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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
Muscle & Nerve
|October 15, 2011
Summary
Axon cytoskeleton damage, particularly reduced neurofilament (NF) density, is a constant feature in chronic inflammatory demyelinating polyneuropathy (CIDP) nerve fibers. These axonal lesions are severe even in residual fibers.
Area of Science:
- Neuroscience
- Pathology
- Electron Microscopy
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder affecting peripheral nerves.
- Understanding axonal alterations is crucial for diagnosing and treating CIDP.
Purpose of the Study:
- To investigate the extent and pattern of axon cytoskeleton changes in CIDP.
- To detail neurofilament (NF) and microtubule alterations in CIDP axons.
Main Methods:
- Nerve biopsies from 7 CIDP patients and 5 controls were analyzed.
- Morphometric transmission electron microscopy (TEM) was used for ultrastructural analysis.
Main Results:
- TEM revealed decreased neurofilament (NF) density in all CIDP cases, irrespective of fiber size.
- Axonal lesions, specifically reduced NF density, were observed even in normally myelinated fibers.
- Microtubule numbers increased in some CIDP cases, correlating with GAP-43 expression.
Conclusions:
- Axonal damage, particularly neurofilament loss, is a consistent and severe finding in CIDP residual fibers.
- These findings highlight the significance of axonal pathology in CIDP progression.
