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Review
Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype
  1. Emily K Mathey1,
  2. Susanna B Park1,2,
  3. Richard A C Hughes3,
  4. John D Pollard1,
  5. Patricia J Armati1,
  6. Michael H Barnett1,
  7. Bruce V Taylor4,
  8. P James B Dyck5,
  9. Matthew C Kiernan1,
  10. Cindy S-Y Lin6
  1. 1Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia
  2. 2Neuroscience Research Australia & Prince of Wales Clinical School, University of New South Wales, Randwick, New South Wales, Australia
  3. 3MRC Centre for Neuromuscular Diseases, Institute of Neurology, University College London, London, UK
  4. 4Menzies Research Institute, University of Tasmania, Sydney, New South Wales, Australia
  5. 5Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA
  6. 6Faculty of Medicine, Department of Physiology, Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Randwick, New South Wales, Australia
  1. Correspondence to Dr Cindy S-Y Lin, Faculty of Medicine, Department of Physiology, Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Sydney, NSW 2052 Australia

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.

  • MYELIN
  • NEUROIMMUNOLOGY
  • NEUROPATHY
  • NEUROPHYSIOLOGY
  • SCHWANN CELL

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