Neuromuscular

Chronic Immune Polyneuropathy with Vesicular Demyelination 1, Schwann-process Bulbs & Axon Vesicles

Myelin sheaths
  Patterns
  Thin
  Vesicular pathology
  Demyelination
  Molecular
Schwann cells
  Process bulbs
    Molecular
  Nuclei increased
Axons
  Vescicle-like structures in axoplasm
Histology
Other

Myelin Sheaths: Thin


Toluidine blue stain
Myelination
  Many thinly myelinated large axons


Toluidine blue stain
Myelination
  Many thinly myelinated axons
  Scattered demyelinated axons (Arrow above)
  Many large axons have blue-stained surrounding tissue, often containing Schwann cell nuclei
Myelin Structure
  Irregular around some axons (Arrows below)

Toluidine blue stain

Myelin Sheaths: Thin
Schwann cells: Processes & nuclei surround thinly myelinated axons

Schwann-process Bulbs
  Schwann cell processes & collagen surround thinly myelinated axons
Schwann-process Bulbs
  Schwann cell processes & collagen surround thinly myelinated axons

Myelin Basic Protein on & around Axons
  Present on larger axons without myelin sheath (Yellow) (Arrow)
  Thin layer (Red) surrounds other large axons (Immature Schwann cell processes)

Neurofilament Green; MBP Red

NCAM on & around Axons
  Present (Red) around a few larger axons (Arrow)
  Small axons
    Loss: NCAM regions (Red) are empty or have only a few axons
    Some remaining small axons are beaded (Yellow)

Neurofilament Green; NCAM Red

P0 Protein around Axons
  Mild-Moderate loss of larger axons

Neurofilament Green; P0 Red

Schwann cell processes
  Scattered in collagen around axon

Myelin Pathology: Vesicular
Vesicular pathology
  In inner region of myelin sheath

Myelin: Vesicular pathology
  Replaces inner region of myelin sheath
Schwann cell processes
  Surround myelinated axon
Axoplasm
  Contains vesicular structures

Myelin
  Vesicular changes: Replace regions of thin myelin sheath
Axoplasm: Contains vesicles
Myelin
  Vesicular changes: Within & at external regions of myelin sheath

Axon
  Large
  Axoplasm: Abnormal with vesicles
Myelin
  Vesiculated near axon

Myelin Sheath: Absent
Schwann cell processes: Surround demyelinated axon
Schwann cell nuclei: Multiple; In and around Schwann cell processes

Myelin, Molecular
  Abnormal co-expression of P0 & NCAM (Yellow) in sheaths (Red)

NCAM Green; P0 Red

Axoplasm Pathology

Myelin Sheath
  Thin
  Variable width
Schwann cells
  Processes: Surround axon
  Nuclei: Multiple present
Axon
  Large
  Axoplasm: Abnormal with vesicles

Myelin Sheath
  Thin
Schwann cells
  Processes: Surround axon
Axon
  Large
  Axoplasm: Abnormal with vesicles

Myelin Sheath
  Thin
Schwann cells
  Few Processes: Around axon
Axon
  Large
  Axoplasm: Abnormal with many vesicles

Myelin sheath
  Shape: Flat
  Little axoplasm visible inside



H&E stain
Endoneurial edema

H&E stain


Gomori trichrome stain
Myelinated Axon loss
  Moderate
Myelin Sheaths
  Many abnormal: Reduced (Red) staining


VvG stain
Myelinated Axon loss
  Moderate


VvG stain
Endoneurial Microvessel
  Occluded


Acid phosphatase stain
Myelin: Mild, diffuse acid phosphatase stain


ATPase pH 4.3 stain
Endoneurium: Normal


Neurofilament stain
Large axons
  Reduced number
  Remaining large axons have no, or little associated myelin (Arrow)


NCAM stain
NCAM abnormally stains remaining myelin sheaths (Arrow)

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References
1. Ann Neurol 1996;39:625-35

4/20/2025