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Review
Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment
  1. Helmar Christoph Lehmann1,
  2. David Burke2,
  3. Satoshi Kuwabara3
  1. 1Neurology, University Hospital of Cologne, Köln, Germany
  2. 2Institute of Clinical Neurosciences, University of Sydney, Sydney, New South Wales, Australia
  3. 3Neurology, Chiba University School of Medicine, Chiba, Japan
  1. Correspondence to Dr Helmar Christoph Lehmann, Neurology, University Hospital of Cologne, Köln 50937, Germany; helmar.lehmann{at}uk-koeln.de

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

  • CIDP
  • immune neuropathy
  • peripheral nerve
  • neuroimaging
  • epidemiology

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Footnotes

  • Contributors All authors contributed to and approved the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; externally peer reviewed.

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