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A Simple Approach to Induce Experimental Autoimmune Neuritis in C57BL/6 Mice for Functional and Neuropathological Assessments
Published on: November 9, 2017
Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy
Richard A C Hughes1, David Allen, Anna Makowska
1Department of Clinical Neuroscience, King's College London, Guy's Hospital, London, UK. richard.a.hughes@kcl.ac.uk
Journal of the Peripheral Nervous System : JPNS
|March 8, 2006
Summary
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) involves immune cell activation and potential autoantigens like P0. Understanding regulatory T-cell dysfunction is key to differentiating CIDP from Guillain-Barré syndrome.
Area of Science:
- Neuroimmunology
- Neurology
- Pathology
Background:
- Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by endoneurial inflammation.
- Key features include T cell and macrophage activation, with chemokine and receptor expression.
Purpose of the Study:
- To elucidate the immunological underpinnings of CIDP.
- To identify potential autoantigens involved in CIDP pathogenesis.
- To differentiate CIDP from Guillain-Barré syndrome based on immune mechanisms.
Main Methods:
- Analysis of endoneurial lesions in CIDP.
- Investigation of myelin protein antigens (P2, P0, PMP22) in experimental autoimmune neuritis models.
- Detection of antibodies against myelin proteins in CIDP patients.
Main Results:
- Evidence suggests myelin proteins, particularly P0, may act as autoantigens in CIDP.
- Antibodies to P0 were detected in 20% of CIDP cases.
- Regulatory T-cell dysfunction is implicated in persistent or recurrent CIDP.
Conclusions:
- CIDP pathogenesis involves immune cell infiltration and potential autoimmune responses to myelin antigens.
- Failure of regulatory T-cell mechanisms distinguishes CIDP from acute inflammatory demyelinating polyradiculoneuropathy (AIDP).
- While current treatments offer short-term relief, long-term efficacy of immunosuppressants requires further investigation through controlled trials.
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