title: Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype.
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.
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia.
authors: Emily K
authors: EK
authors: Mathey
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia Neuroscience Research Australia & Prince of Wales Clinical School, University of New South Wales, Randwick, New South Wales, Australia.
authors: Susanna B
authors: SB
authors: Park
authors: MRC Centre for Neuromuscular Diseases, Institute of Neurology, University College London, London, UK.
authors: Richard A C
authors: RA
authors: Hughes
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia.
authors: John D
authors: JD
authors: Pollard
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia.
authors: Patricia J
authors: PJ
authors: Armati
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia.
authors: Michael H
authors: MH
authors: Barnett
authors: Menzies Research Institute, University of Tasmania, Sydney, New South Wales, Australia.
authors: Bruce V
authors: BV
authors: Taylor
authors: Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
authors: P James B
authors: PJ
authors: Dyck
authors: Brain and Mind Research Institute, University of Sydney, Sydney, New South Wales, Australia.
authors: Matthew C
authors: MC
authors: Kiernan
authors: Faculty of Medicine, Department of Physiology, Translational Neuroscience Facility, School of Medical Sciences, University of New South Wales, Randwick, New South Wales, Australia.
authors: Cindy S-Y
authors: CS
authors: Lin
mesh_terms: D006801:Humans; D009186:Myelin Sheath / Q000276:immunology / Q000473:pathology*; D010641:Phenotype; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000276:immunology / Q000473:pathology*; D012583:Schwann Cells / Q000276:immunology / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology
keywords: MYELIN; NEUROIMMUNOLOGY; NEUROPATHY; NEUROPHYSIOLOGY; SCHWANN CELL