaining larger axons
  p0 is also present in Schwann cells with no associated axons (Büngner bands)
  Normals
    p0 is present in myelin on most myelinated axons
    Few or no empty p0 cells without axons

Neurofilament (Green) & P0 (Red) stains

NCAM
  Present around most small, unmyelinated axons
  NCAM is also present in Schwann cells with no associated axons (Büngner bands)
  Few large axon stained for neurofilaments
  Normals
    Few or no empty p0 cells without axons
    Many large axons with no associated NCAM

Neurofilament (Green) & NCAM (Red) stains

Büngner bands
  p0 and NCAM co-localize in Schwann cells with no associated axons (Yellow)
  Small NCAM positive cells (Green) are associuated with remaining small, unmyelinated axons
  Normal: Many p0 positive myelin sheaths with no NCAM

NCAM (Green) & p0 (Red) stains


Congo red stain
Endoneurial Vessels: Large with thick walls (Arrows)

Toluidine blue stain

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12/28/2018