4.7 Article

The diagnosis of chronic inflammatory demyelinating polyneuropathy: a Delphi-method approach

Journal

JOURNAL OF NEUROLOGY
Volume 260, Issue 12, Pages 3015-3022

Publisher

SPRINGER HEIDELBERG
DOI: 10.1007/s00415-013-7100-2

Keywords

Chronic inflammatory demyelinating polyneuropathy; Diagnosis; Delphi method

Funding

  1. Biogen-Idec
  2. CSL-Behring
  3. Genzyme
  4. Laboratoire Francais des Biotechnologies
  5. Merck-Serono
  6. Novartis
  7. Teva
  8. Editions Scientifiques L&C, Expression Sante
  9. Natus
  10. Scien
  11. SNF-Floerger
  12. CSL Behring
  13. LFB
  14. Pfizer
  15. Biogen Idec
  16. CSL-Behring AG Switzerland
  17. Baxter
  18. Octapharma
  19. Debiopharm
  20. GSK
  21. Ipsen
  22. Bayer Shering Pharma

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The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) is based on a set of clinical and neurophysiological parameters. However, in clinical practice, CIDP remains difficult to diagnose in atypical cases. In the present study, 32 experts from 22 centers (the French CIDP study group) were asked individually to score four typical, and seven atypical, CIDP observations (TOs and AOs, respectively) reported by other physicians, according to the Delphi method. The diagnoses of CIDP were confirmed by the group in 96.9 % of the TO and 60.1 % of the AO (p < 0.0001). There was a positive correlation between the consensus of CIDP diagnosis and the demyelinating features (r = 0.82, p < 0.004). The European CIDP classification was used in 28.3 % of the TOs and 18.2 % of the AOs (p < 0.002). The French CIDP study group diagnostic strategy was used in 90 % of the TOs and 61 % of the AOs (p < 0.0001). In 3 % of the TOs and 21.6 % of the AOs, the experts had difficulty determining a final diagnosis due to a lack of information. This study shows that a set of criteria and a diagnostic strategy are not sufficient to reach a consensus for the diagnosis of atypical CIDP in clinical practice.

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