title: Axon cytoskeleton ultrastructure in chronic inflammatory demyelinating polyneuropathy.
abstract: BACKGROUND
  To detail the extent and pattern of axon cytoskeleton alterations in chronic inflammatory demyelinating polyneuropathy (CIDP).
  
  
  METHODS
  Nerve biopsies from 7 cases of CIDP, including 4 cases with severe fiber loss, were compared with 5 controls by morphometric transmission electron microscopy (TEM).
  
  
  RESULTS
  Despite demyelination of single fibers, myelin ultrastructure was otherwise normal. Contrary to immunolabeling, TEM revealed a decrease in neurofilament (NF) density in every case, although there were pronounced variations among fibers even in the same sample. The NF decrease reached the same extent in large- and small-diameter fibers. It was observed in normally myelinated fibers, suggesting they were demyelinated at a distance from the section. Minimal inter-NF distance increased roughly inversely to NF density. Microtubules increased in 3 cases previously characterized by increased growth-associated protein (GAP-43) immunolabeling.
  
  
  CONCLUSIONS
  These data demonstrate the severity and constancy of axonal lesions, and especially of NF, in residual fibers in our cases of CIDP.
authors: Neurology Department, University Hospital, 4 rue Larrey, F49933 Angers Cedex 9, France. catherine.fressinaud@univ-angers.fr
authors: Catherine
authors: C
authors: Fressinaud
authors: Frédéric
authors: F
authors: Dubas
mesh_terms: D000368:Aged; D001369:Axons / Q000473:pathology / Q000648:ultrastructure*; D001706:Biopsy; D016022:Case-Control Studies; D003599:Cytoskeleton / Q000648:ultrastructure*; D005260:Female; D019922:GAP-43 Protein / Q000378:metabolism; D006801:Humans; D008297:Male; D008870:Microtubules / Q000648:ultrastructure; D008875:Middle Aged; D009186:Myelin Sheath / Q000648:ultrastructure; D009454:Neurofibrils / Q000648:ultrastructure; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000378:metabolism / Q000473:pathology*; D012720:Severity of Illness Index
chemical_list: D019922:GAP-43 Protein