Neuromuscular

Chronic Immune Demyelinating Polyneuropathy, Relapsing, Severe

Axons & Myelin
Edema
Microvessels
Non-inflammatory
Perineurium
Other CIDP pathology
Childhood
Demyelination, Active
Differential fascicular Δ
Onion bulbs
Segmental demyelination
Sub-acute onset
Thin myelin sheaths
Tomacula

Relapsing CIDP, Severe: Electrodiagnostic summary

Relapsing CIDP: Endoneurial edema, No lymphocytic inflammation

H&E stain

H&E stain

H&E stain

Gomori trichrome stain

VvG stain


Toluidine blue stain
Subperineurial Edema
Loss of Myelinated Axons: Severe

Toluidine blue stain

Endoneurial Microvessels: Endothelial cells prominent

H&E stain

H&E stain

Gomori trichrome stain

Gomori trichrome stain
CIDP relapsing: Endoneurial microvessels with prominent endothelial cells

Alcian blue stain

Acid phosphatase stain

VvG stain
Endoneurial Microvessels: Large endothelial cells

Toluidine blue stain

From R Schmidt
Endoneurial Microvessels: Large endothelial cells

From R Schmidt

CIDP: Myelinated Axons: Markedly Reduced

Gomori trichrome stain

VvG stain
CIDP relapsing: No Myelinated axons are stained

Alcian blue stain


Neurofilament stain
CIDP relapsing
  Larger axons, that would normally be myelinated, are present (Arrow)
  The largest axons have been lost

Neurofilament stain


Toluidine blue stain
CIDP relapsing
  Larger axons, that would normally be myelinated, are present
  The largest axons have been lost

Toluidine blue stain

Neurofilament (Green) + P0 (Red) stain
CIDP relapsing
  The largest axons have been lost
  Larger axons, that would normally be myelinated, are present but have no surrounding cells that stain for either NCAM or P0
  Axons surrounded by P0 (Above) are often unusually small

Neurofilament (Green) + NCAM (MBP) (Red) stain

Neurofilament (Green) + NCAM (MBP) (Red) stain

From R Schmidt
CIDP relapsing
  Larger (Intermediate-sized) axons, that would normally be myelinated, are present &
  surrounded by Schwann cell processes but not myelin

From R Schmidt

From R Schmidt
CIDP relapsing
  Larger (Intermediate-sized) axons, that would normally be myelinated, are present &
  surrounded by Schwann cell processes but not myelin

From R Schmidt

From R Schmidt

From R Schmidt

Axon, Small (Top left): Surrounded by several Schwann cell processes
Axon, Large (Center of image)
  Contains serpentiginous membrane structures in cytoplasm
  Surrounded by basal lamina: Possible withdrawal of Schwann cell process

From R Schmidt


From R Schmidt
CIDP relapsing
  Myelinated axons surrounded by Schwann cell processes & Schwann cells

From R Schmidt

CIDP relapsing: Small axons are relatively preserved

Neurofilament stain

CIDP relapsing: Loss of large axons, Chronic
  Schwann cells around remaining small axons stain abnormally for MBP
  There are no MBP-containing myelin sheaths around axons

Neurofilament (Green) + Myelin Basic Protein (MBP) (Red) stain

CIDP relapsing: Schwann cells
  Büngner band Schwann cells (Yellow) co-stain for NCAM & P0

NCAM (Green) + P0 (Red) stain

CIDP relapsing, Schwann cells: Non-myelinating Schwann cells are diffusely distributed in endoneurium

NCAM stain

CIDP, Relapsing
  Endoneurial cells: Darkly stained
  Epineurial vessels: Normal smooth muscle

ATPase pH 4.3 stain

Small histiocytic cells in Endoneurium

Acid phosphatase stain

Multiple small epineurial vessels

UEA I stain


C5b-9 stain
Peineurial Pathology: Patchy
  C5b-9 staining of perineurium is pale (Above, Left)
  Perineurium: Damaged structure; Cellular invasion (Below)

Toluidine blue stain


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4/15/2021