Blood-nerve barrier in IgM paraproteinemic neuropathy: a clinicopathologic assessment

Acta Neuropathol. 1998 Feb;95(2):184-92. doi: 10.1007/s004010050785.


We report the pathologic findings in a patient with sensorimotor neuropathy associated with Waldenström's macroglobulinemia, particularly in relation to blood-nerve barrier defects. The monoclonal IgM was of kappa type and possessed anti-HNK-1 activity. A sural nerve biopsy specimen revealed severe loss of myelinated and unmyelinated nerve fibers and gaps between adjacent endothelial cells of small endoneurial vessels. Postmortem findings 3 years later included severe loss of myelinated nerve fibers and diffuse infiltration by lymphoplasmacytic B cells throughout the peripheral nervous system, sparing the central nervous system. Findings in this case suggest an immune attack against endoneurial endothelial cells with permeation of IgM into peripheral nerve tissue.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autopsy
  • Biopsy
  • Endothelium, Vascular / pathology*
  • Endothelium, Vascular / ultrastructure
  • Female
  • Humans
  • Immunoglobulin M / blood
  • Immunoglobulin M / cerebrospinal fluid
  • Intercellular Junctions / pathology*
  • Intercellular Junctions / ultrastructure
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Nerve Fibers / pathology
  • Nerve Fibers / ultrastructure
  • Reference Values
  • Spinal Cord / pathology
  • Spinal Nerve Roots / pathology
  • Sural Nerve / blood supply*
  • Sural Nerve / pathology*
  • Sural Nerve / ultrastructure
  • Waldenstrom Macroglobulinemia / immunology
  • Waldenstrom Macroglobulinemia / pathology*


  • Immunoglobulin M