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Axon cytoskeleton ultrastructure in chronic inflammatory demyelinating polyneuropathy
Catherine Fressinaud1, Frédéric Dubas
1Neurology Department, University Hospital, 4 rue Larrey, F49933 Angers Cedex 9, France. catherine.fressinaud@univ-angers.fr
Introduction:
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.
Conclusion:
These data demonstrate the severity and constancy of axonal lesions, and especially of NF, in residual fibers in our cases of CIDP.
