Article

Journal of Neurocytology

, Volume 25, Issue 1, pp 33-51

Early nodal changes in the acute motor axonal neuropathy pattern of the Guillain-Barré syndrome

  • J. W. GriffinAffiliated withDepartment of Neuroscience, Johns Hopkins University School of MedicineDepartment of Neurology, Johns Hopkins University School of Medicine
  • , C. Y. LiAffiliated withDepartment of Neurology, Second Teaching Hospital of Hebei Province
  • , C. Macko
  • , T. W. HoAffiliated withDepartment of Neurology, Johns Hopkins University School of Medicine
  • , S. -T. HsiehAffiliated withDepartment of Neurology, Johns Hopkins University School of Medicine
  • , P. XueAffiliated withDepartment of Neurology, Second Teaching Hospital of Hebei Province
  • , F. A. WangAffiliated withDepartment of Neurology, Second Teaching Hospital of Hebei Province
  • , D. R. CornblathAffiliated withDepartment of Neurology, Johns Hopkins University School of Medicine
  • , G. M. McKhannAffiliated withZanvyl Krieger Mind-Brain Institute, Johns Hopkins UniversityDepartment of Neurology, Johns Hopkins University School of Medicine
    • , A. K. AsburyAffiliated withDepartment of Neurology, University of Pennsylvania School of Medicine

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Summary

The axonal patterns of Guillain-Barré syndrome, associated in many cases with antecedentCampylobacter jejuni infection, are now recognized as frequent causes of acute flaccid paralysis in some regions of the world. This study examined ultrastructurally the PNS of seven cases of the acute motor axonal neuropathy form of Guillain-Barré syndrome. In this disorder previous studies of advanced cases have found Wallerian-like degeneration of motor fibres in the spinal roots and peripheral nerves, with little lymphocytic inflammation or demyelination. The present study was focused on identifying early changes and establishing the sequence of changes. By electron microscopy the earliest and mildest changes consisted of lengthening of the node of Ranvier with distortion of the paranodal myelin, and in some instances with breakdown of the outermost myelin terminal loops. At this stage many nodes had overlying macrophages which extended their processes through the Schwann cell basal lamina covering the node and apposed the axolemma. Macrophage processes then extended beneath the myelin terminal loops, and the whole macrophage entered the periaxonal space at the paranode. Macrophage processes dissected the axon from the adaxonal Schwann cell plasmalemma and the macrophages advanced into the internodal periaxonal space, where they typically surrounded a condensed-appearing axon. At this stage the adaxonal Schwann cell cytoplasm regularly degenerated and disappeared, so that the periaxonal space was bounded by the innermost myelin lamella, and the axolemma of many fibres could not be seen. The internodal myelin sheath and the abaxonal Schwann cell cytoplasm remained normal. This arrangement appeared to be stable for some time, but in many fibres the axon subsequently underwent Wallerian-like degeneration. By interfering with impulse conduction, these nodal and periaxonal changes may explain paralysis in some pathologically mild cases. In addition, at early stages, these changes may be reversible, thus explaining the rapid recovery of some patients who become paralysed with acute motor axonal neuropathy. These observations, taken together with previous studies, suggest that acute motor axonal neuropathy is an antibody- and complement-mediated disorder in which the relevant epitopes are present on the nodal and internodal axolemma.