Relapsing CIDP, Severe: Electrodiagnostic summary
Relapsing CIDP: Endoneurial edema, No lymphocytic inflammation
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevvlp.jpg)
H&E stain
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H&E stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevhehp.jpg)
H&E stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevgtmp.jpg)
Gomori trichrome stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevvvpmp.jpg)
VvG stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbmp.jpg)
Toluidine blue stain
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Subperineurial Edema
Loss of Myelinated Axons: Severe
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbhp2.jpg)
Toluidine blue stain
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Endoneurial Microvessels: Endothelial cells prominent
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevhelp.jpg)
H&E stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevhehp2.jpg)
H&E stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevgtmp2.jpg)
Gomori trichrome stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevgthp.jpg)
Gomori trichrome stain
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CIDP relapsing: Endoneurial microvessels with prominent endothelial cells
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevalbhp.jpg)
Alcian blue stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevacphp.jpg)
Acid phosphatase stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpvvghp.jpg)
VvG stain
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Endoneurial Microvessels: Large endothelial cells
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbvhp.jpg)
Toluidine blue stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp5.jpg)
From R Schmidt
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Endoneurial Microvessels: Large endothelial cells
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp7.jpg)
From R Schmidt
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CIDP: Myelinated Axons: Markedly Reduced
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevgtmp3.jpg)
Gomori trichrome stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevvvgmp.jpg)
VvG stain
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CIDP relapsing: No Myelinated axons are stained
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevalblp.jpg)
Alcian blue stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevnfmpar.jpg)
Neurofilament stain
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CIDP relapsing
Larger axons, that would normally be myelinated, are present (Arrow)
The largest axons have been lost
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevnfhp.jpg)
Neurofilament stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbvhp3.jpg)
Toluidine blue stain
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CIDP relapsing
Larger axons, that would normally be myelinated, are present
The largest axons have been lost
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbvhp4.jpg)
Toluidine blue stain
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![](../pics/biopsy/myelin/cidprelapsesev/nfgrp0rd.jpg)
Neurofilament (Green) + P0 (Red) stain
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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
![](../pics/biopsy/myelin/cidprelapsesev/nfgrncamrd.jpg)
Neurofilament (Green) + NCAM (MBP) (Red) stain
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![](../pics/biopsy/myelin/cidprelapsesev/nfgrncamrd3.jpg)
Neurofilament (Green) + NCAM (MBP) (Red) stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp3.jpg)
From R Schmidt
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CIDP relapsing
Larger (Intermediate-sized) axons, that would normally be myelinated, are present &
surrounded by Schwann cell processes but not myelin
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp4.jpg)
From R Schmidt
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp6.jpg)
From R Schmidt
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CIDP relapsing
Larger (Intermediate-sized) axons, that would normally be myelinated, are present &
surrounded by Schwann cell processes but not myelin
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemhp3.jpg)
From R Schmidt
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemvhp3.jpg)
From R Schmidt
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemhp.jpg)
From R Schmidt
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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
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemhp2.jpg)
From R Schmidt
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemmp.jpg)
From R Schmidt
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CIDP relapsing
Myelinated axons surrounded by Schwann cell processes & Schwann cells
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevemhp4.jpg)
From R Schmidt
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CIDP relapsing: Small axons are relatively preserved
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevnflp.jpg)
Neurofilament stain
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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
![](../pics/biopsy/myelin/cidprelapsesev/nfgrmbprd.jpg)
Neurofilament (Green) + Myelin Basic Protein (MBP) (Red) stain
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CIDP relapsing: Schwann cells
Büngner band Schwann cells (Yellow) co-stain for NCAM & P0
![](../pics/biopsy/myelin/cidprelapsesev/ncamgrp0rd.jpg)
NCAM (Green) + P0 (Red) stain
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CIDP relapsing, Schwann cells: Non-myelinating Schwann cells are diffusely distributed in endoneurium
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevncam.jpg)
NCAM stain
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CIDP, Relapsing
Endoneurial cells: Darkly stained
Epineurial vessels: Normal smooth muscle
ATPase pH 4.3 stain
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Small histiocytic cells in Endoneurium
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevacp.jpg)
Acid phosphatase stain
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Multiple small epineurial vessels
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevuea1.jpg)
UEA I stain
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![](../pics/biopsy/myelin/cidprelapsesev/cidpsevc5b9mp2.jpg)
C5b-9 stain
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Peineurial Pathology: Patchy
C
5b-9 staining of perineurium is pale (Above, Left)
Perineurium: Damaged structure; Cellular invasion (Below)
![](../pics/biopsy/myelin/cidprelapsesev/cidpsevtbhp6.jpg)
Toluidine blue stain
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4/15/2021