Focal demyelination: Chronic
No, or little, remyelination
Probably at region of persistent temporal dispersion
Toluidine blue stain
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Myelinated axons
Focal endoneurial region with reduced numbers
Demyelinated axons are common in this area
Remaining myelinated axons often have thin myelin
Toluidine blue stain
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Region of nerve with chronic demyelination but little or no remyeliation
Many completely demyelinated axons
Schwann cells & their processes surrounding the demyelinated axons
Phagocytosis of myelin is complete: No
histiocytes or myelin debris
This pathology is likely associated with a region of
chronic partial conduction block or temporal dispersion on electrodiagnostic testing
Electron micrographs: From Robert Schmidt MD
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Nerve conduction studies (Median nerve)
Temporal dispersion
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Demyelinated axons
Axons
No surrounding myelin
Cytoplasm is dark & contains closely spaced neurofilaments.
Some myelinated axons may remain in the same area (Right)
Schwann cells & their processes
Surround the demyelinated axons
May be multiple Schwann cells around single axon
Electron micrograph: From Robert Schmidt MD
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Electron micrographs: From Robert Schmidt MD
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Axons: Chronic demyelination
No myelination around axon
Schwann cell processes
Multiple
Surround axon
No onion bulbs
Lipofuscin debris (Arrow)
Usually only present in non-myelinating Schwann cells
Electron micrographs: From Robert Schmidt MD
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Nerve conduction studies (Ulnar nerve)
Temporal dispersion
NCV: Moderate slowing
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Electron micrographs: From Robert Schmidt MD
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Schwann cell processes
Surround demyelinated axon
Have associated basal lamina
Electron micrographs: From Robert Schmidt MD
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Electron micrographs: From Robert Schmidt MD
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Electron micrographs: From Robert Schmidt MD
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Hypomyelinated axons
Surrounded by few, or multiple, Schwann cell processes
Electron micrographs: From Robert Schmidt MD
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Loss of Small Axons: Collagen Pockets (Same nerve as above)
Electron micrographs: From Robert Schmidt MD
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Electron micrographs: From Robert Schmidt MD
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Pi Granules
Electron micrographs: From Robert Schmidt MD
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Chronic demyelination
4/7/2021