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Unusual Inflammatory Neuropathy

Jon Wilson, MD neuropathologist at arkana laboratories
By Jon Wilson, MD

Feb 07, 2024

Unusual Inflammatory Neuropathy
Figure 1: FFPE hematoxylin and eosin

Clinical History:

This 60-year-old patient presented with a history of chronic progressive paresthesias involving all four extremities. Electrodiagnostic studies showed evidence of a generalized motor and sensory neuropathy with axonal and demyelinating features. Physical examination showed decreased sensation to pain, temperature, vibration and tactile stimulation to both feet.

Which of the following is the most likely diagnosis based on figures #1 to #5?

A. Vasculitis

B. CIDP

C. Sarcoidosis

D. Leprosy

 

 

Figure 1: Medium magnification images showing features of a chronic inflammatory neuropathy with thickening of the perineurium and extensive fibrosis of the endoneurium. Neurofilament immunohistochemical stain (also performed on this case but not shown) demonstrated that the nerve fascicles were devoid of axons (end-stage nerve).

Figure 2: Higher magnification images showing features of a chronic inflammatory neuropathy with thickening of the perineurium and extensive fibrosis of the endoneurium.

Figure 3: High magnification image of special stain for acid fast bacilli (AFB stain) shows numerous positive staining rods. In the left image the organisms are within Schwann cells and macrophages. In the right image the organisms are within endothelial cells of endoneurial small blood vessels.

Figure 4: Electron microscopy showed multiple organisms within Schwann cell and macrophage cytoplasm.

Figure 5: Electron microscopy also confirmed the presence of organisms within endothelial cell cytoplasm.

 

Correct Answer: Leprosy

  • The correct answer is “Leprosy” (more specifically leprous neuropathy, with morphologic features of lepromatous type disease).
  • Cases of leprosy have recently been identified in the United States.
  • Please see references

 

Reference(s) / additional reading:

 

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Quick note: This post is to be used for informational purposes only and does not constitute medical or health advice. Each person should consult their own doctor with respect to matters referenced. Arkana Laboratories assumes no liability for actions taken in reliance upon the information contained herein.